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OBJECTIVE: Relapsed/refractory AML cases are much more resistant to chemotherapy. Venetoclax is a highly sensitive BCL-2 inhibitor. It was aimed to evaluate the effects of venetoclax therapy on real-world R/R AML survival outcomes, the effects of the cytogenetic characteristics of the patients and previous clinical applications on treatment response, and venetoclax treatment toxicity. PATIENTS AND METHODS: The study included patients who only received a venetoclax-based salvage on R/R AML patients from Turkey. The study included a total of 62 patients from 6 different centers in Turkey. Response to 2 cycles of venetoclax treatment was assessed by bone marrow blast rate. The demographic data, cytogenetic characteristics, AML type, MDS type, response rates and overall survival of the patients after venetoclax combination treatment were assessed. Median age of the patients was 65 (19-85). Mean number of prior treatments was 2.67 ±1.75. RESULTS: 13 patients (21%) had a history of allogenic stem cell transplantation. 58 (93.5%) had received HMA therapy before venetoclax. 36 patients (58.1%) had de-novo AML, and 25 (40.3%) previously had MDS. Treatment response was evaluated as complete remission (n = 21, 33.9%), partial response (n = 17, 27.4%), and treatment failure (n = 24, 38.7%). Patients in the TF group were significantly more likely to have poor cytogenetic and to have received allogeneic transplants. The mean estimated overall survival after the venetoclax treatment was 9.13 ± 0.75 months. CONCLUSIONS: The study population consisted of a group of patients who had relapsed or primary refractory disease with poor prognosis, despite numerous rounds of chemotherapy. It is our belief that the high response rates obtained with the combination of venetoclax/HMA, and having obtained positive results with poor risk patients, indicated a promising perspective for R/R AML patients. [ABSTRACT FROM AUTHOR]

Background: This study planned to investigate the relationship of dynamic thiol/disulfide homeostasis with the prognosis of myelodysplastic syndrome (MDS). Methods: 80 patients who had been diagnosed with MDS between 2012 and 2017 and who were older than 18 were included in the study together with 80 healthy control subjects. The MDS diagnosis was confirmed using bone marrow aspiration-biopsy immunostaining. Dynamic thiol/disulfide homeostasis and ischemia-modified albumin (IMA) levels were examined. Results: The average IMA (0.71±0.08 vs. 0.67±0.09; p=0.002), median disulfide (18.0 vs. 11.6; p

Background: The purpose of this study is to investigate whether or not reticulocyte hemoglobin equivalent (RETHe) is a superior indicator of blood count and other iron parameters in terms of diagnosing iron deficiency (ID) and iron deficiency anemia (IDA), and thus evaluating a patient's response to oral iron treatment. M ethods: The research population consisted of 217 participants in total: 54 control, 53 ID, 58 non-ID anemia, and 52 IDA patients. A hemoglobin (Hb) value of < 130.0 g/L was defined as indicating anemia for men, while an Hb value of < 120.0 g/L was defined as indicating anemia for women. All patients were administered 270 mg oral elemental iron sulphate daily. Results: The RET-He was significantly lower in the IDA group, compared to other groups (IDA: 21.0 ± 4.1, ID: 26.0 ± 4.9, non-ID anemia: 32.1 ± 6.8, control: 36.6 ± 7.0; < 0.001). The ID group had a lower RET-He compared to the non-ID anemia group and the control group. On the 5th day of treatment, the ID and IDA group showed no significant differences in terms of Hb while the RET-He level demonstrated a significant increase. The increase in the RET-He level observed in the IDA group on the 5th day was significantly higher compared to the increase observed in the ID group. A RET-He value of 25.4 pg and below predicted ID diagnosis with 90.4% sensitivity and 49.1% specificity in IDA patients, compared to the ID group. Conclusions: The results of our study, therefore, suggest that RET-He may be a clinically useful marker in the diagnosis of ID and IDA.

Objective: Acute graft versus host disease (GVHD) occurs in 20-80 % of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Of these patients, 40 % will be resistant to steroids, which is the standard first-line approach. There is no standard second line treatment approach for patients with steroid refractory acute GVHD (SR-aGVHD). Alpha-1 antitrypsin is a protease inhibitor and has anti-inflammatory and immune regulatory properties. Here we report the outcomes and safety data of 17 patients treated with alpha-1 antitrypsin for SR-aGVHD., Material and Methods: Patients who received at least 2 lines of alpha-1 antitrypsin treatment for SR-aGVHD at five transplant centers in Türkiye were included in this retrospective study., Results: The median number of alpha-1 antitrypsin treatment line patients received was 4 (range, 2-5). The median time between alpha-1 antitrypsin administration and response was 65 days (range, 10-138 days). Overall response rate was 70.6 %. When the first- and second-month response rates were compared according to GVHD organ involvement, we found that the response rates were similar in skin, liver and gastrointestinal system involvement (p = 0.281 and p = 0.305, respectively). No grade 3-4 anemia, thrombocytopenia or neutropenia was observed after alpha-1 antitrypsin treatment. Two patients had cytomegalovirus infection and 1 patient had pneumonia. At a median follow-up of 7 months, overall survival was 70.6 % and median overall survival was not reached., Conclusion: In conclusion, alpha-1 antitrypsin is an effective and safe treatment option in patients with SR-aGVHD, with response rates of up to 70 % in patients with skin, liver and gastrointestinal system involvement. Larger studies are needed to establish a standard second and subsequent treatment approach in patients with SR-aGVHD., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: Therapeutic plasma exchange has been a well-known treatment method for many years and is widely available. It leads to the improvement of neurological symptoms in autoimmune neurological diseases by the removal of antibodies. The aim of this study was to present therapeutic plasma exchange responses and procedure-related adverse events in patients with autoimmune neurological diseases based on our 11-year experience., Method: A retrospective evaluation was conducted on adult patients who underwent a therapeutic plasma exchange procedure due to neurological diseases between January 2013 and January 2024. Data were gathered from electronic and written hospital and apheresis unit records., Results: A total of 265 patients underwent 1274 procedures with a preliminary diagnosis of autoimmune neurological disease. Five patients were excluded from the analysis due to their final diagnoses. The most common clinical indications were Guillain-Barré syndrome (45.4%), myasthenia gravis (26.1%), and multiple sclerosis (19.2%). The overall response rate was 81.3%, with 21.7% exhibiting a complete response and 59.6% demonstrating a partial response. With the exception of one patient (hypertensive crisis), no complications necessitating the termination of the procedure were observed. The most prevalent complication was an easily manageable allergic reaction., Conclusion: Therapeutic plasma exchange has been demonstrated to be an efficacious and safe treatment option in autoimmune neurological diseases, with a favorable overall response rate and a manageable mild-to-moderate side effect profile., (© 2024 International Society for Apheresis and Japanese Society for Apheresis.)

Introduction: Thrombocytopenia is among the most common complications following hematopoietic stem cell transplantation and is associated with increased mortality and morbidity with no standard treatment yet. In this multicenter and retrospective study, we aim to present our multi-center experience of Eltrombopag treatment in patients with isolated thrombocytopenia following HSCT., Material-Method: A total of 73 patients from 5 centers who underwent autologous or allogeneic stem cell transplantation, had no primary disease relapse, all of whom had neutrophil engraftment, complete chimerism, and who were diagnosed with Prolonged Isolated Thrombocytopenia (PIT) or Secondary Failure Of Platelet Recovery (SFPR) were included in the study. The patients were initiated on Eltrombopag at a dose of 50-150 mg. Complete response was defined as a platelet count >50×10 9 /L for 7 consecutive days with no transfusion support., Results: A total of 50.3% of the patients underwent Autologous and 49.7% Allogeneic Stem Cell Transplantation, 54.8% were diagnosed with PIT, and 45.2% were diagnosed with SFPR, and the treatment with 50-150 mg/day Eltrombopag was initiated on the median day +42. Complete response was achieved in 71.2% of these patients on the median day 23 of the treatment. No significant effects of the initial dose (50-150 mg/day) were detected in the Complete Response in the multivariate analysis on response. An insufficient number of Megakaryocytes in the bone marrow before Eltrombopag treatment was determined as an independent risk factor in determining the response (OR 3.57, 95% CI 1.21-10.55). The overall survival of the patients who did not respond to Eltrombopag was found to be significantly worse than that of patients who responded (p=0.022, HR:2.74, 95% CI 1.12-6.54)., Conclusion: As a result of the present study, Eltrombopag treatment was found to be effective and safe in thrombocytopenia that develops following hematopoietic stem cell transplantation. It was concluded that its use may be more effective in patients with sufficient bone marrow megakaryocytes before the treatment and an initial dose of 50 mg/day may be appropriate in terms of cost, effectiveness, and toxicity. Large-scale randomized and controlled prospective studies are needed to determine the roles of Eltrombopag treatment in patients with post-transplant PIT and SFPR., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: Myelofibrosis is reported in around 40% of newly diagnosed chronic myeloid leukemia (CML) patients and have an important role in the pathobiology and prognosis of CML. This retrospective study aimed to evaluate the effects of bone marrow (BM) fibrosis on disease prognosis and the effects of specific tyrosine-kinase inhibitors (TKIs) on BM fibrosis in CML patients., Methods: The study included 96 patients (>18 years) diagnosed with chronic phase (CP) CML. The clinical and demographic information were collected from the medical files. Post-treatment BM aspirate and core biopsy samples were analyzed for the presence of fibrosis and dysplasia., Results: The mean age of the study patients was 52.69 years; 47.9% of the patients were female. At the onset, 53 (63.1%) patients had BM fibrosis. The difference in the overall survival of the patients with respect to BM fibrosis grades was significant (p = .001). Within the BM fibrosis grade groups, there were significant differences between grade 0 vs. grade 2, grade 0 vs. grade 3, and grade 1 vs. grade 3 (p = .005, p = .002, and p = .003 respectively) There was no significant association between the presence of BM fibrosis at the onset and not responding to first-line therapy (p = .724). Moreover, no significant association was found between the presence of BM fibrosis at the onset and molecular (p = .623) or cytogenetic response (p = .535) to first-line therapy. Additionally, the association between the type of second-line and third-line therapy and molecular response (p = .773 and p = .424, respectively) or cytogenetic response (p = .298 and p = .641) was not significant., Conclusion: Although BM fibrosis seems to be a crucial complication of CML with a poor prognosis, it can be reversed via TKI treatment which may result in improved survival. It might be considered to check the BM for this complication on a regular basis during therapies to test its prognostic influence in CML patients in prospective controlled trials. Further studies focused on this issue are required to utilize BM fibrosis as a candidate prognostic factor., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: We present a case of bilateral maculopathy associated with deferoxamine mesylate (DFO) treatment., Methods: A 53-year-old man with myelodysplastic syndrome (MDS) received DFO therapy due to elevated ferritin levels. He was then referred to ophthalmology clinic due to blurred vision. He was diagnosed as bilateral neurosensory retinal detachment of the macula. During follow up, best corrected visual acuity (BCVA), optical coherence tomography (OCT), fundus fluorescein angiography (FFA), and fundus autofluorescence (FAF) were evaluated., Results: At first visit, OCT showed bilateral foveal neurosensory detachment. Hyperfluorescence of the macula and the peripapillary region were found on FFA. After discontinuation of DFO, BCVA improved from 20/120 to 20/60 with resolution of the foveal detachments on OCT scan. Four weeks later, FAF showed bilateral mottled hyperautofluorescence and hypoautofluorescence at the macula and the peripapillary region., Conclusion: Deferoxamine can cause acute retinal toxicity. Haematologists should be alert to visual complaints associated with DFO therapy, as early diagnosis and discontinuation of the medication allows recovery of visual function with residual fundus findings., Competing Interests: Declarations of Competing Interest None., (Copyright © 2023. Published by Elsevier B.V.)

Introduction: The availability of a clinical decision algorithm for diagnosis of chronic lymphocytic leukemia (CLL) may greatly contribute to the diagnosis of CLL, particularly in cases with ambiguous immunophenotypes. Herein we propose a novel differential diagnosis algorithm for the CLL diagnosis using immunophenotyping with flow cytometry., Methods: The hierarchical logistic regression model (Backward LR) was used to build a predictive algorithm for the diagnosis of CLL, differentiated from other lymphoproliferative disorders (LPDs)., Results: A total of 302 patients, of whom 220 (72.8%) had CLL and 82 (27.2%), B-cell lymphoproliferative disorders other than CLL, were included in the study. The Backward LR model comprised the variables CD5, CD43, CD81, ROR1, CD23, CD79b, FMC7, sIg and CD200 in the model development process. The weak expression of CD81 and increased intensity of expression in markers CD5, CD23 and CD200 increased the probability of CLL diagnosis, (p < 0.05). The odd ratio for CD5, C23, CD200 and CD81 was 1.088 (1.050 - 1.126), 1.044 (1.012 - 1.077), 1.039 (1.007 - 1.072) and 0.946 (0.921 - 0.970) [95% C.I.], respectively. Our model provided a novel diagnostic algorithm with 95.27% of sensitivity and 91.46% of specificity. The model prediction for 97.3% (214) of 220 patients diagnosed with CLL, was CLL and for 91.5% (75) of 82 patients diagnosed with an LPD other than CLL, was others. The cases were correctly classified as CLL and others with a 95.7% correctness rate., Conclusions: Our model highlighting 4 markers (CD81, CD5, CD23 and CD200) provided high sensitivity and specificity in the CLL diagnosis and in distinguishing of CLL among other LPDs., Competing Interests: Conflicts of interest The authors have no competing interests., (Copyright © 2021 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U. All rights reserved.)

Objective: The aim of this study was to compare the incidence of factors associated with an increased risk of thrombosis in patients with essential thrombocythemia., Methods: A total of 200 patients followed-up in our unit with a diagnosis of essential thrombocythemia in 13 years were analyzed retrospectively., Results: Of the study participants, 60.5% were females and 39.5% were males, with an overall mean (±SD) age of 54.93 (±14.21) years. In 119 patients, Janus Kinase 2 was positive with 56.3% of cases. When two patient categories were defined as those with or without history of thrombosis, no significant differences were found in terms of Janus Kinase 2 positivity, mean age, as well as white blood cells and platelet counts (p>0.05). Also, no significant differences in thrombotic event incidence were found between patient categories defined on the basis of cut-off values for white blood cells (cut-off values of 15×103/mm3 and 8.7×103/mm3) and platelets (cut-off values of 1500×103/mm3) (p>0.05)., Conclusion: Although our results are generally in line with the published data, some divergence from previous results has been observed with respect to risk factors for thrombotic events. Absence of a correlation between leukocytosis and thrombosis may be related with the significant decline in white blood cells after treatment. Also, a significant reduction in platelet counts occurring in association with treatment is linked with a lowered incidence of thrombosis. Janus Kinase 2-positive patients had a similar thrombosis frequency with that reported in the literature.

There are different drug combinations and conditioning regimens in lymphoma transplants. However, no randomized data is available to demonstrate the superiority of any regimen and the optimal choice is unknown. In this analysis, we compared the efficacy, toxicity and the survival outcomes of the BEAM and the high dose ICE (hdICE) conditioning regimens in relapsed NHL and relapsed/refractory Hodgkin Lymphoma patients undergoing auto-SCT. 83 patients with relapsed/refractory HL or relapsed NHL who were treated with Auto-SCT between 2006 and 2016, were analyzed retrospectively. 52 patients (62.7%) received BEAM, while 31 patients (37.3%) received hdICE. Between two groups there is no significant difference in age, gender, diagnosis, disease stage, chemosensitivity, ECOG performance status, time from diagnosis to transplant, salvage regimens and previous lines of chemotherapy. After a median of 59-month follow-up, PFS and OS rates of both groups were similar (5-year PFS was 51.6% in BEAM group, 48.8% in hdICE group, p  = 0.71; 5-year OS was 58% in BEAM group, 54.8% in hdICE group, p  = 0.93). The median neutrophil (11 vs. 10 days, p  = 0.06) and platelet engraftment (13 vs. 11 days, p  = 0.01) was faster and demand of transfusions were lesser in hdICE group ( p  = 0.03). However, severe renal toxicity was significantly higher in hdICE group in our study ( p  = 0.01). hdICE conditioning regimen may be used as an alternative to BEAM, with similar survival outcomes and toxicity profile, especially transplant centers that experience some difficulties in the availability of the carmustine., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest., (© Indian Society of Hematology and Blood Transfusion 2020.)

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 < 200.5 IU (P = .010)., 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., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Stem cells are collected from donors and infused to the recipient in allogenic peripheral stem cell transplantations. The use of frozen stem cells can promote donor compatibility, and overcoming possible problems due to insufficient stem cell mobilization will also be easier. Nevertheless, studies about the use of frozen peripheral stem cells in allogenic transplantation are extremely rare. In this study, we aimed to compare the clinical outcomes of allogenic stem cell transplants from frozen or fresh stem cell products., Materials and Methods: This retrospective analysis was conducted between April 2004 and September 2018 in the bone marrow transplantation unit of Ankara Numune Training and Research Hospital. Clinical data of patients who received allogenic peripheral stem cell transplantations from fully matched sibling donors were compared for 42 fresh and 30 frozen stem cell transplants., Results: While the platelet engraftment period, febrile neutropenia period, hospitalization period, and 100-day mortality rates did not show any differences, the neutrophil engraftment period was longer in the frozen group (mean: 14 days vs. 16 days, p = 0.006). Acute and chronic graftversus-host disease (GVHD) rates were similar in both groups; however, the rate of grade 3 or4 chronic liver GVHD was slightly higher in transplants performed with fresh stem cells compared to the frozen group (p = 0.046). Overall survival was similar between the groups (p = 0.700)., Conclusion: The use of frozen peripheral stem cells in allogenic stem cell transplantation may be a reasonable option that can be applied without causing a significant change in clinical results., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

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 ≤ 0.1% on the International Scale [BCR-ABL1 IS ]) by 12 months, was previously reported (66.1% [80% CI, 59.7%-72.0%]). ClinicalTrials.gov identifier NCT01274351 Results: By 24 months, 83.0% of patients achieved MMR, and 50.9% achieved MR 4.5 (BCR-ABL1 IS ≤0.0032%). Safety results at 24 months were consistent with those at 12 months. No additional deaths or disease progressions to accelerated phase/blast crisis were observed between 12 and 24 months., Discussion: Treatment with nilotinib 300 mg twice daily for 2 years provided high MMR with a good safety/tolerability profile in newly diagnosed CML-CP patients in Turkey. Assessment of MMR across time points showed increasing rates through 18 months, after which as lower rate of increase was observed. The safety profile of nilotinib 300 mg twice daily with 24 months of follow-up was similar to that observed at 12 months, and no new safety concerns were identified. These efficacy and safety findings are consistent with the results from the 12-month analysis of this study and from previous nilotinib studies. These findings support nilotinib as an option for frontline treatment of CML-CP., Conclusion: Frontline nilotinib treatment provided sustained efficacy, with good tolerability, over 24 months in newly diagnosed CML-CP patients.

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-ABL1 IS ]) 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 MR 4.5 (BCR-ABL1 IS ≤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.

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-ABL1(IS)]) 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 MR(4.5) (BCR-ABL1(IS) ≤ 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.

Background: In recent years, the clinical and biological features governing the clinical course of chronic lymphocytic leukemia (CLL) have been most extensively studied. Human leukocyte antigen-G (HLA-G) allows tumor cells to escape from the antitumor effect of the immune system. Recent studies have shown that various tumor cells show an increased HLA-G expression. Data regarding HLA-G expression in CLL are limited and controversial. The aim of this work is to evaluate flow cytometry study of HLA-G expression on cell surface and assess its relationship with other prognostic factors (CD38, ZAP70, beta 2 microglobulin [β2MG]) in patients with CLL., Design and Methods: Forty-five newly diagnosed CLL cases. White blood cell count, lymphocyte absolute count, hemoglobin level, platelet count, serum lactate dehydrogenase activity, and serum β2MG level were studied at admission. In each patient, morphologic diagnosis of B-CLL was confirmed by flow cytometry HLA-G, CD38 and ZAP70 expression levels were measured with four-color flow cytometry., Results: HLA-G positivity ranged between 1% and 12% in CLL patients. A significant correlation was found with CD38, ZAP70, disease stage, and β2MG (P < 0.001). The off-treatment follow-up period was longer in the HLA-G negative group (P < 0.022)., Conclusions: In conclusion, we suggest that, in addition to other prognostic factors, surface HLA-G expression can be considered as an independent prognostic factor. However, our work should be confirmed by further prospective studies, a longer off-treatment follow-up period, and a standardized method., (© 2015 Wiley Periodicals, Inc.)

Background: In this study, we aimed to determine a cutoff level for CD38 that would aid us in identifying chronic lymphocytic leukemia patients in need of early therapy and predicting patients at sufficiently low risk who would likely exhibit a rapid improvement; we also aimed to find out if CD38 expression would show variability during disease course and determine the extent of CD38 expression., Methods: 124 patients were diagnosed with CLL. CD38 and ZAP-70 expression levels were measured with four color flowcytometry. Time from diagnosis to initial therapy was calculated for all patients. CD38 expression was studied for a second time during follow-up in 50 patients., Results: For cutoff levels of 7%, 20%, and 30%, CD38 expressions were 61.3%, 25%, and 24.2%, respectively. At all three cutoff levels there were significant correlations with all parameters except age between CD38+ vs. CD38- groups (p < 0.001). The comparative rates of starting therapy for cutoff levels of 7%, 20%, and 30% in CD38+ and CD38- groups were 77.5% vs. 6.25%; 100% vs. 30.7%, and 100% vs. 31.5%, respectively (p < 0.001). Multiple Cox Proportional Hazards Regression analysis: for a cutoff level of 7%, survival was affected by STAGE, ZAP70, and CD38., Conclusions: A CD38 cutoff level of 7% determined by standardized laboratory techniques is an important prognostic marker. However, the number and frequency of repeat measurements of CD38 expression, and cutoff level of CD38 expression that significantly predict disease prognosis should be further determined by future cohort studies.

Multiple myeloma has been associated with the development of thromboembolic events. Thrombin activatable fibrinolysis inhibitor (TAFI) is a carboxypeptidase B-like proenzyme, which potently inhibits fibrinolysis. The purpose of the present study was to assess the TAFI levels in patients with newly diagnosed multiple myeloma. Twenty-seven newly diagnosed multiple myeloma patients (16 women and 11 men) and 27 age-matched healthy individuals (14 women and 13 men) were included in the study. Serum TAFI levels were significantly increased in patients with multiple myeloma (46 ± 13. 3 vs. 36. 6 ± 9.7 μg/ml) compared with healthy individuals. Serum TAFI levels were negatively correlated with serum albumin (CC: -0.453, P < 0.05) and hemoglobin levels (CC: -0.392, P < 0.05) and positively correlated with the β-2 microglobulin levels (CC: 0.524, P < 0.05). In this study, we observed significantly elevated TAFI levels in patients with multiple myeloma and higher serum TAFI levels were suggested to be associated with higher disease stage. With these results, a possible role of elevated TAFI levels in thromboembolic manifestations in the course of multiple myeloma can be suggested.

Objective: Immune thrombocytopenic purpura (ITP) is also known as idiopathic thrombocytopenic purpura. Increased platelet destruction and insufficient platelet production are both responsible for its etiopathogenesis. ITP can be diagnosed after excluding other possible causes of thrombocytopenia., Materials and Methods: One hundred forty-three cases of chronic ITP that were monitored in a hematology clinic were retrospectively evaluated. All cases received first line treatment of 1 mg/kg/day prednisolone. Corticosteroid nonresponsive (CN) cases and corticosteroid-dependent (CD) cases underwent splenectomies., Results: The rate of CN/CD cases was found to be 53% (n=76). Sixty-six percent of these cases (n=50) underwent splenectomies. The ratio of non-responsive cases to relapse cases after splenectomy (SN/SR) was 30% (n=15). The total number of cases was 41, including those without splenectomy (n=26) and with SY/SR (n=15). Helicobacter pylori (Hp) eradication, immunosuppressive agents and danazol treatments were administered to patients (n=10, n=14 and n=4, respectively). Currently, 13 patients are being monitored without treatment. Fifteen patients who were non-responsive to Hp eradication treatment, immunosuppressive treatment or danazol treatment are still being monitored without any treatment., Conclusion: Optimal treatment is not available for splenectomy-resistant cases of ITP. The response rates for Hp eradication treatment, immunosuppressive treatments and anabolic agents are low. Therefore, larger studies with more patients are required using new agents, such as thrombopoietin (TPO) receptor agonists and anti-CD20 monoclonal antibodies.

The increased risk for thrombosis is known as hypercoagulability or thrombophilia. Here, we investigated risk factors for thrombophilia which were screened in young adult patients presenting with thrombotic events or with recurrent abortions with unknown etiology. A total of 115 patients aged between 16 and 50 years who were found to harbor thrombophilia were retrospectively evaluated. The laboratory investigations performed for the assessment of thrombophilia included protein C, protein S, antithrombin III deficiencies, activated protein C resistance, factor V Leiden (FVL), prothrombin 20210A (PT 20210) and methylenetetrahydrofolate reductase (MTHFR) gene mutations, factor VIII elevation, lupus anticoagulant and antiphospholipid antibodies (APA). In 66% of the cases a single thrombophilic defect was identified while some of the patients had combined thrombophilic defects. The most common thrombophilic defect was mutation in the MTHFR gene, and was followed by FVL mutation, the presence of APA and PT 20210 gene mutation, respectively. The patients were divided into two different age groups, 16-35 and 36-50 years, and arterial thrombosis was more common in the older age group. Our results indicated that some important thrombophilic defects such as gene mutations may appear in young adult patients presenting with thrombotic events.

Extramedullary involvement in the absence of bone marrow disease is rare in patients with acute promyelocytic leukemia (APL). We report two patients with APL who had central nervous system (CNS) relapse without evidence of cytologic and molecular disease of bone marrow after all-trans-retinoic acid (ATRA) treatment. Both of the patients were treated successfully with combination of intrathecal chemotherapy and radiotherapy with or without systemic chemotherapy. Although increasing number of cases with extramedullary involvement of APL after ATRA including therapy have been reported, further studies with a large series of patients are necessary to determine whether ATRA increases the risk of development of extramedullary involvement of disease in patients with APL.

A 42-year-old woman was diagnosed with myelodysplastic syndrome with fibrosis that developed bilaterally, cervical lymphadenopathy and cutaneous infiltration by trilineage extramedullary hematopoiesis after granulocyte colony-stimulating factor therapy because of severe neutropenia. Hepatosplenomegaly was not observed during her follow-up. Extramedullary hematopoiesis disappeared after growth factor therapy was stopped. Although the neutropenia was alleviated by growth factor administration, the appearance of an unusual involvement of extramedullary hematopoiesis should be kept in mind., (Copyright 2006 S. Karger AG, Basel.)

Background: Reactivation of hepatitis B in patients receiving chemotherapy for acute leukemia may give rise to a variety of clinical patterns including hepatitis, asymtomatic hepatic dysfunction, massive hepatic necrosis and fatal hepatic failure. Lamivudine is a nucleoside analogue which can directly suppress Hepatitis B virus (HBV) replication. We reviewed our combined experience to evaluate the role of lamivudine as prophylaxis in acute leukemia patients who were HBsAg carriers treated with chemotherapy between July 2000 and October 2002 at the Numune Education and Research Hospitals (Ankara, Turkey) retrospectively., Methods: We investigated 75 acute leukemia patients who received chemotherapy. Thirteen (17.3%) of 75 acute leukemia patients were HbsAg positive and of 7 (53.3%) were HBV DNA positive. Two patients (patients 5 and 6) had a chemotherapy regimen that included corticosteroids and were HBsAg and HBV DNA negative but anti HBc total positive. HBsAg positive patients with or without HBV DNA positivity were treated with a dose of 100 mg/day lamivudine commencing when chemotherapy was initiated. Lamivudine started at the beginning of chemotherapy and was maintained for 6 months following the cessation of chemotherapy. During lamivudine treatment, Aspartate aminotransferase (AST), Alanine aminotransferase (ALT), Gama glutamile transpeptidase (GGT), Alkaline phosphatase (ALP) were followed., Results: Of the 8 patients who presented with hepatic dysfunction during the first chemotherapy cycle, 4 improved during the second course. After completing chemotherapy, the levels of hepatic enzymes were in the normal range in all but one patient. With lamivudune prophylaxis, HBV DNA positivity did not develop in any of the HBV DNA negative patients. The two patients who received corticosteroids with their first chemotherapy cycle became positive for HBsAg and HBV DNA and were given Lamivudine when the seroconversion was established. Median follow up from the diagnosis of leukemia was 14.5 months. Survival rate at the end of follow up was 5 (38%) for the 13 patients., Conclusions: As this infection is endemic in our country and the exposure to blood products is high in these patients, HBV infection is more common. Prophylaxis with daily administration of lamivudine to HBsAg carriers who are candidates for chemotherapy seems to be effective and may prevent chemotherapy induced HBV reactivation and hepatic failure.