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Introduction:Mucormycosis is an aggressive-progressive invasive fungal infection caused by mold fungi in the division of mucorales of the zygomycetes class with high mortality, and is the most common fungal infection in patients with hematologic malignancies.Methods:This study retrospectively evaluated patients with mucormycosis diagnosis between January 2015 and December 2019, including demographic features, hematologic diseases and comorbidities, radiological evaluations, symptoms and signs, treatments, and outcomes.Results:Maxillofacial 9/19 (47.37%) of patients and 10/19 (59.9%) rhinoorbital mucor. Hematologic malignancy was observed in 15 (78.95%) patients, whereas others had additional pre-disposing factors, such as diabetes mellitus and chronic renal failure. The most common find-ings were persistent fever, mucopurulent nasal flux, and periorbital edema. Endoscopic sinus surgery + medication was administered in 12/19 (62.2%) patients and antifungal therapy in 7/19 (37.8%). In addition, 15/19 (79.95%) patients died and 4/19 recovered with sequela.Conclusion:The first large-scale mucormycosis study from our country will guide in determining the treatment algorithm. Effective and early surgery and antifungal application reduce mortality in mucormycosis by early diagnosis and multidisciplinary approach, without bone destruction in the paranasal sinus computed tomography with recurrent fever and earlystage sinusitis finding by performing a biopsy.

Introduction:Chronic myeloid leukaemia (CML) is a myeloproliferative neoplasm characterised by the overproduction of haematopoietic cells in the granulocytic series, involving translocation of chromosomes 9 and 22. The first tyrosine kinase inhibitor, imatinib, used in the treatment of CML, represents one of the most successful targeted therapies marking a new era in the treatment of CML. Many scoring systems such as the Hasford and Sokal systems have been developed to stratify CML patients into risk categories, and to predict patient outcome. We aimed to evaluate the relationship between blood lymphocyte count (BLC) at diagnosis and molecular response to imatinib therapy as a prognostic factor.Methods:A total of 108 chronic phase CML patients diagnosed between January 2010 and January 2020 were evaluated. Patient characteristics, laboratory results, BLC and response to treatment were recorded.Results:The median BLC was 4,665/mm3 and patients were divided into two groups according to the median BLC as ≤4,665/mm³ and >4,665/mm³. The responses at 3, 6, 12 month and the final status of patients, namely achievement of major molecular response or not, did not differ between the two groups of patients.Conclusion:The introduction of new therapeutic options in CML necessitates improvement in existing risk scoring systems. No direct relationship was found between the initial BLC and imatinib response in CML. However this is the first study exploring the role of BLC at diagnosis in CML patients receiving imatinib, and further studies that look into lymphocyte subgroups as well as bone marrow lymphocyte count at diagnosis might allow a more precise evaluation about the contribution of lymphocyte count to risk assessment in CML patients.

Abstract Background Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma and may occur with lymph node and/or extranodal involvement. Recurrence in patients with diffuse large B-cell lymphoma usually occurs within the first few years after treatment and may occur in a different area outside the initial localization. Case presentation A female Turkish patient who was diagnosed with nodular sclerosing Hodgkin lymphoma through lymphadenopathy examination reached remission after chemotherapy and radiotherapy. In the 11th year of follow-up and at the age of 45, newly developed multiple lymphadenopathies were diagnosed with a pathological result of diffuse large B-cell lymphoma in her advanced examination. Due to massive splenomegaly and cystic necrotic splenic residues, splenectomy was performed after eight cycles of a first-line chemotherapy regimen and two cycles of high-dose methotrexate treatment for central nervous system prophylaxis. A pericardial mass (maximum standardized uptake value 34.8), which was not present at the time of diagnosis and interim evaluation of positron emission tomography/computed tomography, was detected through chest pain in the third month after the last screening, although a complete response had been obtained. Pathological examination of the pericardial area revealed the pathological result was a recurrence. Conclusions Patients with diffuse large B-cell lymphoma have an aggressive clinical course, but cardiac involvement is very rare. In our patient’s case, pericardial involvement was observed after treatment and scanning revealed that recurrence took place in an area different from the pericardium. Cooperation of clinicians and pathologists and rapid evaluation are very important in cases of diffuse large B-cell lymphoma relapse. Although a tumoral invasion of the pericardium mostly suggests secondary malignancies, it should be kept in mind that recurrence of lymphoma is also possible.

Objective: We present data of patients with relapsed/ refractory T cell lymphomas treated with brentuximab vedotin (BV) in real-world practice. Material and Method: This study is an observational, multi-center, retrospective study. The data of patients (n=17) treated with BV alone from January 2014 until July 2020 in thirteen centers from Turkey were collected. Results: Bv was given as salvage chemotherapy to 17 patients with median age of 53. Nine (52.9%) patients had diagnosis of peripheral T cell lymphoma, not otherwise specified; 8 (47.1%) patients had anaplastic large T cell lymphoma. The median follow-up of the cohort was 20 months. Nine (52.9%) patients had complete response, 5 (29.5%) had partial response, 3 (17.6%) had progressive disease. The safety results aligned with the established profile of BV, included 2 pneumonia and 1 thrombocytopenia with grade 4. The median progression free survival of the cohort was 10 months. BV cycle and response to BV therapy were found to have an effect on the univariate analysis. Conclusion: In patients with relapsed/ refractory T cell lymphomas, BV seems to have convincing antitumor activity with favorable safety profile.

Introduction: This study aimed to evaluate the results obtained with the preference of eltrombopag according to the line of treatment in patients diagnosed with ITP. Methods: This retrospective study included 51 patients who were treated with eltrombopag for chronic ITP at 3 different centers. Diagnosis of ITP was based on the current literature. Results: Thirty patients (58.8%) received eltrombopag as second-line treatment, 16 patients (31.4%) as third-line, 3 patients (5.9%) as fourth-line treatment, and 2 patients (3.9%) as fifth-line treatment. Twenty-four out of 30 patients (80%) who received eltrombopag as second-line therapy and 12 out of 16 patients who received eltrombopag as third-line therapy demonstrated durable response with no further treatment requirements. Eltrombopag treatment in the second or third line did not affect treatment outcomes (p=0.72). Conclusion: Eltrombopag in the second or third line of treatment did not have a significant effect on treatment response. The earlier line choice of eltrombopag, duration, and possibility of sustained response should be taken into consideration. The lack of a significant relationship between the line of treatment and response to eltrombopag as a TPO-RA should be considered encouraging in terms of long-term follow-up.

Introduction: Renal artery stenosis (RAS) is a clinical picture that is evaluated as a renovascular disorder and includes many diseases in its etiology. The primary purpose of the present study was to reveal the etiology of cases followed up in the nephrology outpatient clinic due to RAS; to describe their demographic, clinical, and radiological features; and investigate their treatment and prognosis. The second aim of the study was to investigate the relationship between isolated RAS cases that cannot be attributed to any etiological cause and Takayasu’s arteritis. Methods: Patients were included in retrospectively examining all patient files that were registered between January 1996 and 2018. Demographic data of the patients, date of diagnosis, initial physical examination findings, comorbid diseases, imaging findings, interventional and medical treatments, need for kidney replacement therapy, and time to hemodialysis/transplantation were recorded. Results: Out of the 17427 (8800 M/8627 F) patients, a total of 134 (70 M/64 F) patients aged over 18 years with RAS were included in the study; 60 (55%) patients had atherosclerotic RAS, whereas 23 (21.1%) patients were diagnosed with vasculitis. In total, 16 patients (13.5%) died with a mean of 6±3.4 years after admission. It was found that advanced age, low GFR at diagnosis, and small kidneys are significant and poor prognostic factors. Conclusion: A more systematic approach model can be developed in terms of applying “maximal medical treatment”, which is our approach that we can currently describe as incomplete in cases of RAS with an early atherosclerotic process.

Introduction: Leptin (LEP) and LEP receptor (LEPR) play roles in cancer progression. We evaluated LEP-2548G/A and LEPR 668 A/G variants in patients with multiple myeloma (MM). In addition, the methylation status of CpG sites at 31 and 51 nucleotides (nt) according to the transcription start region of the LEPgene was examined. Methods: DNA was extracted from the peripheral blood of study participants who were healthy controls and patients with MM. These variants were analyzed using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. The methylation at -31 and -51 nt in the LEPwas performed using the methylation-specific PCR method. The 2-year progression-free survival (PFS) and 2-year overall survival (OS) were evaluated according to prognostic factors. Results: There was no significant difference in the genotype distributions of LEPR 668A/G and LEP-2548G/A between the control and patient groups (p>0.05). We found that -31 and -51 nt regions of the LEP gene were unmethylated in the patient group compared with the control group (p=0.051 and p=0.001, respectively). The -31 nt methylation was unchanged in 15 patients (78.94%). PFS and OS were higher in these patients than in the others. In multivariate analysis, the methylated/unmethylated ratio at -31 nt methylation was associated with a poor prognosis (p=0.020). Conclusion: To our knowledge, our study is the first to examine these variants and their methylation status in Turkish patients with MM. Our results showed that LEP gene -31 nt unmethylation was associated with PFS and OS. These results need to be confirmed in different ethnic and larger sample groups.

Objective: Hepatitis C virus (HCV) infection still maintains its importance since it is one of the most important causes of liver cirrhosis and hepatocellular carcinoma. Our hospital, located in İstanbul, which is the 10t most crowded city in the world, has a patient cohort where epidemiological change can be observed due to its deep-rooted history and serving people of different nations in terms of settlement. Main aim in this study is to evaluate the change in HCV epidemiology in our country over the years. Methods: Patients who were at the age of 18 and above and whose HCV-RNA was positive between January 2001 and January 2021 were evaluated. Results: 1.166 patients whose HCV genotype was determined were evaluated. The mean age of the population is 52±14.75 years, 83.53% of all patients was infected with genotype 1 (GT1), 8.23% with GT3, 5.83% with GT2, 2.23% with GT4 and 0.17% of them with GT5. While the GT1 rate decreased in patients over the years, an increase was found in other GTs. GT1 and GT2 were more common in females (p

Introduction:Ruxolitinib is a small -molecule inhibitor of the JAK1/2 pathway. This study aimed to reveal the results and side-effect profile of the use of ruxolitinib as a treatment option in polycythemia vera (PV).Methods:A total of 34 patients with PV from 18 different centers were included in the study. The evaluation of the response under treatment with ruxolitinib was determined as a reduction in spleen volume (splenomegaly size: ≥35%) by imaging and control of hematocrit levels (≤45%) compared to baseline.Results:While the number of patients in which a reduction in spleen volume and hematocrit control was achieved was 19 (55.9%) at 3 months of treatment, it was 21 (61.8%) at 6 months. Additionally, while the number of side effects was negatively correlated with the reduction in spleen volume (Spearman’s rho: -0.365, p=0.034), a decrease in the hematocrit level was positively correlated (Spearman’s rho: 0.75, p=0.029). Those without a reduction in spleen volume experienced more constipation (chi-square: 5.988, Fisher’s exact test: p=0.033).Conclusion:This study shed light on the use of ruxolitinib in PV and the importance of splenomegaly on studies planned with larger patient groups.

Abstract Objective Studies on the genetic background of patients with multiple myeloma (MM) have been increasing; two important factors considered in such works are uncoupling protein-2 (UCP-2) and nuclear receptor subfamily 3 group C member 1 (NR3C1). We aim to reveal the association of MM with NR3C1 and UCP-2 gene polymorphisms. In this prospective study, 200 patients diagnosed between January 2009 and 2018 and 200 healthy individuals were included. For patients who had undergone autologous stem cell transplantation and control subjects, we statistically compared the CC, GC, and GG genotypes and the C and G alleles of the NR3C1 gene, as well as the AA, AG, and GG genotypes and the A and G alleles of the UCP-2 gene. Results While the AA genotype was significantly more common in the MM group (p = 0.001), the GG genotype was significantly more common in the control group (p = 0.016). Overall survival was found to be significantly shorter in patients with the UCP-2 GG genotype (p = 0.034). It was also found that having the GG genotype of the UCP-2 gene was a 2.48-fold risk factor for mortality. The fact that overall survival is significantly shorter in MM patients with the UCP-2 GG genotype and its definition as a risk factor for mortality have been put forward for the first time in the literature.

Introduction:Multiple myeloma (MM) constitutes approximately 10% of hematological malignan-cies with abnormal proliferation of plasma cells, which causes an abnormal increase of monoclonal immunoglobulin light chains. We aim to examine our young myeloma cases’ data and compare light chain disease with other subtypes, which are known to have a worse prognosis in all age groups.Methods:Fifty-five MM patients diagnosed and treated between January 2010 and 2020 under 60 years old, were analyzed retrospectively. Their demographic data, laboratory, treatment subtypes, MM subtypes, CRAB findings (hypercalcemia, renal failure, anemia, and presence of lytic bone lesions), treatment, and responses to the first-line treatment were analyzed. The patients were divided into two groups, the light chain and other myeloma subgroups, and compared statistically.Results:Seventeen patients were female (30.9%), and 38 were male (69.1%). The median age was 54 (range: 34-59) years. The median duration of follow-up was 30 (range: 3-108) months. The MM sub-types examined were: immunoglobulin G (IgG)/Kappa 13 (23.6%), kappa light chain 10 (18.2%), lambda light chain 10 (18.2%), IgG/Lambda 9 (16.4%), IgA/Lambda 8 (14.5%), and IgA/Kappa 5 (9.1%). There was no significant difference between the two groups except for albumin and calcium values. In the other myeloma subtype group, albumin and calcium values were significantly lower (p

Waldenström’s macroglobulinaemia is a disease in the B lymphoproliferative diseases group with immunoglobin M monoclonality and may present with different clinical manifestations. A 52-year-old male patient presented with complaints of weight loss and shortness of breath. Computed tomography (CT) results revealed a mass with a malignant soft tissue density in the abdomen. In the thoracic images, free fluid of up to 130 mm was found between the pleural leaves on both sides. His biopsies from the abdominal mass and bone marrow were reported to be compatible with a lymphoplasmocytic lymphoma in the presence of plasmoid differentiation B cell neoplasia and a Waldenström clinic. Cyclophosphamide -adriamycin -vincristine -prednisolone regimen was used in the first cycle chemotherapy. Positron emission tomography/ CT imaging, which was performed on the 15th day after the second cycle of chemotherapy, showed that the patient’s tumour was stable in size, but metabolic partial regression was observed. Bortezomib-dexamethasone-rituximab regimen was planned because of the resistant chylothorax. The patient had a dramatic clinical response and the chylothorax regressed completely after the first cure treatment. Chylothorax is a rare clinical presentation with chyle in the pleural area. It has traumatic and non-traumatic causes. Non-traumatic causes are most frequently seen due to malignancies. Waldenström’s macroglobulinaemia has a very rare incidence, and the clinical association between Waldenström’s macroglobulinaemia and chylothorax is very interesting. Standard treatment regimens vary, but the response to treatment also varies. Our case was resistant to the first line treatment, but had a dramatic response to the bortezomib and rituximab-based treatment.