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Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). The treatment of older NHL patients has always been a struggle; however, treatment statistics have begun showing favorable results similar to those of younger DLBCL patients thanks to newer treatment protocols. Here, we analyze the progress of our own elderly DLBCL patients who were followed between 2000 and 2016 in our center.Eighty-seven DLBCL patients, who were diagnosed and treated in the Dokuz Eylül University Department of Hematology between 2000 and 2016, were included in this study. Median age was 72 (65-89) years and 13 (14.9%) patients were older than 80 years.Median follow-up time was 19 months and 45 patients (51.7%) died during the follow-up period. Median overall survival (OS) was 55 months and median progression-free survival was calculated as 27 months. Sixty-three patients (72.4%) received standard R-CHOP therapy. Complete response was seen in 46 (52.9%) patients. The median survival time for patients who had complete response was 136 months (p0.001); however, OS was not statistically different between older (80 years) and younger patients (p=0.236).According to our findings, we think that being able to complete standard R-CHOP therapy is vital for the survival rate of elderly DLBCL patients.Diffüz büyük B hücreli lenfoma (DBBHL), Hodgkin dışı lenfomalar (NHL) arasında en sık görülen tiptir. Yaşlı NHL hastalarının tedavisi genellikle zorlu olmakla beraber günümüzde gelişen tedavi protokolleri ile birlikte yaşlı hastalarda da genç DBBHL hastalarına benzer oranda daha iyimser sonuçlar elde edilmektedir. Bu çalışmaya Dokuz Eylül Üniversitesi Hematoloji Anabilim Dalı’nda 2000-2016 yılları arasında takip ve tedavi edilmiş 87 yaşlı DBBHL hastası dahil edildi.Yaş ortalaması 72 (65-89), 80 yaşının üzerinde 13 (%14,9) hasta mevcuttu. Ortalama takip süresi 19 ay olup, tüm takip süresinde hastaların 45’inin (%51,7) öldüğü gözlendi.Ortalama genel sağkalım süresi 55 ay iken progresyonsuz sağkalım süresi 27 ay olarak hesaplandı. Hastaların 63’ünün (%72,4) standart R-CHOP tedavisi almış olduğu görüldü. Ek olarak hastaların 46’sında (%52,9) tam yanıt alındığı gözlendi. Tam yanıt izlenen hastaların ortalama genel sağkalım süresi 136 ay olarak saptandı (p0,001), ancak, 80 yaş üzeri hastalar ile altındaki hastaların genel sağkalım süreleri arasında anlamlı istatistiksel fark izlenmedi (p=0,236).Bu veriler ışığında yaşlı DBBHL’li hastalarda standart R-CHOP tedavisinin tamamlanmasının sağkalım süresine önemli etkisi bulunduğunu söyleyebiliriz.

Introduction: High-dose chemotherapy (HDC) and autologous stem cell transplantation(ASCT) still remains in the treatment of myeloma patients even during the period of new agents. Materials and Methods: We analysed the prognostic affect of pretransplant characteristics and transplant modalities on response, in 150 autologous transplant of 144 multiple myeloma (MM) patients who were transplanted in our centre between 2008 to 2017. We evaluated the affect of age, type of MM, previous treatment regimens, status pre and postfrom transplantation, time of ASCT, neutrophil and platelet engraftmant days, dose of reinfused CD34+ cells, plasma cell infiltration, international staging system(ISS) and Durie -Salmon stage at diagnosis. We examined the affect of these status on overall survival(OS) and eventfree survival(EFS). Results: The median OS and EFS after transplanation were 41 and 28 months, respectively. Median OS after the diagnosis was 57 months. Transplant-related mortality was 3,3%. We found that the lower beta 2- microglobulin levels,lower ISS stage,lower plasma cell infiltration, achievement good responds at the +100th day of post transplant were statistically significant independent predictor factors for longer EFS and OS. When the patients were given chemotherapy regimen with bortezomib before transplantation, these patients were seen to be a better response rate. There was showed a relationship between the using of bortezomib before transplantation with EFS(P = 0.017), but there was no relationship with OS. Conclusions: Our analysis confirms HDCT-ASCT as an effective and safe therapeutic strategy in multiple myeloma patients. This results were independent of age, first line treatment regimens and renal insufficiency. Patients with a high ISS stage were found to have shorter survival(P = 0.002). However, the EFS and OS were longer of the patients whose have good response at the 100th day of transplantation(P = 0,002, P = 0,02).

Objectives Nilotinib is a BCR-ABL1 tyrosine kinase inhibitor approved for the treatment of patients with chronic myeloid leukemia in chronic phase (CML-CP). This study was the first prospective evaluation of the efficacy and safety of nilotinib in Turkish patients with newly diagnosed CML-CP. The primary endpoint of the study was the rate of major molecular response (MMR; BCR-ABL1 ≤ 0.1% on the International Scale [BCR-ABL1IS]) by 12 months. Methods Patients with newly diagnosed CML-CP were treated with nilotinib 300 mg twice daily. This analysis was based on the first 12 months of follow-up in a 24-month study. This study is registered with ClinicalTrials.gov (NCT01274351). Results Of 112 patients enrolled, 66.1% (80% CI, 59.7-72.0%) achieved MMR and 22.3% achieved a deep molecular response of MR4.5 (BCR-ABL1IS ≤0.0032%) by 12 months. During the first year of treatment, one patient progressed to blast crisis and two patients died. Safety results were consistent with previous studies. Most adverse events (AEs) were grade 1/2. Most frequently reported nonhematologic AEs of any grade were elevations in bilirubin, alanine aminotransferase, and triglycerides. Conclusion These results support the use of nilotinib 300 mg twice daily as a standard-of-care treatment option for patients with newly diagnosed CML-CP with low and intermediate risk.

Amaç: Birçok faktör hematopoetik kök hücre (HKH) engraftmanını etkiler. Bu çalışmada hematolojik kanseri olan hastalarda kemik iliğindeki myelofibrozis ve anjiogenezisin engraftman üzerine etkisi araştırılmıştır. Gereç ve Yöntem: Otolog kök hücre nakli yapılan 34 hasta (20 erkek, 14 kadın) verileri değerlendirildi. Nakilden önceki son kemik iliğindeki fibrozis 0-3 arasında derecelendirildi, anjiogenezis stereolojik metod ile ölçüldü. Hastalar anjiogenezis parametrelerinin yoğunluğuna göre de iki gruba ayrıldı. Bulgular: Hastaların çoğunluğu (%73.5) multipl myelom idi ve yarısında fibrozis saptandı. On bir hasta derece 1, 6 hasta derece 2 fibrozise sahipti. Trombosit ve eritrosit engraftman günleri açısından derece 2 fibrozisi olan grupla fibrozis saptanmayan grup arasında istatistiksel anlamlı farklılık vardı. Derece 1 ve 2 fibrozis gruplarında fibrozisi olmayanlara göre VSD ve NVES düzeyleri anlamlı olarak yüksekti. Toplam yaşam derece 2 fibrozisi olan grupta daha düşük olmakla birlikte istatistiksel anlamlılık yoktu. Sonuç: Kemik iliği myelofibrozisinin bağımsız bir risk faktörü olduğu saptanmıştır. Bunun toplam yaşamı etkilemeden otolog nakil sürecinde trombosit ve eritrosit engraftmanı üzerine negatif etkisi olabilir. Fibrozisin derecesi ile doğru orantılı olarak artmış anjiogenezis arasındaki ilişkiyi açıklamak için ileri çalışmalar gereklidir. Purpose: Hematopoietic stem cell (HSC) engraftment is influenced by many factors. We investigated the effects of bone marrow fibrosis and angiogenetic structure on engraftment in patients with hematological malignancies. Materials and Methods: Data were collected from 34 patients (20 males and 14 females) who underwent autologous HSC transplantation. Bone marrow myelofibrosis was graded from 0 to 3, angiogenesis was quantified using a stereological method in the most recent bone marrow biopsy before the transplantation. Patients were categorized into two groups according to intensity of angiogenesis parameters. Results: Half of the patients had fibrosis and majority had multiple myeloma (73.5%). Eleven patients had grade 1, six had grade 2 myelofibrosis. The engraftment day (ED) for platelets and erythrocytes was significantly different between the grade 2 fibrosis and non-fibrosis groups. VSD and NVES levels were significantly higher in the grades 1 and 2 fibrosis groups than the no fibrosis group. While the overall survival time was shorter in the grade 2 fibrosis group than the others, the difference was not statistically significant. Conclusion: Bone marrow fibrosis was found to be independent risk factor. It may have a negative effect on platelet and erythrocyte engraftment time of autologous transplantation process but this effect does not influence survival.

Peripheral blood is the prefered source for hematopoietic stem cells for hematopoietic stem cell transplantation. The efficiency of peripheral blood stem cell (PBSC) collection can vary among devices. In this study we aimed to compare feasibility and effectivity of apheresis procedures of the different systems. Two apheresis systems [Com.Tec (Fresenius Healthcare) and Spectra Optia (Caridian BCT)] were used in our center for the collection of PBSCs for autologous and allogeneic transplantation. We retrospectively analysed 190 apheresis procedures performed in healthy donors and patients between June 2012 and November 2014 in Department of Hematology, Dokuz Eylul University. PBSCS were collected by Fresenius cell separator (64 procedure) or Spectra Optia cell separator (126 procedure). Mobilization treatments were G-CSF (26.8%), cyclophosphamide plus G-CSF (48.4%), prelixafor plus G-CSF (14.7%), ESHAP (10%) and others. Patient and donor characteristics (age, weight, volume processed, disease, mobilization regimes) were similar in Fresenius and Spectra Optia apheresis groups. Altough both collected PBSCs efficiently, the amount of CD34+ cell in product collected by Spectra Optia device was significantly higher (p

Objective High-doses of melphalan treatment with autologous stem cell transplantation in multiple myeloma (MM) remains a major treatment modality in suitable patients. A minimal dose of 2x106/kg CD34+ cells is preferred to achieve engraftment. Some patients need multiple leukapheresis procedures to achieve the necessary number of CD34+ cells, but this can cause a high volume of stem cell product that cannot be given in a single day. Whether or not the number of infusion days affects engraftment has not been studied before. We aimed to evaluate the impact of reinfusion of stem cells on multiple days on engraftment results. Materials and methods Demographic features, CD34+ cell doses, neutrophil and platelet engraftment days, hospitalization days, and number of infusion days of 149 autologous transplantations of 143 MM patients were evaluated retrospectively. Results The data of 143 MM patients who were transplanted were analyzed retrospectively. Median age was 55±8.5 (range: 26-70) years with a male/female ratio of 91/58. Hospitalization days for all patients were 24±6 (range: 14-50) days. Mean CD34+ cell number was (7.5±5.3)x106/kg (range: 1.5-31x106/kg). CD34+ cells were reinfused in 1 day in 80.5% (n=120) of the patients, 2 days in 18.2% of the patients (n=27), and 3 days in 1.3% of the patients (n=2). For 29 patients, reinfusion was applied in more than 1 day because of the high volume of stem cell product. We did not see any dimethyl sulfoxide toxicity, cardiac arrhythmia, or volume overload complications. Hypertensive attacks during infusion were easily controlled by furosemide treatment. In the group with multiple infusions, the infused CD34+ cell numbers had a mean of (4.8±2.8)x106/kg, and in the single infusion group the mean was (8.1±5.5)x106/kg. There were no statistical differences between the two groups regarding platelet and neutrophil engraftment days (p=0.850, r=0.820 and p=0.500, r=0.440). There was no statistical difference between the two groups for hospitalization days (p=0.060, r=0.050). Conclusion In cases with a high volume of stem cell product to acquire adequate stem cells, reinfusion can be safely applied across multiple days without any delay in engraftment.

WOS: 000445328900014 PubMed ID: 30053318 Background: Recent reports have showed that neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and monocyte-to-lymphocyte ratio (MLR) are predictors of progression-free survival (PFS) and overall survival (OS) in many types of cancer. This study evaluates the predictive value of NLR, MLR, and PLR for survival in MM patients treated with to ASCT. Methods: A set of data consisting of 150 patients who underwent autologous stem cell transplantation (ASCT) for MM was collected retrospectively. The prognostic value of NLR, MLR, and PLR was investigated with Kaplan-Meier method. Results: The prognostic value of NLR, MLR, and PLR was analyzed by a receiver operating characteristic (ROC) curve established to determine the cutoff. These cutoff values of NLR, PLR, and MLR were found 1.46, 86, and 0.27, respectively, on the 100th day of post-transplantation period. The overall survival (OS) and the post-transplantation OS of the patients with high NLR, MLR, and PLR levels on the 100th day of post-transplantation were shorter than the other group (P = 0.05, P = 0.018 [NLR], P = 0.05, P = 0.002 [MLR], P = 0.000, P = 0.001 [PLR]). The post-transplantation progression-free survival (PFS) of the patients with high NLR, MLR, and PLR levels on the 100th day of post-transplantation was shorter as well (P = 0.036, P = 0.001, P = 0.001, respectively). Conclusion: As increased NLR, MLR, and PLR predicted poor clinical outcome in MM patients with autologous transplantation in this study, they may serve as cost-effective and rapidly available prognostic biomarkers for these patients.

WOS: 000448137100007, PubMed ID: 29486663, Objectives: This report presents final results (24 months of follow-up) from the first prospective, national study of frontline nilotinib in chronic myeloid leukemia (CML) patients in Turkey. Methods: Patients with newly diagnosed Philadelphia chromosome-positive CML in chronic phase (CML-CP; N = 112) received nilotinib 300 mg twice daily. The primary endpoint, which was the cumulative rate of major molecular response (MMR; BCR-ABL1, Novartis Pharmaceuticals CorporationNovartis, This work was supported by the Novartis Pharmaceuticals Corporation.

It has been shown that imatinib mesylate, a drug used in the treatment of chronic myelogenous leukemia, inhibits the effect of stem cell factor, which has a central role in erythropoiesis. In some polycythemia vera (PV) patients, it has inhibited autonomous erythroid colony growth in vitro and decreased the need for phlebotomy. In this study we have investigated the effect of insulin like growth factor (IGF)-I, stem cell factor (SCF) and erythropoietin (Epo) with interleukin (IL)-3, granulocyte macrophage-colony stimulating factor (GM-CSF) and granulocyte-colony stimulating factor (G-CSF) in the presence of imatinib mesylate on the erythroid progenitors derived from peripheral blood mononuclear cells of three patients with PV and four healthy controls in semisolid medium. Erythroid colony formation from hematopoietic progenitors obtained from healthy controls was observed only in the presence of all cytokines. However, the number of erythroid colonies could not reach that of patients with PV. Inhibition of imatinib mesylate on erythroid colony growth was evident. Hematopoietic progenitors of patients with PV displayed two types of colony formation: the first type was exogenous cytokine-independent and was hypersensitive to current cytokines, and the second displayed hypersensitivity to current exogenous cytokines, but was exogenous cytokine-dependent. For both types, the inhibitory effect of imatinib mesylate was striking in the presence of all cytokines including IL-3, GM-CSF and Epo. There is no direct evidence yet that imatinib mesylate could inhibit the effect of IL-3, G-CSF, GM-CSF, Epo and IGF-I on erythropoiesis. Considering former studies together with results of this study, it can be argued that imatinib mesylate is effective in PV on the intersecting signal transduction mechanisms in which stem cell factor and its receptor may have a part.Kronik myeloid lösemi tedavisinde kullanılan imatinib mesilat aynı zamanda kök hücre faktörünü de inhibe etmektedir. Imatinib mesilatın bazı polistemia veralı hastalarda otonom eritroid koloni gelişimini inhibe ettiği ve flebotomi ihtiyacını azalttığı da gösterilmiştir. Bu çalışmada, imatinib mesilat varlığında, polistemia veralı üç hasta ile sağlıklı dört kontrolden elde edilen periferik kan eritroid öncül hücreleri üzerine yarı-katı ortamda insülin benzeri büyüme faktörü-l (IGF-l), kök hücre faktörü (SCF) ve eritropoietin (EPO) ile interlökin-3 (IL-3), granülosit-koloni stimule edici faktörün (GM-CSF) ve granülosit-koloni stimule edici faktörünün (G-CSF) etkisi araştırıldı. Sağlıklı kontrollerden elde edilen hematopoietik öncü hücrelerden eritroid koloni gelişimi sadece tüm sitokinlerin varlığında gözlendi. Bununla birlikte eritroid kolonilerin sayısı polistemia veralı hastalardan elde edilen sayılara ulaşamadı. Imatinib mesilatın eritroid koloni gelişimi üzerindeki inhibe edici etkisi belirgindi. Polistemia veralı hastaların hematopoietik öncüllerinden iki tip eritroid koloni gelişimi gözlendi; ilki eksojen sitokinlerden bağımsız ve sitokinlere aşırı duyarlılık gösteren, ikincisi ise eksojen sitokinlere aşırı duyarlılık gösteren tip idi. Her iki tipte de IL-3, GM-CSF ve EPO de dahil olmak üzere, sitokinlerin varlığında imatinib mesilatın inhibe edici etkisi belirgindi. Imatinib mesilatın bu etkisinin IL-3, G-CSF, GM-CSF, EPO ve IGF-l üzerine olduğunu söylemek için henüz yeterli kanıt yoktur. Bu çalışmanın sonuçları daha önceki çalışmalardan elde edilen bilgilerle birlikte değerlendirildiğinde, imatinib mesilatın eritroid koloni gelişimini inhibe edici etkisinin kök hücre faktörünün ve reseptörlerinin rol aldığı sinyal ileti yolları üzerinden olduğu söylenebilir.

Fludarabine-containing combinations have additive cell killing against leukemic blasts in vitro. It has also been shown that imatinib mesylate combined with fludarabine or cladribine had an additive effect on CML CFU-GM cells. In this regard, we aimed to investigate the effect of fludarabine-imatinib mesylate combination against CML blastic phase cell lines K562 and Meg-01. XTT test was performed for proliferation and inhibition assay. According to obtained data, five different effective concentrations of each drug in 25 different combinations were tested. Results of the combination studies were analyzed with isobologram. At IC20, imatinib mesylate and fludarabine combination showed synergism and strong synergism in K562 and Meg-01 cells, respectively. At IC50 and IC75, combination indexes (CI) indicated strong synergism and synergism. Based on our results, the fludarabine- based chemotherapy regimens can be used for those patients with CML blastic phase in combination with imatinib mesylate.In vitro koşullarda fludarabin içeren kombinasyonların lösemik blastlar üzerinde ve fludarabin ya da kladribinin imatinib mesilat ile kombinasyonlarının KML CFU-GM hücrelerine karşı aditif etkinliğe sahip oldukları gösterilmiştir. Bu gözlemlere dayanarak biz de fludarabin ve imatinib mesilat kombinasyonunun KML blastik faz hücre serileri K562 ve Meg-01 üzerine olan etkinliğini araştırmayı planladık. Proliferasyon ve inhibisyon göstergesi olarak XTT testini kullandık. Elde edilen verilere göre, her ilacın beş farklı etkin konsantrasyonu 25 farklı kombinasyon halinde test edildi. Kombinasyon çalışmalarının sonuçları izobologram ile analiz edildi. İmatinib mesilat ve fludarabinin kombinasyonunun K562 ve Meg-01 hücre serileri için IC20 kombinasyon indeksi değerleri sırası ile sinerjistik ve güçlü sinerjistik, IC50 ve IC75 değerlerinde de güçlü sinerjistik ve sinerjistik etkinliği göstermekte idi. Bu sonuçlar KML blastik fazdaki hastalar için imatinib mesilat ile kombine edilen fludarabin bazlı rejimlerin kullanılabileceğini göstermektedir.

The clinical course of patients with inflammatory bowel disease (IBD) is frequently complicated by thromboembolic events and may involve the arterial and venous systems. Although not uniformly documented, several studies document substantial alterations in markers of coagulation and fibrinolysis in patients with IBD.45 patients with IBD (31 UC,14 CD) were included in the study. Age and sex matched 16 volunteers were used as a control group. TAFI antigen was determined using an ELISA kit VisuLiseTM for quantitative measurement.Inflammatory parameters such as white blood cell, platelet levels, erythrocyte sedimentation rate, C-reactive protein were found to be significantly higher in active disease group compared to inactive patients. Coagulation parameters of prothrombin time, activated partial thromboplastin time and d-dimer levels showed no significant difference between active and inactive IBD. Fibrinogen levels were significantly higher in clinically active IBD patients. Plasma TAFI levels demonstrated no significant difference between active and control, inactive and control as well as active and inactive groups. We observed no significant changes in levels of β-TG and PF-4 between active and inactive disease group.We studied plasma TAFI levels in IBD. In conclusion, plasma TAFI levels does not appear to represent to be a marker of activation in IBD in contrast to literature. So further studies covering more patients with different clinic and disease activity status might improve the perspective on this issue.GİRİŞ: İnflamatuar barsak hastalığının (IBH) seyri, arteriyel ya da venöz sistemde olabilen tromboembolik olaylar ile sık olarak komplike olmaktadır. Tümü olmasa da çalışmaların çoğu IBH olan hastalarda koagülasyon ve fibrinoliz göstergelerindeki değişiklikleri ortaya koymaktadır. YÖNTEM: Çalışmaya IBH olan 45 hasta (31 UC, 14 CD) dahil edildi. Yaşı ve cinsiyeti uyumlu 16 gönüllü kontrol grubu olarak alındı. TAFI antijeninin kantitatif olarak saptanması için VisuLiseTM ELISA kiti kullanıldı. SONUÇLAR: Beyaz küre sayısı, trombosit sayısı, eritrosit sedimentasyon hızı ve C-reaktif protein gibi inflamasyon belirteçleri aktif hastalığı olanlarda belirgin yüksek bulundu. Aktif ya da inaktif IBH olanların protrombin zamanı, aktive parsiyel tromboplastin zamanı ve d-dimer düzeyleri gibi koagülasyon ölçekleri arasında anlamlı fark bulunmadı. Hastalığı aktif olanların fibrinojen düzeyleri belirgin olarak daha yüksekti. Aktif ya da inaktif hastalığı olanlar ile sağlıklı kontrollerin plazma TAFI düzeyleri arasında anlamlı bir fark gösterilemedi. Aktif ve inaktif hastalığı olanların β-TG ve PF-4 düzeyleri arasında da anlamlı bir değişiklik gözlenmedi. SONUÇ: IBH’da TAFI düzeylerini araştırdık. Literatürdeki bilgilerle çelişecek şekilde TAFI düzeyleri IBH’nın aktivasyon göstergesi olarak kullanılabilir gözükmemektedir. Hastalığın farklı aşamalarında ve aktivasyon düzeylerinde olan daha fazla hastayı kapsayan çalışmaların yapılması konunun daha iyi aydınlamasına yardımcı olacaktır.

t(1;3)(p36;p21) is a recurrent reciprocal translocation found in a subset of myelodysplastic syndrome (MDS)/acute myelogenous leukemia (AML) characterized by trilineage dysplasia, especially dysmegakaryopoiesis and poor prognosis. In the literature, some authors have suggested that this recurrent translocation is closely associated with prior chemotherapy including alkylating agents in various hematologic malignancies. We identified a recurring translocation, t(1;3)(p36;p21), in our patient with MDS/AML(M2), although she had not been given any kind of treatment previously.(1;3)(p36;p21) sitogenetik bulgusu, Myelodisplastik Sendrom (MDS)/Akut Myeloid Lösemi (AML) olgularının özellikle dismegakaryopoez ve kötü prognoz ile seyreden bir kısmında gözlenen tekrarlayan bir resiprokal translokasyondur. Bazı yazarlar bu translokasyonun değişik hematolojik kanserlerde alkilleyici ajanlarla yapılan kemoterapiyle sıkı bir ilişkisi olduğunu ileri sürmüşlerdir. Bu çalışmada, daha önce kemoterapi uygulanmamış t(1;3)(p36;p21) bulgulu bir MDS/AML(M2) olgusu sunulmaktadır.

Thrombocytopenia may be the presenting cytopenia of myelodysplastic syndrome (MDS) and is named as refractory thrombocytopenia (RT) and categorized in the refractory cytopenia with multilineage dysplasia (RCMD) group according to the recent World Health Organization (WHO) classification of the acute leukemias and MDS. Abnormal cytogenetics can be found in 60% to 80% of patients with MDS. Most common cytogenetic abnormalities include monosomy 5, 5q-, monosomy 7, trisomy 8, deletion 20q and loss of X or Y chromosome. Here we report clinical features and outcomes of nine patients with RT. Cytogenetic abnormalities were detected in seven. Among two patients who have a normal karyotype at diagnosis, one of them transformed to acute myeloid leukemia (AML). During a median follow-up of 29 months, two patients died of hemorrhagia and one of AML. The features and prognosis of patients with RT needs to be determined by larger series.Trombositopeni myelodisplastik sendromu (MDS)’nun ilk bulgusu olabilir ve refrakter trombositopeni (RT) adı altında Dünya Sağlık Örgütü (WHO)’nün akut lösemi ve MDS sınıflandırmasında multilinaj displaziıe e.lik eden refrakter sitopeni (RCMD) grubuna dahil edilmektedir. MDS’li olguların %60-80’inde anormal sitogenetik bulgular olur. En sık görülen sitogenetik anomaliler arasında monozomi 5, 5q-, monozomi 7, trizami 8, 20q delesıonu ve X ya da Y kromozom kaybı vardır. Biz bu yazıda RT’li dokuz olgunun klinik özelliklerini bildirdik. yedi olguda sitogenetik anomali saptandı. Tanı sırasında normal karyotipe sahip iki olgudan bir tanesi AML’ye dönüştü. Median 29 aylık bir izleme sonunda, iki olgu hemorajiden kaybedilirken, bir olgu AML’ye dönüşerek kaybedildi. RT’li olguların özellikleri ve prognozlarının daha geniş serilerde değerlendirilmesi gerekmektedir.

Multiple myeloma (MM) is a hematologic cancer characterized by malignant proliferation of plasma cells and their precursors. Immunosuppressive CD4+CD25+Foxp3+ regulatory T (Treg) cells are increased in the peripheral blood of patients with MM. On the basis of this finding, we sought to evaluate the ex vivo effect of CD4+CD25+Foxp3+ Treg cells on the anti-tumor effect of the proteosome inhibitor bortezomib on MM cells. We collected peripheral blood and bone marrow aspiration samples from 20 patients with newly diagnosed MM and isolated CD4+CD25+Foxp3+ Treg cells from peripheral blood mononuclear cells. The bone marrow mononuclear cells were cultivated in RPMI at 37 degrees C and 5% CO2 for 72 hours. The LD50 doses of bortezomib, isolated Treg cells, and their combination were added. After 24 hours, the viability of CD138+ myeloma cells was evaluated by WST-1. We compared the anti-tumor effect of bortezomib alone and in combination with Treg expansion and statistically analyzed the measured differences with respect to the clinical parameters of the patients. Treg cells had varied effects on bortezomib, increasing, decreasing, or not changing its anti-tumor effect. The increased in vitro anti-tumor effect of bortezomib after Treg cell expansion was correlated in patients who did not develop bortezomib resistance in vivo (p = 0.022). These patients with in vivo non-bortezomib-resistant MM also responded to Treg expansion with decreased cell viability (p = 0.024). Our data indicate that the ex vivo expansion of Treg cells increased the cytotoxic effect of bortezomib in clinically sensitive cases. Copyright (C) 2016 ISEH - International Society for Experimental Hematology. Published by Elsevier Inc.

PubMed ID: 27501474, Objective: Nilotinib is a BCR-ABL1 tyrosine kinase inhibitor approved for the treatment of patients with chronic myeloid leukemia in chronic phase (CML-CP). This study was the first prospective evaluation of the efficacy and safety of nilotinib in Turkish patients with newly diagnosed CML-CP. The primary endpoint of the study was the rate of major molecular response (MMR; BCR-ABL1 ? 0.1% on the International Scale [BCR-ABL1IS]) by 12 months. Methods: Patients with newly diagnosed CML-CP were treated with nilotinib 300 mg twice daily. This analysis was based on the first 12 months of follow-up in a 24-month study. Results and Conclusions: Of 112 patients enrolled, 66.1% (80% CI, 59.7–72.0%) achieved MMR and 22.3% achieved a deep molecular response of MR4.5 (BCR-ABL1IS ? 0.0032%) by 12 months. During the first year of treatment, 1 patient progressed to blast crisis and 2 patients died. Safety results were consistent with previous studies. Most adverse events (AEs) were grade 1/2. Most frequently reported nonhematologic AEs of any grade were elevations in bilirubin, alanine aminotransferase, and triglycerides. These results support the use of nilotinib 300 mg twice daily as a standard-of-care treatment option for patients with newly diagnosed CML-CP. © 2016 Informa UK Limited, trading as Taylor & Francis Group., Novartis Pharmaceuticals Corporation, This study was funded by Novartis Pharmaceuticals Corporation.

The curative treatment approach for diffuse large B-cell lymphoma (DLBCL) is controversial even in the rituximab (R) era. The aim of this study was to examine the FcγRIIIA gene polymorphism distribution of DLBCL patients who had been treated with R-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. Furthermore, we investigated the impact of FcγRIIIA gene polymorphism on the overall response rate (ORR) and overall survival (OS).Patients from 3 centers in the Aegean region of Turkey who had newly diagnosed CD20-positive DLBCL were enrolled in the study. The single nucleotide polymorphisms of the FcγRIIIA gene were analyzed by real time-PCR. The response to treatment was determined in the middle and at the end of the protocol. During 2 years of follow-up, the patients were clinically and radiologically evaluated for disease status every 3 months.Thirty-six patients were included in the study and the distributions of F/F, V/F, and V/V types of alleles of FcγRIIIA were 25%, 50%, and 25%, respectively. Twenty-seven patients were considered as evaluable according to ORR and OS. The patients' ORR was 87.5%, 100%, and 50% in the F/F, V/F, and V/V allele groups, respectively. We did not establish any statistically significant differences among the 3 alleles groups in respect to ORR (p=0.93). The OS within 2 years in the F/F, V/F, and V/V allele groups was 62.5%, 100%, and 100%, respectively. The OS in the F/F allele group was found to be lower than in the other 2 allele groups (p=0.01).The distribution of gene polymorphisms in our study group was similar to those of previous studies. While ORR was similar between the groups, our results highlight a lower OS in F/F patients compared to other allele groups of FcγRIIIA.Amaç: Diffüz büyük B hücreli non-Hodgkin lenfomada (DLBCL) kür sağlayıcı tedavi yaklaşımı rituximab çağında olmamıza rağmen tartışmalı bir konudur. Bu çalışmanın amacı R-CHOP (siklofosfamid, doksorubisin, vinkristin ve prednizon) rejimi alan DLBCL hastalarında FcγRIIIA gen polimorfizminin dağılımını incelemekti. Ayrıca FcγRIIIA gen polimorfizminin tüm yanıt oranları (ORR) ve tüm yaşam (OS) üzerine olan etkisini araştırmaktı. Gereç ve Yöntemler: Türkiye’nin Ege Bölgesi’ndeki üç merkezden yeni tanı almış CD-20 pozitif DLBCL hastaları çalışmaya dahil edildi. FcγRIIIA’daki tek gen polimorfizmi gerçek zamanlı-PCR ile incelendi. Tedaviye yanıt, planlanmış olan protokolün ortasında ve sonunda değerlendirildi. İki yıllık takip süresince her üç ayda bir hastalığın hem klinik, hem de radyolojik durumu ele alındı. Bulgular: Çalışmaya dahil edilen 36 hastada, FcγRIIIA’nın F/F, V/F ve V/V alellerinin dağılımı sırasıyla %25, %50 ve %25’ti. ORR ve OS verilerine göre 27 hasta değerlendirilebilir olarak kabul edildi. Hastaların ORR değerleri F/F, V/F ve V/V alel gruplarına göre sırasıyla %87,5; %100 ve %50 olarak hesaplandı. Hastaların ORR değerleri açısından üç alel grubu arasında istatistiksel olarak anlamlı fark saptanmadı (p=0,93). F/F, V/F ve V/V gruplarında iki yıllık OS %62,5, %100 ve %100 bulundu. F/F alel grubunun OS’si diğer iki alel grubundakinden daha düşük bulunmuştur (p=0,01). Sonuç: Gen polimorfizmi dağılımı sonuçlarımız önceki çalışmalarda bulunanlarla benzerdir. Gruplar arasında ORR değerleri arasında fark yokken, sonuçlarımız F/F hastalarının FcγRIIIA’nın diğer allel gruplarına göre daha kısa bir OS değerine sahip olduğunu göstermektedir.

The serum albumin (SA) level has been reported to be an independent prognostic biomarker that may serve as a surrogate representative of disease biology in patients diagnosed with myelodysplastic syndrome (MDS). However, its prognostic ability has not been tested in a model adjusting for comorbidities. We analyzed 200 patients who were diagnosed as having de novo MDS. Median overall survival (OS) of all patients was 25 months and median leukemia-free survival (LFS) was 24 months. Median OS according to the SA level groups of 4.0 mg/dL were 24, 39 and 77 months, respectively. SA level remained an independent predictor of both LFS and OS even when adjusting for the hematopoietic cell transplant comorbidity index (HCT-CI) and the International Prognostic Scoring System (IPSS) or World Health Organization classification-based Prognostic Scoring System (WPSS). Our findings indicate that SA level at the time of diagnosis is a significant and independent predictor of LFS and OS even when adjusting for commonly used prognostic systems and comorbidities.

© 2015 Elsevier Inc. All rights reserved.Introduction Essential thrombocythemia (ET) is the most common of the myeloproliferative neoplasms. For better predicting the occurrence of thrombotic events, an International Prognostic Score of Thrombosis for ET (IPSET-Thrombosis) was recently developed. We aimed to investigate the validity of IPSET-Thrombosis in a Turkish patient cohort and to compare the efficacy of IPSET-Thrombosis and conventional risk scoring systems in predicting thrombosis-free survival. Patients and Methods We retrospectively evaluated the clinical characteristics and risk factors for thrombosis in 112 Turkish patients. Median thrombosis-free survival and Harrell C concordance indexes were calculated for both conventional and IPSET-Thrombosis. Results Median age of 112 patients included in the study was 61 (range, 27-90) years at the time of diagnosis. When patients were stratified according to the conventional risk stratification system, 43.8% of patients were in the low-risk group and 56.2% in the high-risk group. A total of 22.4% of low-risk and 42.9% of high-risk patients had at least one thromboembolic event. When patients were stratified according to the IPSET-Thrombosis, 33% were in the low-risk group, 26.8% in the intermediate-risk group, and 40.2% in the high-risk group. Considering IPSET-Thrombosis risk groups, 5.4% of low-risk, 26.7% of intermediate-risk, and 66.2% of high-risk patients had at least one thromboembolic event. Regarding IPSET-Thrombosis risk groups, 10-year thrombosis-free survival was 86.8% for low-risk, 39.4% for intermediate-risk, and 32.9% for high-risk groups (P

Leukemic infiltration of the gingiva is most commonly reported to be associated with monocytic subtypes of acute myeloblastic leukemia (AML) but rarely with myelodysplastic syndromes (MDS). Here we report a case of CD56(+) chronic myelomonocytic leukemia (CMML) who developed gingival involvement simultaneously when the leukocyte count elevated. At that time no increase in peripheral or bone marrow blasts were observed. Gingival hypertrophy regressed with the treatment of hydroxyurea. Three months later, bone marrow blast count elevated and the patient was treated with two courses of AML-like regimen and then one course of consolidation therapy. The patient is in complete hematological remission for one and a half years. Similar to other extramedullary involvements, gingival hypertrophy in CMML can be a harbinger of the disease entering a more aggressive phase requiring systemic chemotherapy.

To explain the pathogenesis of preeclampsia with coagulation induction or a defective fibrinolysis, various hemostatic parameters were studied and different treatment modalities targeting these parameters were evaluated. Considering the role of TAFI in down-regulation of fibrinolysis, in our study we have investigated whether TAFI contributes to impaired fibrinolysis in patient with preeclampsia. Thirty patients with preeclampsia (mean age +/- SD 25.7 +/- 4.53; range 17-36) and 30 normal pregnant women as control group (mean age +/- SD 28 +/- 5.26; range 21-38) were included in our study. TAFI antigen was determined using an ELISA kit for quantitative measurement. The mean TAFI antigen levels were 12.55 +/- 1.88 microg/ml in patients with preeclampsia and 12.29 +/- 3.0 microg/ml in normal pregnant women. A statistically significant difference was not found between TAFI antigen levels of two groups (p0.05). In order to clarify the role of TAFI in the pathogenesis of preeclampsia, in addition to plasma TAFI levels, its synthesis, activation and metabolism should also be evaluated.

Multiple induction regimens have been developed for adult patients with acute lymphoblastic leukemia (ALL). However, there have been no prospective randomized trials that directly compare these regimens. In this study, we wanted to evaluate the outcome of 50 adult ALL patients treated with BFM (i.e. Berlin-Frankfurt-Munster, n = 20) and hyper-CVAD (n = 30) protocols between March 2006 and October 2012. The median age was 25 years in the BFM group and 30.5 years in the hyper-CVAD group with a male/female ratio of 15:5 and 17:13, respectively. Forty-five percent of the patients in the BFM group and 30.3% in the hyper-CVAD group were

Tissue factor pathway inhibitor (TFPI) is a Kunitz-type proteinase inhibitor that has a crucial role in haemostasis and is primarily synthesized in the vascular endothelium. We investigated plasma total TFPI, antiphospholipid antibodies, and some other coagulation and fibrinolytic system parameters in 30 patients with Behcet's disease and 15 controls by the enzyme-linked immunosorbent assay method. TFPI levels were significantly higher in the Behcet's group (119 ± 57.5 ng/ml) compared with the control group (74.8 ± 31.5) (P < 0.009). We also noted a statistical significance in TFPI levels between patients with active disease (n:16) (139 ± 55) and patients without activation (n:14) (96 ± 53) (P < 0.03), whereas inactive patients lacked any significance when compared with the control group (P < 0.29). Other parameters disclosed no statistical significance between patients and control group except for elevated fibrinogen and plasminogen activator inhibitor-1 levels in the patient group (P < 0.003). Increased levels of TFPI may reflect a defensive mechanism like in other diseases characterized by thrombotic tendency and represent a parameter of disease activity. Am. J. Hematol. 68:225–230, 2001. © 2001 Wiley-Liss, Inc.

Cutaneous T-cell lymphoma is a term used for mycosis fungoides and Sézary syndrome, the distinct clinical entities where the skin is the primary organ of involvement. Sézary syndrome is the leukemic variant of mycosis fungoides, presenting with generalized erythroderma, lymphadenopathy, and atypical cells (the Sézary cells) in the peripheral blood and bone marrow. The dissemination of cutaneous T-cell lymphoma may occur with no exception of the organs; however, no prior report exists about the Sézary syndrome secondarily involving the breasts. We report the clinical and radiological findings of bilateral breast involvement in a case of Sézary syndrome.

Little is understood about the basic biological mechanisms that underlie the reasons for acute transformation in chronic myeloid leukemia (CML). Progression of disease may include inactivation of one or more tumor suppressor genes (TSGs). A widely used methodology for indirectly detecting somatic inactivation of TSGs is searching loss of heterozygosity (LOH) for polymorphic loci located in or near the gene(s) of interest. We aimed to analyze DNA of chronic phase and blastic phase archive material of 15 CML patients for LOH using D1S430, D2S123, D3S1611, D11S29, D14S65, D17S520, BAT 40 markers, the dinucleotide repeat located in the ABL gene and the trinucleotide repeat located in the BCR gene (amplification of the trinucleotide in the BCR gene could not be succeeded). LOH was identified by a %50 lost of one of the alleles intensity. LOH was detected with the ABL dinucleotide repeat and D2S123 marker in two patients and with the D14S65 marker in three patients. The three patients exhibiting LOH at the D14S65 locus, all proceeded through lymphoid blast crisis. The D14S65 marker is located at the 14q32 locus which contains the immunoglobulin heavy chain gene and the TCL1 oncogene. 14q32 abnormalities at the molecular level, may be predictive for lymphoid blast crisis, whether or not they are detectable cytogenetically.

The hemostatic activity of blood shows a circadian variation with a higher frequency of acute coronary events in the morning. The thrombotic tendency of blood is influenced by many factors, including platelets. Diurnal changes of in vivo platelet activation were investigated by whole blood flow cytometry in 10 young healthy male volunteers using anti-GMP-140 (anti-alpha-granule membrane protein 140 kD) monoclonal antibody at 3h intervals from 06:00 to 24:00. We also studied circulating platelet aggregates to investigate whether there exists a similarity between the results of these methods. Results of flow cytometric analysis indicate that there is an increase in platelet activation during the period from 06:00 to 09:00. Platelet activation then decreases gradually during the period from noon to midnight. These changes are accompanied by a similar trend in circulating platelet aggregates. This suggests that GMP-140 expression on platelets is synchronized with or followed by platelet aggregate formation in vivo, and increased platelet activation may predispose individuals to thrombosis at this time.

A non-Hodgkin’s Iymphoma case with actinomycosis of the tongue in a forty-three years old male patient is described in this article. The patient hospitalized because of NHL relapse and ifosphamide, methotrexate, bleomycin, prednisolone and intrathecal methotrexate were given as chemotherapy. In the course of the hospital stay, fungemia has been documented and an isolated lesion has emerged in the tongue. Wedge biopsy was performed yielding the diagnosis of actinomycosis. The case is reported from the point of its rare localization and clinical presentation, diagnostic methods, differential diagnosis and treatment. Actinomycosis should be beared in mind in immunocompromised patients with oral lesions.

To evaluate the clinical significance of immunoperoxidase staining for CD10, bcl-6, mum-1 and bcl-2 to subdivide DLBCL into prognostic subgroups, we analysed 50 DLBCL cases using immunohistochemical methods.Fifty DLBCL patients were evaluated retrospectively. The expression of CD10 was associated with better OS (p=0.04) whereas expression of mum-1 was associated with worse OS (p=0.009). There were no significance of OS in case of expression of bcl-6 (p=0.05) and bcl-2 (p=0.3). They were subclassified using CD10, mum-1, bcl-6 as germinal center B-cell like (GCB) lymphoma (30%) and non-GCB lymphoma (70%). The OS and EFS (event free survival) were longer in GCB group (p=0.002) and 5-year OS for GCB group was 92% compared with only 44% for the non-GCB group (p=0.02). The OS of the GCB group also was longer compared to that of the non-GCB group in low IPI subgroup (p=0.01).The existence of survival differences between GCB a non-GCB group also in the patients with low IPI score, showed the importance of prognostic classification in the risk-adaptive treatment approaches. The classification as GCB and non-GCB based immunostains may enable to define more accurate prognostic groups in DLBCL.

A patient with mantle cell non-Hodgkin's lymphoma presented herself with fever, nausea, right upper quadrant pain on the 7th day of R-CHOP chemotherapy. After hospitalization with the suspicion of acute cholecystitis, she received antibiotherapy with G-CSF because of emerging neutropenia at the 10th day of chemotherapy. Abdominal computed tomography revealed small infarcts in the spleen and kidneys. The ecchymotic lesion which developed on her right lateral malleolus, became bullous in the following days and treated as ecthyma gangrenosum. Although the patient was afebrile with a normal neutrophil count on the third day of antibiotherapy, she developed acute renal failure and deteriorated rapidly. The patient underwent hemodialysis but expired on the 10th day of hospitalization. Post mortem autopsy findings showed ischemic infarction and necrosis of parenchyma due to mycotic thrombosis of arteries and veins of many organs (heart, lung, diaphragm, kidneys, spleen, gut mucosa) as well as invasion of vessel walls and parenchyma by mucor. We reviewed mucormycosis in the light of this case.

In a retrospective analysis of 113 patients with primary myelodysplastic syndromes (MDS) diagnosed according to French-American- British (FAB) classification, we evaluated the prognostic impact of FAB and World Health Organisation (WHO) classifications, International Prognostic Scoring System (IPSS), and other clinical and laboratory variables. The median age was 69. IPSS could be applied to 75 patients classified according to the FAB criteria and to 50 patients reclassified according to the WHO criteria. At a median follow-up of 24 months, 22 patients (19.5%) transformed to acute myelogenous leukaemia (AML). Overall survival (OS) of patients differed significantly between the FAB and WHO subgroups (p50.0001). In WHO classification, significant differences were observed in both OS and leukaemia free survival (LFS) between patients with RA/ RARS and refractory cytopenia with multi-lineage dysplasia/refractory cytopenia with multi-lineage dysplasia and ringed sideroblasts (RCMD/RS-RCMD) (p = 0.0001). High-risk according to IPSS score and blood transfusion need were significantly predictive for a shorter survival and higher risk of transformation. Hemoglobin

Preeclampsia has been associated with increased platelet activation detected before disease onset. Platelets are involved in hemostasis and also directly initiate an inflammatory response of the vessel wall. Inappropriate activation of platelets may be involved in pathogenesis in preeclampsia by promoting coagulation and thrombosis, and also as a mediator of inflammation. Platelets may release inflammatory mediators such as soluble CD40 ligand. The plasma level of soluble CD40 ligand was investigated during preeclamptic (n =20) and normal pregnancies (n = 20) to emphasize inflammatory response in preeclampsia. The mean soluble CD40 ligand levels were 1.08 ± 0.43 ng/mL in patients with preeclampsia and 0.76 ± 0.24 ng/mL in healthy pregnant women, which was statistically significant ( P = .01). To clarify whether inflammation may cause inappropriate endothelial cell activation or inappropriate endothelial cell activation may start this inflammatory response, future studies are needed in a larger study population.

A 35-year-old man with systemic non-Hodgkin’s lymphoma and bilateral choroidal involvement is described. Indocyanine green angiography depicts choroidal involvement much better than fluorescein angiography and seems to be superior in diagnosing and monitoring patients with systemic non-Hodgkin’s lymphoma and choroidal involvement. [Ophthalmic Surg Lasers Imaging 2006;37:236-239.] AUTHORS From the Ophthalmology Department (AOS, GA), the Hematology Department (MAO, BU), and the Pathology Department (SO, AK), Dokuz Eylül University, Izmir, Turkey. Accepted for publication September 12, 2005. Address reprint requests to A. Osman Saatci, MD, Mustafa Kemal Sahil Bulvan, No: 73, A Blok, Daire 9, Narlidere, 35320, Izmir, Turkey.

An increased incidence of thromboembolic events has been described in women receiving systemic chemotherapy for breast cancer. The effect of anthracycline-based adjuvant chemotherapy regimens on fibrinolytic system markers of plasminogen activator inhibitor-1 (PAI-1) and thrombin activitable fibrinolysis inhibitor (TAFI) was investigated in patients with operable breast cancer. Twenty-four patients with operable breast cancer (median age, 54.5 years; range, 37-72 years) enrolled in our study. Stage I-II and stage IIIA cases received EC (Epirubicin 90 mg/m2/d1, I.V. and cyclophosphamide 600 mg/m2/d1, I.V.) and FEC (5-fluorouracil 500 mg/m2/d1, I.V., epirubicin 100 mg/m2/d1, I.V., and cyclophosphamide 500 mg/m2/d1, I.V.) as an adjuvant chemotherapy regimen, respectively. Each group consisted of 12 patients. Blood samples were obtained at baseline and just before the third cycle of EC and fourth cycle of FEC chemotherapy regimens. Plasma TAFI antigen and PAI-1 levels did not disclose any statistical difference between basal and postchemotherapy levels within each group and between two groups. Although postchemotherapy D-dimer levels were statistically higher in the FEC group than in the EC group, results in both groups were within normal ranges. More studies concerning the role of fibrinolytic system in breast cancer patients receiving chemotherapy, probably including cases with advanced stage and with different chemotherapy regimens and dose intensities, are needed.

Orbital presentation of non-Hodgkin's Lymphoma (NHL) is uncommon but occurs both as the only site of disease and as a site of recurrence. Primary orbital NHLs are usually low-grade, and mostly extranodal marginal zone/mucosa associated lymphoid tissue B-cell neoplasms. They are commonly associated with chronic inflammatory conditions. The issue of bilaterality in orbital lymphoma is not very common. The onset of malignant lymphoproliferation may precede, follow, or exist simultaneously with scleroderma. Different treatment modalities were applied in orbital NHL such as surgery, radiation therapy, chemotherapy or both. We report a 57-year-old man with scleroderma presenting with eyelid hernias who had biopsy-proven marginal zone NHL, successfully treated with radiotherapy and combined chemotherapy, and also review the literature.

A 62-year-old woman being treated for stage IIIC rectal. adenocarcinoma was diagnosed with primary non-Hodgkin lymphoma of the breast after a 4-year follow-up. This case illustrates the importance of close and Long-term follow-up as well as of differential diagnostic procedures for second primary malignancies after the initial diagnosis and treatment of a solid tumor. (c) 2004 Elsevier Ltd. All rights reserved.

Various coagulation abnormalities occur in thyroid disorders and its range may vary from subclinical laboratory abnormalities to clinically significant disorders of coagulation. Tissue factor pathway inhibitor (TFPI), which inhibits the initial reaction of tissue factor mediated coagulation pathway, is reported to be increased in patients with Graves disease (GD) in one study. Hyperthyroid (n = 10), hypothyroid (n = 10) and subclinical hypothyroid (n = 10) patients and control cases (n = 16) were evaluated for free and total tissue factor pathway inhibitor (tTFPI), plasminogen activator inhibitor-1 (PAI-1) and tissue plasminogen activator (tPA) levels in disease and euthyroid states. Free TFPI levels were significantly higher in hyperthyroid patients compared with the control group and subclinic hypothyroid patients (p < 0.001), but not with hypothyroid patients (p > 0.05). After the euthyroid state was obtained in the hyperthyroid group, the levels of total TFPI (p < 0.05), free TFPI (fTFPI) (p < 0.005), t-PA (p < 0.005) and PAI-1 (p < 0.02) decreased significantly. In hypertbyroid patients, there was a strong correlation between thyroid functions and free TFPI levels. In conclusion, we hypothesize that coagulation abnormalities seen in thyroid disorders cannot be explained directly with the impaired fibrinolytic activity but also with the elevated fTFPI levels. Both increased plasma fTFPI and PAI-1 levels could be markers of the peripheral activity of thyroid hormones. (C) 2003 Elsevier Ltd. All rights reseved.

Nasal-type T/natural killer (NK)-cell lymphoma, a distinct clinicopathological entity is highly associated with Epstein-Barr virus which shows an aggressive course. We present a CD56 + nasal-type T/(NK)-cell lymphoma case with systemic manifestations of rhabdomyolysis and polyserositis who died of multiorgan failure shortly after his admission to hospital in spite of adequate chemotherapy and supportive care.

Acquired hemophilia is a rare, life-threatening coagulopathy in adults caused by the development of autoantibodies against factor VIII. Bypass agents such as recombinant factor VIIa (rFVIIa) are usually preferred for bleeding control; however, thromboembolic complications may occur. We report here a case that presented with extensive cutaneous and mucosal bleedings due to factor VIII inhibitors and was treated successfully with rFVIIa and steroid therapy, but was complicated with a life-threatening thromboembolic attack during follow-up. Blood Coagul Fibrinolysis 23:69-70 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.

Hemophagocytic syndrome (HPS) is a rare clinicopathological disorder characterized by systemic proliferation of phagocytizing histiocytes associated with fever, cytopenias, lymphadenopathy, hepatosplenomegaly, and disseminated intravascular coagulopathy. We present the association of hemophagocytic syndrome associated with inappropriate secretion of antidiuretic hormone (SIADH) in two cases of hematological malignancies; anaplastic large cell lymphoma (ALCL) and acute myeloblastic leukemia (AML M4) In the patient with lymphoma, the diagnosis of lymphoma, HPS and SIADH were concurrent. In the patient with AML, HPS and SIADH were observed while the patient was in hematological remission. Thus it seems that patients with HPS may also carry a risk for the development of SIADH; the relationship with HPS and SIADH should be further investigated.

We investigated the effect of granulocyte-colony-stimulating factor (G-CSF) alone (G-CSF group, n = 7) or after cyclophosphamide (CY) administration (CY+G-CSF group, n = 7) on serum lactate dehydrogenase (LDH) levels and LDH isoenzymes in a rabbit model. In the G-CSF group, an elevation of LDH (all isoenzymes) which was parallel to a sharp increase in leukocyte counts was noted on the first day of G-CSF administration. Despite the increased leukocyte counts on day 5, LDH levels were decreased. In the CY+G-CSF group, the peak elevation of LDH levels, which was prominent in isoenzymes LDH3, LDH4 and LDH5, occurred on day 5 of G-CSF administration and corresponded to the peak levels of leukocytes. In both groups, there was no correlation between LDH levels and leukocyte counts. Our data suggest that an elevation of LDH during the administration of G-CSF may be related to the differentiation of myeloid progenitors. Copyright (C) 2002 S. Karger AG, Basel.