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Objective: Imatinib is a commonly used first generation tyrosine kinase inhibitor for patients with chronic myeloid leukemia (CML). The efficacy has been reported as very high even in recent studies. Material and methods: A retrospective analysis was made of newly diagnosed CML patients treated with Imatinib as a first-line agent from January 2010 to January 2020. The patients were classified as those who obtained an adequate response and those for whom treatment was discontinued due to inadequate efficacy. The two groups were compared to analyze factors predicting the efficacy of the agent. Results: Evaluation was made of a total of 47 CML patients, comprising 20 females (42.6%) and 27 males (57.4%) with a median age of 55 years. Imatinib was discontinued in 19 patients because of inadequate response, and 28 patients were still continuing the treatment at the end of median 33.3 months follow-up duration. At the end of follow-up, there were 44 survivors (93.6%), and 3 non-survivors (6.4%). Median Bcr-Abl (IS, %) at the time of diagnosis in patients with response was higher than patients in discontinued group (67.6 [0.0-291.4] vs 41.9 [0.0-208.5], p=0.022). All other disease and demographic characteristics were similar in both groups (p>0.05). Conclusion: Almost 10 years of follow-up demonstrated that there is still an unmet need to determine factors predicting the response to Imatinib in CML patients. Larger population-based studies are required to specify patients with high risk at the time of diagnosis to monitor closely.

Introduction Predictive parameters that can affect the course of this infection have been the main topic of research since the beginning of the COVID-19 (Coronavirus disease 2019) pandemic. Since the discovery of blood groups, the effect of these on infectious diseases has always been of interest. Objectives To analyze the effect of ABO blood group on mortality, hospitalization duration and hematological and cytokine storm parameters in patients with COVID-19. Patients and methods: This retrospective study was conducted on 140 patients diagnosed with COVID-19. Demographic characteristics, laboratory parameters including ABO blood group, complete blood count (CBC) parameters, biochemical tests, cytokine storm parameters, duration of hospitalization, and final status (discharge or death) were recorded. Results: The 140 patients included in the analysis comprised 72 (51.4%) males and 68 (48.6%) females with a mean age of 66.3±14.0 years. . Age and gender, hospitalization duration and mortality rates were similar in all blood group types. Only D-dimer levels were found to be higher in blood group A compared with other blood groups. Conclusion: Although no difference in mortality was determined between groups, the D-dimer level was statistically significantly higher in COVID-19 patients with A blood group. Larger studies are needed to reflect D-dimer levels on the clinical course of infection, and thus on daily practice.

What is known and objective Primary myelofibrosis (PMF) is characterized by myeloid cell proliferation and prominent bone marrow fibrosis. Ruxolitinib, a selective inhibitor of JAK 1 and 2, significantly reduces constitutional symptoms and spleen size compared with placebo, and has significant clinical benefits in patients with myelofibrosis. The most common haematological side effects are thrombocytopenia and anaemia, and the most common non-haematological side effects are grade 1-2 diarrhoea and pyrexia. Leukocytoclastic vasculitis is small vessel vasculitis, characterized histopathologically by immune complex-mediated vasculitis of the dermal capillaries and venules in the lower extremities, which can be seen as palpable purpura. Although the cause is 50% idiopathic, the aetiology of leukocytoclastic vasculitis can be collected under many headings. Case summary The case is here presented of a patient with PMF who developed leukocytoclastic vasculitis after ruxolitinib treatment. Ruxolitinib was discontinued as the lesions were thought to be drug-related and all skin lesions disappeared approximately 2 months after termination of the drug. When the ruxolitinib treatment was restarted at the same dose (2 × 15 mg), the skin lesions recurred. The drug dose was reduced to 1 × 15 mg, and the rashes disappeared. Currently, the patient has no active complaints and is being followed up with ruxolitinib 1 × 15 mg without any complications. What is new and conclusion To the best of our knowledge, leukocytoclastic vasculitis due to ruxolitinib is extremely uncommon. This case report can be considered to contribute to the literature of this rare event.

There are only a few predictive markers that can truly aid therapy decisions in patients with acute myeloid leukemia (AML). The current study aimed to examine the impact of easily available common laboratory parameters on the course and prognosis of patients with AML. Gender, initial bone marrow blast percentage, mean platelet volume (MPV), lymphocyte-to-monocyte ratio, treatment regimen, and complete remission (CR1) were found to have a statistically significant effect on both OS and PFS (

Saçlı hücreli lösemi (SHL) nadir görülen kronik lenfoproliferatif hastalıklar arasında yer alan, relaps ve remisyonlar ile seyreden indolent bir lenfomadır. Tedavisinde pürin analoğu (PA) kullanımı ile hastaların prognoz ve sağ kalımında önemli gelişmeler kaydedilmiştir. Bu çalışmamızda ilk basamakta kullanılan purin analogu tedavisinin iki farklı protokol ile verilmesinin nüks ve sağ kalım üzerine etkisini araştırdık. Kliniğimizde 2009-2019 yılları arasında SHL nedeniyle PA ile tedavi edilen 20 vakayı retrospektif olarak inceledik. Bunlardan tek siklus PA uygulanan hastalar ile iki siklüs ardışık PA tedavisi alan hasta gruplarını tanı anındaki klinik özellikleri, laboratuvar bulguları yanıt ve nüks oranları açısından karşılaştırdık. Her iki grupta yanıt oranları eşit olmakla birlikte, ilk basamak tedaviye tam yanıt oranı %90, kısmı yanıt oranı %10 olarak değerlendirilmiştir. Hastalarımızın median takip süresi 69 aydır ve bu süre içerisinde her iki gruptan birer hastada nüks izlenmiştir. Tek siklus tedavi alan hastalardan 1 tanesi hastalık dışı nedenle eks olmuştur. Ardışık iki siklus 2Cd-A uygulanmasının yanıt ve nüks oranı ilişkili üstünlüğü gösterilememiştir. İlk basamak tedavide uygulanan 2Cd-A, yüksek yanıt oranı ile etkili bir ajandır.

Introduction: Central nervous system lymphomas (CNSLs) require effective treatment strategies due to aggressive nature of disease. Despite therapeutic approaches having improved in the last decades, there is no standard treatment for these patients. As a CNSL targeted-therapy IDARAM protocol was developed, the outcomes were reported with a few studies. We observed the R-IDARAM protocol in our CNSL cases, and we discuss the effectiveness, tolerability, and toxicity with a review of the literature in this article. Subjects and Methods: We retrospectively analyzed response rates, progression-free survival, adverse events, and long-term side effects in patients who were treated by modified R-IDARAM as standard clinical care of CNSL in our hematology department. Results: Response was achieved in five of nine patients. Three patients (two primary CNSL and one secondary CNSL) are still being followed up without disease progression with a median duration of follow-up of 79 months (88, 79, and 17 months, respectively). Manageable hematological side effects including thrombocytopenia and neutropenia were experienced by all patients. Conclusion: R-IDARAM protocol may be an option with high early response rates and manageable toxicity. Hematological side effects are the main problem, and long-term neurological toxicity is not common. Eligible patients must continue with autologous stem cell transplantation due to poor long-term survival outcomes.

We report a case of a localized, massive, diffuse large B cell splenic lymphoma diagnosed by splenectomy. A 61-year-old man complaining of abdominal pain was admitted to our hospital. On abdominal tomography examination, there was seen to be a significant increase in the spleen dimensions and a lesion of heterogenous density was observed to be covering almost all of the spleen parenchyma. Intraoperatively, a massive hematoma involving approximately 95% of the spleen and ischemic areas were observed, so it was decided to perform splenectomy. With an improvement in general condition and no active complaints, the patient was discharged, but then failed to attend for follow-up examinations. At 7 months after the splenectomy, the patient again presented at the Emergency Department with complaints of abdominal pain, nausea, and vomiting. On abdominal tomography, a solid mass was observed, approximately 12 × 9 cm in size with irregular contours in the margins of the splenectomy location. The diagnosis was reported as non-germinal center diffuse large B cell lymphoma showing involvement with the colon and small intestine segments. It was learned that the patient had not attended follow-up examinations and when the splenectomy material of 17.5 × 13.5 × 6 cm was examined, it was reported as non-germinal center diffuse large B cell lymphoma. This case is of importance in respect of the development of recurrence in less than a year as splenectomy only without systemic chemotherapy was insufficient in the treatment of diffuse large B cell lymphoma with isolated spleen involvement.

Background Hairy cell leukemia is a rare B-cell lymphoproliferative disorder. It has an indolent course with relapse and remission periods. The aim of this study was to investigate the clinical characteristics and risk factors affecting the outcome of patients with hairy cell leukemia. Patients and Methods The retrospective data of 65 patients were evaluated according to initial hematologic and biochemical parameters, response rates, progression-free survival, and overall survival. Factors effecting response and survival rates were analyzed. Results The median follow-up duration was 62.8 months (range, 5.7-229.3 months). The result of the analysis showed that the patients with relapse/progressive disease had higher lactate dehydrogenase (LDH) levels at the time of diagnosis than patients without relapse/progression (median [range], 243 [137-540] vs. 179 [99-334] U/L, P = .01). Patients with LDH ≥ 200.5 IU at the time of diagnosis were demonstrated to have a shorter progression-free survival than those with LDH Conclusion Serum LDH level is significantly associated with relapse/progression in hairy cell leukemia patients. Patients with higher LDH levels at diagnosis should be monitored closely even if they experience complete remission.

Objective: The aim of this study was to investigate the survival data of patients with acute myeloid leukemia (AML) and to determine the risk factors that can be easily evaluated. Method: A retrospective analysis was made of the AML patients admitted to our center between 2009-2018. Demographic data and disease data were analyzed and response rates, overall survival (OS) and progression-free survival (PFS) rates were calculated. Factors affecting survival were determined using Cox-regression analysis. Results: A total of 119 patients were included in the study during the 9-year study period. Of these, 21 patients had secondary AML and 11 had acute promyelocytic leukemia (APL). The mean follow-up period was 12.43 ± 15.63 months. OS of all patients was 9.20 months and PFS was 7.23 months. Age and leukocyte count at the time of diagnosis were found to have a significant impact on both OS and PFS (p

Introduction Ibrutinib is an oral irreversible inhibitor of Bruton’s tyrosine kinase signaling. It is a well-tolerated agent with some side-effects, the most common of which are atrial fibrillation, diarrhea, upper respiratory tract infection, fatigue, nausea, rash and cytopenias. Most of these toxicities are mild, although some have a severe clinical course. Case report The case is here reported of a chronic lymphocytic leukemia patient with ibrutinib-induced polyneuropathy. A 63-year-old male patient with chronic lymphocytic leukemia was given ibrutinib as a third line treatment regimen. After the 10th month of therapy he had progressive complaints of numbness and tingling in his legs. The patient was diagnosed as grade 3 sensorineural polyneuropathy with electromyography. Management and outcome: Considering that ibrutinib treatment may cause neuropathy, the ibrutinib was discontinued, after which the neuropathic complaints improved. However, the neck and axillary lymph nodes were enlarged and treatment had to be re-started therefore ibrutinib was started at a low dose and gradually increased. The patient is currently in the 14th month of treatment and still using ibrutinib without any severe side-effects. Discussion To the best of our knowledge, polyneuropathy as a unique side-effect of ibrutinib has not been previously reported. In addition to the well-known side effects of ibrutinib treatment, it should be kept in mind that polyneuropathy may also develop.

Introduction Coronavirus disease 2019 (COVID-19) were declared as pandemic by World Health Organization. With this study, we aimed to define our patients who were followed up with malign or benign hematological diagnoses and diagnosed with COVID-19; determine the distribution of this infection in patient groups and contribute to the literature by creating descriptive statistics with its clinical and demographic features.Patients and methods: It is planned to retrospectively examine patients with a history of COVID-19 who were followed up in Hematology Department of Dışkapı Yıldırım Beyazıt Training and Research Hospital with benign and malignant diagnoses.Results: 88 patients who had COVID-19 infection while being followed in our clinic due to hematological diseases were included in the study. 77 patients had been followed by hematologic malignancies and 11 patients had been followed by benign hematological disease.In the group with malignancy, COVID was found most frequently in patients diagnosed with Philadelphia chromosome negative myeloproliferative neoplasms (22%), nonhodgkin lymphoma (19%) and multiple myeloma (16%). ITP (64%) was the most common disease in patients with benign hematological disorder who had COVID history. 52 (67%) of the malignant cases and 8 (73%) of the bening cases were found to be followed up with the disease in remission.The all patient's most common symptoms at COVID-19 diagnosis were fever (77%), cough (70%) and weakness (65%). 45% of the patients were isolated at home, 48% were required hospitalization. 49% of patients had mild; 27% had moderate and 24% had severe COVID-19 infection. Almost all of the patients in the moderate and severe disease group were followed up in patients diagnosed with malignant hematological disease. 16 (18%) patients received mechanical ventilation and 16 (18%) patients was transferred intensive care unit. All of the patients who were intubated and needed intensive care were diagnosed with malignant hematological disease. 17 patients died due to COVID-19 infection. The mortality rate was 22% in patients with diagnosis of malignant hematological diseases, and 19% when all patients (malignant and bening) were included. Conclusion: In conclusion, the COVID-19 pandemic is a problem all over the world. Determining the course of the disease in certain diagnostic groups is important in the management of both the main disease and the COVID-19 infection. Therefore, the contribution of such recording studies to the literature is important and valuable., GirişCoronavirus hastalığı 2019 (COVID-19), Dünya Sağlık Örgütü tarafından pandemi olarak ilan edildi. Bu çalışma ile malign veya benign hematolojik tanılarla takip edilen ve COVID-19 tanısı alan hastalarımızı analiz edip; bu enfeksiyonun hasta gruplarına göre dağılımını belirleyerek ve hematolojik hastalardaki seyrini inceleyerek tanımlayıcı istatistikler oluşturup literatüre katkı sağlamayı amaçladık.Araç ve yöntemlerDışkapı Yıldırım Beyazıt Eğitim ve Araştırma Hastanesi Hematoloji Bölümü'nde bening ve malign hematolojik tanılarla takip edilen ve COVID-19 öyküsü olan hastalar retrospektif olarak incelenmiştir.Bulgular: Kliniğimizde hematolojik hastalıklar nedeniyle takip edilirken COVID-19 tanısı alan 88 hasta çalışmaya dahil edildi. 77 hasta hematolojik maligniteler, 11 hastayı da benign hematolojik tanılar ile takip ediyordu. Malign tanılarla izlenen grupta COVID en sık Philadelphia kromozomu negatif miyeloproliferatif neoplazm (%22), non-hodgkin lenfoma (%19) ve multipl miyelom (%16) tanılı hastalarda görüldü. Bening hastalık grubunda ise ITP (%64), COVID öyküsü olan hastalarda en sık görülen hastalıktı. Malign olguların 52'sinin (%67) ve bening olgularının 8'inin (%73) remisyonda hastalıkları remisyonda idi.Hastaların COVID-19 tanısında en sık görülen semptomları ateş (%77), öksürük (%70) ve halsizlik (%65) idi. Hastaların %45'i evde izole edildi, %48'i hastaneye yatırıldı. Hastaların %49'u hafif; %27'si orta ve %24'ü şiddetli COVID-19 kliniğine sahipti. Orta ve ağır hastalık grubundaki hastaların tamamına yakını malign hematolojik hastalık grubunda idi. 16 (%18) hasta yoğun bakım ünitesine devredildi ve tümünün mekanik ventilasyon ihtiyacı oldu. Entübe edilen ve yoğun bakıma ihtiyacı olan hastaların tamamı malign hematolojik hastalık tanısı olan hastalardı. 17 hasta COVID-19 enfeksiyonu nedeniyle öldü. Mortalite oranı malign hematolojik hastalık grubunda %22, tüm hastalar (malign ve bening) dahil edildiğinde ise %19 idi.Tartışma: Sonuç olarak, COVID-19 pandemisi dünya genelinde sorun teşkil etmektedir. Belirli tanı gruplarında hastalığın seyrinin belirlenmesi hem ana hastalığın hem de COVID-19 enfeksiyonunun yönetiminde önemlidir. Bu nedenle bu tür kayıt çalışmalarının literatüre katkısı önemli ve değerlidir.

Introduction All-trans retinoic acid (ATRA) is a physiological metabolite of vitamin A and it is used for the treatment of acute promyelocytic leukemia (APL). Hypercalcemia is a rare side effect of ATRA and it may be potentiated after interaction of ATRA with azole group antifungals. Herein, we have reported an APL case with hypercalcemia that is caused by the interaction of ATRA and posaconazole. Case Report A 49-year-old female patient was diagnosed as APL after the examinations performed upon the detection of pancytopenia when she had presented with the complaints of widespread bruising and fever. After the initiation of posaconazole and ATRA, her serum calcium levels begin to increase (10.3 to 11.1mg/dl). Her vitamin D level was 21.9 ng/ml and PTH 17.8 pg/ml, both were in the normal ranges. The Drug Interaction Probability Scale score of our case was calculated as 6, indicating that the probable adverse drug reaction. Therefore, the high level of serum calcium was attributed to the interaction between ATRA and posaconazole. Management & Outcome Although hypercalcemia with ATRA and other antifungal agents have been previously reported in the literature, this is the first report of hypercalcemia with the concomitant use of ATRA and posaconazole. Discussion This case highlights the importance of monitoring ATRA’s side effects when it is used in combination with drugs inhibiting the cytochrome P450 enzymes. In conclusion, the concomitant use of posaconazole and ATRA may lead to hypercalcemia and serum calcium levels return to normal ranges with the discontinuation of these drugs.

Introduction Imatinib is generally well tolerated by patients. The most common ophthalmic side effects are eyelid edema and periorbital edema. Other side effects which occur at rates of Case report A 51-year-old male with leukocytosis detected in the blood test examination was referred to the Hematology Department. The bone marrow biopsy result was compatible with chronic myeloid leukemia. Imatinib treatment (400 mg/day) was started. In the ninth month of imatinib treatment, the patient complained of a sudden decrease in vision. Vitreous hemorrhage was detected in the left eye and the patient underwent surgery. Vitreous hemorrhage recurred 1 month after the operation. On the fourth day after the discontinuation of imatinib treatment, the patient's ophthalmic complaints improved significantly. The Naranjo algorithm was applied and a score of 9 was detected. The vitreous hemorrhage of the patient was attributed to imatinib, and so the treatment of the patient was switched to bosutinib. Discussion Imatinib is an oral signal inhibitor that targets tyrosine kinase for BCR/ABL, platelet-derived growth factor, stem cell factor, and c-kit (CD117). The conjunctiva and sclera have a large amount of c-kit positive mast cells which are inhibited by imatinib. The inhibition of c-kit positive mast cells by imatinib may be responsible for further exposure of the conjunctival mucosa to injuries.

Case report In this article, we wanted to present our case in which we detected SMZL during examining for defects in coagulation tests and correlated the PT and aPTT elevation with the development of inhibitors against coagulation factors related to this disease. The PT and aPTT values of the patient diagnosed with MZL did not improve in the mixing test, and no other etiology was found. With the second course of chemotherapy, the patient's values improved.

Case report We wanted to present our patient who was diagnosed with NK/T cell type PTLD after kidney transplantation, to contribute to the literature. Posttransplant lymphoma in NK cell phenotype (EBV unrelated) was detected in biopsy taken from the lesions that developed in mouth 11 years after kidney transplantation. It was detected as stage 1E with the examinations. As a result, early recognition of such rare cases and start treatment and reducing immunosuppressive agents are important

Objective Congenital dyserythropoietic anemia is a group of diseases characterized by ineffective erythropoiesis and multinuclear erythroblasts, mostly diagnosed in childhood. Although there are 3 main types, type II is the most common. We present our patient with congenital dyserythropoietic anemia, who was not diagnosed until the age of 49, to contribute to the literature. Case report A 49-year-old male patient was admitted to our hospital with abdominal pain, weakness and yellowing of the eyes. His examinations revealed splenomegaly, cholelithiasis, anemia and hyperbilirubinemia. In the patient's anamnesis, he stated that he had jaundice and weakness since childhood, and that he knew that he had abdominal pain and spleen enlargement with advancing age. Methodology Bone marrow biopsy was performed to the patient for a different diagnosis and cause. Binuclear erythrobasts were observed in the patient (fig. 1). As a result of the new generation sequencing performed on the patient who was evaluated as familial non-immune hemolytic anemia, c.1733TgC homozygous mutation in exon 15 of the SEC23B gene was detected and a diagnosis of congenital dyserythropetic anemia type II was made Results Congenital dyserythropoietic anemias (CDA) represent a large group of diseases that mainly result in ineffective erythropoiesis. Morphological changes observed in the bone marrow over a long period of time were its main diagnostic features. Together with 3 main subtypes, they are examined in a total of 5 subtypes. CDA type II is most common. Clinically normal or slightly increased reticulocyte count is characterized by a variable degree of normocytic anemia. Conclusion Diagnosing CDA cases: It is closely related to the clinician's ability to remember and access genetic tests, especially in advanced ages. Considering that access to genetic tests will increase in the future, many undiagnosed cases may come up. Although our treatment possibilities are limited in the current situation, future treatment methods are promising. However, studies are still needed to understand this disease and its mechanisms.

Case report Pellagra is a systemic disease caused by a deficiency of vitamin B3 .A 19-year-old male patient, who was diagnosed with Burkitt's lymphoma was admitted to the hematology clinic for the second cycle of R-CODOX-M chemotherapy treatment. The patient at risk of malnutrition developed dermatit,diare and demans during treatment. The cause of diarrhea in the neutropenic patient is mostly in the form of infective diarrhea. Diarrhea due to vitamin deficiency should be kept in patients with malnutrition .

Objective Therapeutic plasma exchange (TPE), is based on the removal of pathogenic substrates from plasma with replacement fluid. TPE is being used in the treatment of many neurological diseases, especially Myasthenia Gravis (MG) and Guillain Barre Syndrome (GBS). The aim of this study is to analyse the efficay and safety of TPE experience in neurological disorders. Methodology We reviewed the medical records of all 59 patients who received a total of 267 therapeutic cycles between 2012 and 2021 in our tertiary care university hospital. Respond assesment was evaluated with Medical Research Council (MRC) scoring system. Neutrophil count, lymphocyte count and neutrophil/lymphocyte ratio was recorded before any treatment and 7 days after the last plasmapheresis cycle. Results Of the 59 patients, 30 (50.8%) were male and 29 (49.2%) were female. Of these patients 44.1% were diagnosed with MG, 27.3% with GBS, %8.5 with Multiple Sclerosis (MS). The median number of TPE sessions per patient was 5 [1-7]. 33.9 % of patients had at least one complication that hypotension was the most seen (%22). Overall response rate was %76.3. MRC score was significantly higher in the group with response than the group without symptom regression (p l0.05). Conclusion TPE is a safe and an effective treatment option in neurological diseases. TPE related side effects/complications were generally mild to moderate and manageable. Performing the TPE response evaluation with the MRC scoring system was beneficial for the reliability of the efficacy as a concrete finding.

Objective Autologous hematopoetic stem cell transplantation (aHSCT) after high dose chemotherapy is a standard treatment for multiple myeloma (MM) patients. The successful aHSCT depends on collection of sufficient numbers of hematopoietic progenitor stem cells and sustained engraftment following infusion. The aim of the present study is to determine the the impact of bone marrow fibrosis (BMF) on the clinical outcomes of MM patients who underwent aHSCT. Methodology Retrospectively, bone marrow trephine biopsy analyzed in 73 MM patients who were treated with hematopoietic stem cell transplantation (aHSCT) following bortezomib based induction regimen. The BM biopsy samples of all patients were re-evaluated by a single pathologists The patients divided into 4 groups according to fibrosis degree and the correlations in initial characteristic features, therapeutic response, survival, mobilization and engraftment outcomes were reviewed between the groups. Results Comparative analyses revealed that the median apheresis number was found statistically different according to groups (p=0.04). No significance was detected between the fibrozis grade and the number of peripheral blood CD34+ cell collection results and recovery time of neutrophils and platelets. Overall survival and progression free survival were found similar in groups, however relapse of disease was statistically different in patients with fibrosis (p=0.01). Conclusion After induction treatment, a regression was observed in fibrosis grade of patients who had fibrosis at the time of diagnosis. Therefore we suggest to evaluate fibrosis status in all MM patients during each histopathological examination. Difficulties may be experienced during stem cell collection in transplant eligible MM patients with fibrosis at diagnosis. Therefore, we recommend that clinicians should be more careful in these patients during the induction treatment and stem cell mobilization.

Case report Ionizing radiation and chemotherapeutic agents can cause carcinogenic effects by causing DNA damage.A 40-year-old female patient diagnosed with thyroid papillary carcinoma in 2016 and subsequently administered 150 mCi radioiodine (I¹³¹). Leukocytosis was detected in the examinations performed due to urinary system infection. She was diagnosed t(9:22) p210 positive AML M1-2. The patient had a history of Stargardt Syndrome.Development of t-AML after radioiodine treatment is very rare.

Case report One of the causes of autoimmune hemolytic anemia is drugs. Vaccination is the most important step in the management of the COVID-19 pandemic. After receiving the m-RNA COVID-19 (Pfizer-BioNTech) vaccine, the patient admitted with weakness and jaundice for the last three days. Laboratory results are consistent with AIHA recurrence. Splenectomy was performed after the patient stabilized with rituximab therapy. Especially newly developed therapeutic agents have a potential risk of new side effects.

Objective Although plasma cell neoplasms occupy a large place in hematology practice, POEMS syndrome is very rare. Serum lambda light chain elevation and polyneuropathy, together with organomegaly, endocrinopathy, and skin lesions are the main components of the syndrome. We share our case, which we diagnosed in our clinic, with the belief that it will contribute to the literature. Case report A 51-year-old male patient, who had no history of co-morbidity, drug use, or exposure to toxic substances, was started on supportive treatment in February 2021, who first developed the complaint of impotence. Later, he applied to the neurology outpatient clinic with complaints of weakness and weakness in the feet. After detecting polyneuropathy in his evaluation, IgG Lambda monoclonal gammopathy was detected in serum immune electrophoresis in his evaluation for etiology. Methodology Thereupon, it started to be investigated in terms of plasma cell neoplasms. In the examinations performed, immunoglobulin levels, serum-urine kappa and lambda light chain levels, plasma increase in the bone marrow biopsies and a solitary 3.3 cm sclerotic lesion in the sacral region were detected in the PET-CT of the patient, whose ethology could not be diagnosed. Results A tru-cut biopsy was taken from the sclerotic lesion of the patient, who was thought to be a plasmacytoma and a 20% monoclonal IgG lambda plasma increase was detected. In his physical examination, it was seen that he had increased lesions (Figure-1) and acrocyanosis (Figure-2) on the skin for the last 3-4 months. The patient's current complaints and laboratory results were evaluated with a preliminary diagnosis of POEMS syndrome (Table-1). Conclusion POEMS syndrome is a rare disease and its exact incidence is unknown. It is frequently seen in 5-6 decades, with a median age of 51 years, and 63% of cases are male patients [1]. Chronic and excessive production of proinflammatory and other cytokines (IL-1β, TNFα, IL-6, vascular endothelial growth factor (VEGF) etc.), microangiopathy, edema, effusions, increase in vascular permeability, increase in neovascularization are important in the pathophysiology of the disease.

Case report: Five types of gene variants are seen in hereditary spherocytosis (ANK, SPTB, SPTA1, SLC4A1, EPB42). JAK2 V617F mutation; is most common seen in bcr-abl negative chronic myeloproliferative diseases. As a result of NGS performed before splenectomy, SPTB c.4973+2T> C and JAK-2 c.1849G>T p.(Val617Phe) mutations were detected. Co-occurrence of these two mutations requires special attention in terms of the management of thrombocytosis and side effects that may occur after splenectomy.

GirişBu enfeksiyonun seyrini etkileyebilecek öngörücü parametreler, COVID-19 (Koronavirüs hastalığı 2019) pandemisinin başlangıcından bu yana ana araştırma konusu olmuştur. Kan gruplarının keşfinden bu yana bunların bulaşıcı hastalıklar üzerindeki etkisi her zaman ilgi çekici olmuştur.Araç ve yöntemlerCOVID-19 hastalarında ABO kan grubunun mortalite, hastanede kalış süresi ve hematolojik ve sitokin fırtınası parametreleri üzerindeki etkisini analiz etmek.Bu retrospektif çalışma, COVID-19 tanısı konan 140 hasta üzerinde yapıldı. Demografik özellikler, ABO kan grubunu içeren laboratuvar parametreleri, tam kan sayımı (CBC) parametreleri, biyokimyasal testler, sitokin fırtınası parametreleri, hastanede kalış süresi ve son durum (taburculuk veya ölüm) kaydedildi.Bulgular: Analize dahil edilen 140 hastanın 72’si (%51.4) erkek ve 68’i (%48.6) kadından oluşmakta olup, ortalama yaşları 66.3±14.0 yıldır. Yaş ve cinsiyet, hastanede kalış süresi ve ölüm oranları tüm kan grubu gruplarında benzerdi. Sadece D-dimer değerleri A kan grubunda diğer kan gruplarına göre daha yüksek bulundu.Tartışma: Gruplar arasında mortalite açısından fark saptanmamakla birlikte A kan grubuna sahip COVID-19 hastalarında D-dimer düzeyi istatistiksel olarak anlamlı derecede yüksekti. D-dimer düzeylerini enfeksiyonun klinik seyrine ve dolayısıyla günlük uygulamaya yansıtmak için daha büyük çalışmalara ihtiyaç vardır., Introduction Predictive parameters that can affect the course of this infection have been the main topic of research since the beginning of the COVID-19 (Coronavirus disease 2019) pandemic. Since the discovery of blood groups, the effect of these on infectious diseases has always been of interest.Objectives To analyze the effect of ABO blood group on mortality, hospitalization duration and hematological and cytokine storm parameters in patients with COVID-19. Patients and methods: This retrospective study was conducted on 140 patients diagnosed with COVID-19. Demographic characteristics, laboratory parameters including ABO blood group, complete blood count (CBC) parameters, biochemical tests, cytokine storm parameters, duration of hospitalization, and final status (discharge or death) were recorded.Results: The 140 patients included in the analysis comprised 72 (51.4%) males and 68 (48.6%) females with a mean age of 66.3±14.0 years. . Age and gender, hospitalization duration and mortality rates were similar in all blood group types. Only D-dimer levels were found to be higher in blood group A compared with other blood groups.Conclusion: Although no difference in mortality was determined between groups, the D-dimer level was statistically significantly higher in COVID-19 patients with A blood group. Larger studies are needed to reflect D-dimer levels on the clinical course of infection, and thus on daily practice.

Introduction Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. Herein, we aimed to report a diffuse large B-cell lymphoma (DLBCL) case that was presented as HLH. Case report A 32-year-old man presented to a hospital with complaints of vomiting, nausea and diarrhea in October 2019. Fever and hepatosplenomegaly was detected in physical investigation. Bone marrow aspiration investigation revealed the hemaphagocytosis. HLH-2004 protocol was started for hemophagocytosis. Whole body magnetic resonance imaging (MR) revealed no lymphadenopathy. Bone marrow biopsy revealed high-grade B-cell lymphoma, favoring DLBCL. There were no pathologic cells in lumber puncture investigation. Management and outcome He was diagnosed with secondary hemaphagocytic syndrome due to DLBCL, and chemotherapy was switched to rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (R-EPOCH) regimen. After three cycles of R-EPOCH chemotherapy regimen, complete remission was confirmed with positron emission tomography-computerised tomography (PET-CT) scan. Discussion Our patients’ findings are suitable for six out of eight criteria of hemaphagocytic syndrome. The H-score of our patient was more than 250, reflecting the >99% probability of HLH syndrome. Compatible with literature knowledge, our patient had responded very well to etoposide-containing regimens. In our patient, no lymphadenopathy was detected by physical examination or MR scan, and the diagnosis of DLBCL was only made by the result of bone marrow investigation. In conclusion, herein, we have reported a DLBCL case that had presented with HLH, and clinicians should be aware that B-cell lymphomas may be the underlying cause of HLH.

Myeloproliferative neoplasms (MPNs) are diseases of myeloid series usually with an increase in peripheral blood cells. Among this disease group, only chronic myeloid leukemia (CML) has t(9; 22) (Philadelphia chromosome) leading to the production of BCR-ABL1 fusion gene with tyrosine kinase activity. The main distinction between the diagnosis and treatment of this heterogeneous group is the presence of BCR-ABL1 or JAK-2 V617F mutation. The coexistence of these two mutations may be observed, although not frequently, and can lead to delay in the diagnosis. The case here presents the detection and clinical management of JAK-2 V617F mutation after the suspicion of other MPNs due to fluctuations in clinical and hematological responses of this rare entity in a 78-year-old female patient with CML. To the best of our knowledge, this is the first CML case with coexistence of BCR-ABL1 translocation and JAK2 V617F mutation from Turkey.

Low-grade Nonhodgkin lymphoma (LG-NHL) is characterized by indolent clinical course, which consist of marginal zone lymphoma (MZL), follicular lymphoma (FL), chronic lymphocytic leukemia/small lymphocytic lymphoma, lymphoplasmacytic lymphoma, waldenstrom macroglobulinemia (WM) as the most common subtypes. Factors affecting prognosis and treatment need in these patients have long been the subject of research. A retrospective study was conducted with patients diagnosed with LG-NHL in Hematology Departments of two centres between 2010 and 2018. At the time of diagnosis, demographic and disease characteristics, hematological and biochemical parameters were examined. Using these data, treatment requirements, response and survival rates were calculated. The effect of parameters on survival and need to treatment were analyzed. 93 LG-NHL patients were included in this study. 40 (43%) of these patients were MZL, 28 (30.1%) were FL and 25 (26.8%) were others. In comparison of patients required treatment with patients without treatment, there was significant difference among the number of comorbidity, platelet count, neutrophil count, disease subgroups and ferritin levels. Logistic regression analysis revealed that disease subgroup (other than MZL and FL) and ferritin levels were independent risk factors for need to treatment. Only ferritin level was found to be associated with overall survival. The current study demonstrated an association between serum ferritin levels and prognosis in patients with LG-NHL. Given that it is easily available and low-cost, the initial ferritin level can be used as a prognostic marker for patients with indolent lymphoma.

BackgroundPatients with cancer are at increased risk of thromboembolic complications. There is no evidence-based guideline on the use of routine prophylaxis in hematological malignancies except in patients with multiple myeloma. The purpose of this study was to determine the incidence and risk factors of thrombosis and suggest a rationale for primary thromboprophylaxis in acute leukemia and lymphoma patients.Patients and methodsA retrospective study was conducted on newly-diagnosed acute leukemia and lymphoma patients who presented at our institution from November 2009 to March 2018. The study included a total of 157 patients with acute leukemia and 238 patients with lymphoma. The groups were analyzed to determine the incidence and risk factors of thromboembolic complications.ResultsThe incidence of all thrombotic complications was 10.12% (40/395) including 11.4% (18/157) in patients with acute leukemia and 9.2% (22/238) in patients with lymphoma. The majority of events occurred in the first 6 months. Acute leukemia patients with thrombosis had a higher number of comorbidities than those without thrombosis ( p 3).ConclusionsAcute leukemia patients with any comorbidity and lymphoma patients with higher lactate dehydrogenase and beta-2-microglobulin are at high risk of developing thromboembolic complications. The prophylactic use of anticoagulant should be considered for those patients especially in the first 6 months.

Karakus, Volkan/0000-0001-9178-2850; Demirkan, Fatih/0000-0002-1172-8668; Demircioglu, Sinan/0000-0003-1277-5105; Arat, Mutlu/0000-0003-2039-8557; Cagliyan, Gulsum Akgun/0000-0002-2073-1949; Ozturk, Hacer Berna Afacan/0000-0001-9386-7604; WOS: 000473249400014 PubMed: 31036516 Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

Sarcoidosis is known to be associated with higher incidence of solid tumors and hematological malignancies. ALK(−) CD30(+) anaplastic large cell lymphoma is a type of non-Hodgkin lymphoma showing poor prognosis, and seldom co-occurs with sarcoidosis. As this rare and highly mortal disease did not respond to classical chemotherapies and showed remission with brentuxumab vedontin treatment, we are presenting our first case reported from Turkey hoping to contribute to the literature.

Objective: Full blood count with the associated components is a widely used marker in the diagnosis of patients with diffuse large B cell lymphoma DLBCL as it is simple and inexpensive and can be easily interpreted. The aim of the current study was to evaluate the prognostic role of complete blood count parameters with the associated components in patients with DLBCL.Material and Methods: In this retrospective study, the neutrophil/lymphocyte ratio NLR , lymphocyte/monocyte ratio LMR , and platelet/lymphocyte ratio PLR were evaluated and an analysis was made of the progression-free survival PFS and overall survival OS in patients who were diagnosed with DLBCL and treated with a total of 6 cycles of R-CHOP chemotherapy in the 2011-2017 period.Results: 66 patients were included in the study. In the Cox-Regression Model, high NLR and the low LMR were associated with short OS and PFS; high PLR was associated with short PSF.Conclusion: Pre-treatment NLR, LMR, and PLR values can be used as prognostic factors in DLBCL patients, Amaç: Diffüz büyük b hücreli lenfoma DBBHL tanısı alan hastalarda tam kan sayımı ve ilişkili bileşenleri, prognozun belirlenmesinde basit, ucuz, kolaylıkla yorumlanabilen ve yaygın olarak kullanılan markerlardan biridir. Çalışmanın amacı, DBBHL hastalarında tam kan sayımı parametreleri ile ilişkili bileşenlerin prognostik rolünü değerlendirmektir. Gereç ve Yöntemler: Retrospektif olarak 2011-2017 yıllarında DBBHL tanısı almış ve toplam 6 kür R-CHOP kemoterapisi ile tedavi edilmiş hastaların tanıdaki nötrofil/lenfosit oranına NLR , lenfosit-monosit oranına LMR ve Platelet-lenfosit oranına PLR bakıldı ve progresyonsuz sağkalım PFS ve genel sağkalım OS analizi yapıldı. Bulgular: Çalışmaya 66 hasta dahil edildi. Cox-Regresyon Modelinde, yüksek NLR ve düşük LMR kısa OS ve PFS ile ilişkiliydi; yüksek PLR, kısa PSF ile ilişkiliydi.Sonuç: Çalışmamızın sonucunda tedavi öncesi NLR, LMR, PLR değerlerinin DBBHL hastalarında bağımsız prognostik faktör olarak kullanılabileceğini gösterir

Case report: Bone involvement is rare in DLBCL. 70-yearold patient, applied to the orthopedics clinic dueto knee pain. Kneeprosthesis was planned. During operation suspicious nontumoral lesion with unclear borders was observed. Bone biopsy was taken from the intraoperatively detected lesion and a knee prosthesis was placed. According to PETCT and bonemarrow biopsy results, patient was diagnosed as stage 1E. Awareness of DLBCL with atypical presentation are of great importance in terms of early diagnosis

Objective T-cell prolymphocytic leukemia (T-PLL) is a rare and highly aggressive T cell neoplasm with rapidly progressing clinical course. T-PLL accounts for 2% of mature lymphocytic leukemia in adults. Median overall survival with modern therapy is reported one to three years. Here we report a T-PLL patient with peritoneum involvement and progressive ascite despite anti-CD52 treatment. Case report A 65-year-old man with diabetes mellitus was admitted to hospital due to fatigue for a few weeks. Laboratory workup revealed that white blood cell count 469 × 103/µl (90% lymphocytes), haemoglobin of 11.4 g/dl, platelets of 104 × 103/µl. Medium sized atypical lymphoid cells with partial chromatin condensation and a visible nucleolus were observed on blood smear. Methodology On physical examination, palpable inguinal lymph nodes, splenomegaly 3 cm below the rib margin and a palpable lesion on the helix of left ear were noticed. Punch biyopsy of skin lesion was reported as a mature and immature T cell infiltration which are CD3 and CD10 positive and Tdt, CD34, CD20, CD99 negative. Flow cytometric study of peripheral blood sample was revealed that T-Chronic Lymphocytic Leukemia (T-CLL). Results FMC protocol (fludarabine, mitoxantrone, and cyclophosphamide ) was initiated and followed by intravenous alemtuzumab at a dose of 3 mg on day 1, 10 mg on day 2 and 30 mg on day 3. However after two months of anti-CD52 treatment, his general situation dete­riorated and pleural effusion and abdominal distension developed due to massive ascites. Small, mature lymphocytic cell infiltration was shown in ascites fluid on cytological examination. He died after six months of diagnosis. Conclusion T-PLL is a very aggressive disease with a median survival of less than 1 year. Not all patients diagnosed with T-PLL require treatment immediately.Currently, IV alemtuzumab (anti-CD52) is the accepted best avaliable treatment with very high response rates when given as first-line treatment. However, treatment is notcurative and a minority of T-PLL patients experience long-term disease-free survival.

Giriş: Coronavirus hastalığı 2019, şiddetli akut solunum sendromu coronavirüs 2'nin (SARS-CoV-2) sebep olduğu viral bir antitedir. Klinik ve laboratuvar belirleyicileri, mortalite riski altındaki hastaları belirleyebilir ve tedaviye rehberlik edebilir. Bu çalışmanın amacı, COVID-19 hastalarında laboratuvar parametrelerini analiz etmek ve hangi parametrelerin mortalite ve hastaneye yatışı etkilediğini belirlemektir.Araçlar ve Yöntem: Demografik özellikler, tam kan sayımı (CBC) parametrelerini içeren laboratuvar parametreleri, biyokimyasal testler, pıhtılaşma parametreleri, hastanede kalış süresi ve son durum (taburculuk veya ölüm) kaydedildi.Bulgular: Bu retrospektif çalışma, COVID-19 teşhisi konan 101 hasta üzerinde yapıldı. Analize dahil edilen 101 hasta 52(%51.5) erkek ve 49(%48.5) kadından oluşmaktaydı ve ortalama yaşları 65.7±14.7 idi. İzlem süresi sonunda hayatta kalanlar ve hayatta kalmayanlar arasında karşılaştırmalar yapıldı. Çok değişkenli analiz, ortalama trombosit hacmi (MPV), trombosit dağılım genişliği (PDW) ve laktat dehidrogenazın (LDH) mortalitenin önemli belirleyicileri olduğunu gösterdi. Hastanede kalış süresinin cut-off değeri 10 gün olarak saptandı ve hastalar iki gruba ayrıldı. Tek değişkenli ve çok değişkenli modellerde, hastanede yatış süresinin öngörülmesi için anlamlı bağımsız parametre gözlenmedi.Sonuç: Mevcut çalışmanın sonuçları, MPV, PDW ve LDH' nin mortaliteyi öngörmede önemli bağımsız değişkenler olduğunu göstermiştir. SARS-CoV -2 ve SARS-CoV'nin aynı reseptörü kullandığı bilindiğinden, mutant varyantlar ve ilk varyant için benzer bir yapı ve reseptör olabilir, dolayısıyla bu öngörücü parametrelerin mutant varyantlarda da etkili olduğu düşünülebilir., Purpose: Coronavirus disease 2019 is a viral disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Clinical and laboratory predictors may provide identification of patients at risk of mortality and guide treatment. This study aims to analyze laboratory parameters in COVID-19 patients and to determine which parameters affect mortality and hospitalization.Materials and Methods: Demographic characteristics, the parameters including complete blood count (CBC) parameters, bio-chemical tests, coagulation parameters, duration of hospitalization, and final status (discharge or death) were recorded in patients diagnosed with COVID-19.Results: This retrospective study was conducted with 101 patients diagnosed with COVID-19. The 101 patients included in the analysis comprised 52(51.5%) males and 49(48.5%) females with a mean age of 65.7±14.7 years. Comparisons were made between survivors and non-survivors at the end of the follow-up period. Multiple analyses showed mean platelet volume (MPV), platelet distribution width (PDW), and lactate dehydrogenase (LDH) to be significant predictors of mortality. The cut-off value of the hospitalization period was found to be 10 days; therefore, the patients were divided into two groups. In the univariate and multiple models, no significant independent parameter was observed for the prediction of hospitalization duration.Conclusion: The results of the current study demonstrated that MPV, PDW and LDH were significant independent variables for the prediction of mortality. As SARS-CoV-2 and SARS-CoV are known to use the same receptor, there may be similar structures and receptors for mutant variants and the first variant, so these predictive parameters can be considered effective in mutant variants.

Introduction Synchronous detection of multiple myeloma and acute myeloid leukemia in a single patient is a rare coincidence. Treatment of these patients is still unclear, mostly based on acute myeloid leukemia strategies combined with bortezomib. Case report A 72-year-old male with no medical history was investigated for pancytopenia. On medical examination, he was complicated with a wide and severe skin infection on arm. On examination of bone marrow aspirate, 25% myeloblasts infiltration and additional 10% plasma cells were seen. Acute myeloid leukemia was diagnosed and plasma cell proliferation was attributed to reactive plasmacytosis due to skin infection. However, flowcytometric studies and immunohistochemical examination revealed two different cell populations with 30–40% atypical plasma cells and >20% myeloblasts. Serum M-protein detected by serum electrophoresis test and immunofixation test revealed a monoclonal IgG lambda band. He was diagnosed with concurrent acute myeloid leukemia and multiple myeloma without history of chemotherapy. Management and outcome: The patient was initially treated with bortezomib and dexamethasone for the myeloma. Subsequently, azacitidine was administered subcutaneously for the acute myeloid leukemia treatment. The tru-cut biopsy of the lesion on his arm revealed suppurative inflammatory findings and no malign cells detected. Antibiotherapy was started according to susceptibility. He expired after three months of survival. Discussion The synchronous occurrence of these two different clonal hematological malignancies is rare in hematology practice. Patient-based prospective studies and case series are needed to guide diagnosis and treatment strategies. Furthermore, this report highlights the importance of ruling out reactive plasmacytosis in patients with hematological malignancy who developed severe infections.

We evaluated the safety and efficacy of single-agent ibrutinib in 200 patients presenting with relapsed/refractory CLL in real-world settings. With an estimated median OS of 52 months, 146 patients (75%) achieved at least PR; 16 (8.7%) patients discontinued ibrutinib due to adverse events. The results indicate good safety and efficacy for single-agent ibrutinib in R/R CLL in daily practice. Introduction/Background: The emergence of novel agents targeting the B-cell receptor pathway and BCL-2 has significantly changed the therapeutic landscape of CLL. We evaluated the safety and efficacy of single-agent ibrutinib in relapsed/refractory CLL in real-world settings. Patients/Methods: A total of 200 relapsed/refractory CLL patients with a median age of 68 were included in this retrospective, multicenter, non-interventional study. Data of the study were captured from the patient charts of the par ticipating centers. Results: The median for lines of previous chemotherapy was 2 (1-6); 62 (31.8%) patients had del17p and/or p53 mutations (del17p+ /p53mut). Of the study group, 146 (75%) patients achieved at least PR, while 16 (8.7%) patients discontinued ibrutinib due to TEA. The most common drug-related adverse events were neutropenia (n: 31; 17.4%) and thrombocytopenia (n: 40; 22.3%), which were >= grade 3 in 9 (5%) and 5 (3.9%) patients, respectively. Pneumonia (n: 42; 23.7%) was the most common nonhematologic TEA. Atr ial fibrillation (n: 5; 2.8%) and bleeding (n: 11; 6.3%) were relatively rare dur ing the study period. Within a median follow-up period of 17 (1-74) months, 42 (21%) patients died. The estimated median OS of the study cohort was 52 months. Only the response to ibrutinib (CR/PR vs. SD/PD) was significantly associated with OS. Conclusion: Our results indicate good safety and efficacy for single-agent ibrutinib in R/R CLL in daily practice. (C) 2021 Elsevier Inc. All rights reserved.

Microangiopathic hemolytic anemia (MAHA) can be observed as a paraneoplastic syndrome (PS) in certain tumors. MAHA-related signet ring cell carcinoma (SRCC) of an unknown origin is very infrequent. Herein we present a SRCC case presented with refractory acquired thrombotic thrombocytopenic purpura (TTP). A 35-year-old man applied to the emergency service with fatigue and headache. His laboratory tests resulted as white blood cell 9,020/µL, hemoglobin 3.5 g/dL, platelet 18,000/µL. Schistocytes, micro-spherocytes, and thrombocytopenia were observed in his blood smear. MAHA was present and he was considered as having TTP. Plasma exchange treatment was initiated; however, he was refractory to this treatment. Thorax and abdomen computerized tomography revealed thickening of minor curvature in stomach corpus with hepatogastric and paraceliac lymphadenopathy. Bone marrow (BM) investigation by our clinic resulted as the metastasis of adenocarcinoma. Ulceration and necrosis were observed by gastric endoscopy procedure. Biopsy was taken during endoscopic intervention, which resulted as SRCC. MAHA may be seen as a PS in some tumors, especially gastric cancers. Tumor-related MAHA is generally accompanied by BM metastases. As a result, BM investigation may be used as the main diagnostic method to find the underlying cancer. The clinical course of cases with tumor-related MAHA is usually poor, and these cases are usually refractory to plasma exchange treatment. In conclusion, physicians should suspect a malignancy and BM involvement when faced with a case of refractory TTP.

A 20-month-old boy presented with a double right thumb. His parents reported no health complaints other than the skeletal disorder involving his right hand. He is the first child born to non-consanguineous healthy parents. His mother is 24 years old. There is no family history of any kind of