Unmet clinical needs and challenges in the management of acute leukemia in Turkey: a modified Delphi study.

Aim: To determine the unmet needs and challenges in management, diagnosis, treatment, follow-up and patient-physician communication in acute leukemia (AL). Materials & methods: The study was based on a modified Delphi approach. A questionnaire including the major potential obstacles was circulated twice among 13 hematologists. Results: The obstacles in AL management were limited access to the novel treatments and genetic tests, limited bed capacity, insufficient level of knowledge among allied health personnel, limited availability of psycho-oncological support and low levels of awareness in the population about the importance of stem cell donation. Conclusion: The challenges in the management of AL are critical to guide the efforts to improve the quality of healthcare delivery and the evidence-based decision making at treatment of AL patients.

A single-center experience of haploidentical stem cell transplantation in hematological malignancies.

BACKGROUND: Since well-designed prospective comparative trials are lacking, haploidentical hematopoietic stem cell transplantations approach should be based on the expertise of a particular center. In this study, we aimed to report the results and outcomes of patients who underwent haploidentical hematopoietic stem cell transplantation. METHODS: : Thirty-nine patients who underwent transplantation in our clinic between 2015 and 2022 were retrospectively analyzed. Primary end point of this study is to find out the survival rates of the patients. RESULTS: The overall survival of patients was 29.9 ± 4.9 months. The disease-free survival of the patients was 37.8 ± 5.7 months. The 3-year overall survival rate of the patients was %50 and the 3-year disease-free survival rate of the patients was %53. Nineteen patients were nonsurvivors among a total of 39 patients. Busulfan-fludarabine-thiotepa was the most frequently used conditioning regimen for transplantation. Busulfan-fludarabin-antithymocyte globulin regimen is the second preferred conditioning regimen. Cyclosporine- cyclophosphamide-mycophenolate mofetil was the most widely used graft-versus-host disease prophylaxis regimen. Sixteen patients had graft-versus-host disease, 28% of the patients had acute graft-versus-host disease, and 13% had chronic graft-versus-host disease. Gastrointestinal system consists of the most involved organs in graft-versus-host disease since 15% of the patients had gastrointestinal graft-versus-host disease. First-degree relatives (parent/child) were the most frequent donor source for haploidentical hematopoietic stem cell transplantation. Sepsis was the most frequent reason of death among transplant patients. DISCUSSION: In our center, we prefer to use high dose posttransplantation cyclophosphamide after haploidentical hematopoietic stem cell transplantation for graft-versus-host disease prophylaxis. With this approach, our center's overall survival and disease-free survival rates are comparable and compatible with the literature findings.

Adenosine Deaminase Type II Deficiency: Severe Chronic Neutropenia, Lymphoid Infiltration in Bone Marrow, and Inflammatory Features.

Deficiency of adenosine deaminase type 2 (DADA2) is an autoinflammatory disease characterized with immunologic, hematologic, and neurological features. Here, we presented two patients with severe persistent chronic neutropenia, which required differential diagnosis of congenital and autoimmune neutropenia, myelodysplastic syndrome (MDS), and primary immunodeficiency diseases, including autoimmune lymphoproliferative disease. The therapy of the disease except hematopoietic stem cell transplantation is a challenging experience.

Efficacy of eltrombopag in thrombocytopenia after hematopoietic stem celltransplantation.

BACKGROUND: Thrombocytopenia is a common complication following hematopoietic stem cell transplantation (HSCT). Eltrombopag has been used in thrombocytopenia treatment after HSCT in recent years. Herein, we present our experience of 25 patients treated with eltrombopag for post-HSCT thrombocytopenia. METHODS: Fifteen autologous hematopoietic stem cell transplantation (AHSCT) and 10 allogenic hematopoietic stem cell transplantation (allo-HSCT) recipients treated with eltrombopag for treatment of prolonged isolated thrombocytopenia (PIT) or secondary failure of platelet recovery (SFPR) in the stem cell transplantation unit of Hacettepe University Hematology Department between 2017 and 2021 were included in the study. The primary endpoint of this study is eltrombopag response in patients diagnosed with PIT or SFPR. Platelet count above 50,000/mm3 for five consecutive days without platelet transfusion was considered as eltrombopag response. Overall survival (OS) analyses were calculated based on the time between HSCT and death from any cause. The patients who were alive at the last follow-up were censored at this time for calculation of OS analyses. RESULTS: AHSCT (66.7% (10/15)) and allo-HSCT (50% (5/10)) recipients responded to eltrombopag for the treatment of post-HSCT thrombocytopenia. There was no excess toxicity related to the eltrombopag use. The median response duration of allo-HSCT recipients and AHSCT recipients were 41 (13-104) days and 50 (7-342) days, respectively. There was a statistically significant OS duration difference between the responders and nonresponders in allo-HSCT and AHSCT recipients with p values of 0.005 and 0.02, respectively. DISCUSSION: Eltrombopag is promising for the treatment of thrombocytopenia after AHSCT and allo-HSCT in terms of efficacy and safety.

Long-term results of allogeneic peripheral blood hematopoietic stem cell transplantation for severe aplastic anemia.

OBJECTIVE: Aplastic anemia (AA) is a life-threatening disorder and may be associated with significant morbidity and mortality Currently, the first treatment option is allogeneic hematopoietic stem cell transplant (allo-HSCT) for patients younger than 40 years. Bone marrow is recommended as the stem cell source due to less graft versus host disease (GVHD) risk and better outcomes than peripheral blood (PB)-derived stem cell. The aim of this study is to share the data of AA patients who have underwent PB-derived allo-HSCT in our bone marrow transplantation center. METHODS: Twenty-seven patients who underwent PB-derived allo-HSCT from human leukocyte antigen matched sibling donors were analyzed retrospectively. RESULTS: The median follow-up time was 95.2 months (range, 4.8-235 months). The 10-year survival was 89 %. The median neutrophil and platelet engraftment time was 11 days (range, 9-16 days) and 13 days (range, 11-29 days), respectively. Primary platelet engraftment failure was observed in 1 patient (3.7 %). Acute and chronic GVHD observed in 2 (7.4 %) and 3 (11.1 %) patients, respectively. Neutropenic fever was observed in 13 (44.8 %) of patients until the engraftment after allo-HSCT. One patient died due to CMV infections, two died due to septic shock secondary to fungal infection. CONCLUSION: Although there is no prospective data directly comparing BM with PB as stem cell source in AA, observational studies indicates better OS with BM. PB can be used in certain situations such as higher risk for graft failure and donor preference. This study demonstrated that PB-derived stem cell seems to be a reasonable alternative to BM.

Central nervous system involvement in patients with acute myeloid leukemia

Background/aim: To evaluate the incidence, clinical features, risk factors, and prognosis of central nervous system (CNS) involvement in patients with acute myeloid leukemia (AML). Materials and methods: All AML patients who were admitted to Hacettepe University hospital between 2000 and 2021 were evaluated. The medical records of 548 AML cases were retrospectively analyzed. Results: The frequency of CNS involvement was 2.4% (n = 13) at diagnosis and 4.6% (n = 25) at diagnosis or during follow-up. Parenchymal involvement was seen in 5 patients, leptomeningeal involvement was seen in 11 patients. Three patients had both leptomeningeal and parenchymal involvements, and 6 patients had optic nerve or ocular involvement. In univariate analysis, younger age and extramedullary involvement at diagnosis were associated with CNS disease at diagnosis, and extramedullary involvement at diagnosis was associated with CNS disease during follow-up. In multivariate analysis; younger age and extramedullary involvement at diagnosis were associated with CNS disease at diagnosis and during follow-up respectively. Median overall survival was 5.4 months in patients with CNS disease at diagnosis and 16.9 months in patients with CNS disease during follow-up and 16.2 months in patients with no CNS disease. Conclusion: CNS disease is a rare complication of AML. Younger age and extramedullary involvement at diagnosis are risk factors for CNS involvement.

Assessment of ST2 and Reg3a levels in patients with acute graft-versus-host disease after allogeneic hematopoietic stem cell transplantation

Background/aim: Graft-versus-host disease (GVHD) is a crucial complication leading to significant morbidity and mortality allogeneic hematopoietic stem cell transplantation which occurs in approximately half of the transplant recipients. Suppression of tumorigenicity 2 (ST2) and regenerating islet-derived 3-alpha(Reg3a) might be important biomarkers to predict acute GVHD. Materials and methods: In the present study, blood samples were collected from 17 patients with acute GVHD and 12 control patients after allogeneic stem cell transplantation. ST2 and Reg3a were measured in plasma samples compared in patients with acute GVHD and the controls. Results: Median age of the study population was 42 years (range 19­49). When compared to controls, the mean ST2 levels was significant higher in acute GVHD (9794 ng/dL vs. 2646 ng/dL, P = 0.008). Mean Reg3a level did not show significant difference between control and acute GVHD group (8848 ng/dL vs. 5632 ng/dL, respectively, P = 0.190). Conclusion: The ST2 level might be used as a significant biomarker for predicting acute GVHD.

Lack of awareness of Hepatitis B screening and vaccination in high-risk groups

Background/aim: Hepatitis B virus (HBV) vaccination rates are insufficient in high-risk patients worldwide. This study aimed to investigate the screening, immunization, and vaccination rates in three high-risk groups for HBV infection: allogeneic hematopoietic stem cell transplantation (AHSCT), renal transplantation (RT), and chronic hepatitis C (CHC) groups. Materials and methods: The serological data of consecutive patients between 2014 and 2019 were reviewed using the hospital database. Results: The HBV screening rates were 100.0%, 90.4%, and 82.4% in the AHSCT, CHC, and RT groups, respectively (p = 0.003). The immunization rates against HBV through either previous exposure or vaccination were 79.5%, 71.7%, and 46.5% in the AHSCT, RT, and CHC groups, respectively (p < 0.001). The HBV vaccination rate was significantly low in the CHC group (71.5%, 69.0%, 34.6% in the AHSCT, RT, and CHC groups, respectively, p < 0.001). If patients lost their immunity due to immunosuppressive therapy were accounted, the vaccination rates increased to 95.2% in the AHSCT group and 72.9% in the RT group. The rate of annual screening for HBV status was 97.9% in the AHSCT group, but it was only 23.9% in the RT group. Conclusion: HBV screening and vaccination rates were significantly lower in the RT and CHC groups than in the AHSCT group.

The clinical course of COVID-19 in hematopoietic stem cell transplantation (HSCT) recipients

Background/aim: The disease caused by SARS-CoV-2 was named as COVID-19. There is as yet insufficient information about the effects of HSCT on the clinical course of COVID-19. In the present study, we aimed to investigate the clinical course of COVID-19 in patients who had undergone HSCT. Materials and methods: We analyzed baseline characteristics, clinical course and findings of COVID-19, hospitalization and death rates, overall survival, and case fatality rates of HSCT recipients diagnosed with COVID-19 retrospectively. Results: 57.6% of the patients underwent AHSCT, and 42.4% underwent allo-HSCT. 60.6%, 27.3%, and 12.1% of the patients had mild, moderate, and severe COVID-19 or critical illness, respectively. Overall, 45.5% were hospitalized, 12.1% required intensive care, and 9.1% necessitated invasive mechanical ventilation. The total CFR was 9.1% in HSCT recipients, 22.2% in patients with active hematologic malignancy, and 4.2% in patients without active hematologic malignancy. Conclusion: It can be concluded that mortality of HSCT recipients is lower in patients whose primary disease is in remission compared to ones that are not in remission. Further studies with larger group patients are needed in order to delineate the effects of COVID-19 on HSCT patients.

A unique mutation in the L ferritin coding sequence associated with low serum ferritin level in the presence of normal values of other iron parameters.

A genetic mutation was detected by our clinic in two sisters in a family with low iron levels and mild symptoms. We identified this missense mutation in the FTL gene (c.473T > C; p.Pro158Leu, rs374486686) of the sisters who had weakness symptom and low serum ferritin level. This mutation causes the codon CCG changing into CTG, thus composing an amino acid substitution in which proline 158 is replaced by leucine. The patients with this mutation had unmeasurable serum ferritin levels while other iron parameters' levels are normal. As a result of this mutation, we demonstrated that ferritin connects iron however it can not store iron.

The experience of allogeneic hematopoietic stem cell transplantation in a patient with X-linked adrenoleukodystrophy.

X-linked adrenoleukodystrophy (X-ALD), a progressive neurometabolic disorder that is caused by a defect in the gene ABCD1 (ATP-binding cassette, subfamily D, member 1), which encodes the peroxisomal ABC half-transporter ALD protein. Recently, allogeneic hematopoietic stem cell transplantation (alloHSCT) is the only therapy known to prevent disease progression. In this study, we would like to present our experience of alloHSCT for X-ALD from a HLA matched related sibling by the use of reduced intensity conditioning regimen composed of fludarabine, busulfan and ATG which allows us to reduce procedure-related toxicity and prevent mortality while achieving a curative effect.

Impact of ABO blood group incompatibility on the outcomes of allogeneic hematopoietic stem cell transplantation.

BACKGROUND AND AIM: ABO and Rh compatibility are not required between the donor and recipient for allogeneic hematopoietic stem cell transplantation (alloHSCT). Although ABO incompatibility is not considered a contraindication in alloHSCT, its clinical outcomes are still doubtful. In this study, we analyzed the neutrophil and platelet recovery, graft versus host disease (GVHD), relapse rate, mortality rate, non-relapse mortality and survival in patients who underwent alloHSCT. MATERIALS AND METHODS: Two hundred and sixty four patients with hematological malignant diseases, aplastic anemia and inborn errors of metabolism or the immune system that received an alloHSCT in our HSC transplant center between the years of 2001 and 2018 were evaluated. RESULTS: Indications for alloHSCT included both hematological malignancies (n = 233), aplastic anemia (n = 25) and benign conditions (n = 6). Of these donor recipient pairs, there were 189 (71.6%) matches, 36 (13.6%) major, 29 (11%) minor and 10 (3.8%) bidirectional ABO mismatches. The seventy-four (41.6%) of the ABO match and 27 (38.6%) of the ABO mismatch patients developed GvHD. The 5-year overall survival (OS) was ABO match group and ABO mismatch group were 65% and 73%, respectively (p = 0.36). The 5-year diasease free survival (DFS) for ABO match group and ABO mismatch group were 60% and 69%, respectively (p = 0.17). CONCLUSION: In conclusion, this study showed that ABO mismatch did not seem to have a significant effect on major outcomes after alloHSCT, such as developing GVHD, relapse rate, mortality rate, DFS and OS. ABO incompatibility did not lead to delayed platelet and neutrophil engraftment after alloHSCT.

Cardiac adverse events during stem cell transplantation for hematological malignancies: A single centre experience.

Hematopoietic stem cell transplantation (HSCT) is a highly successful treatment option for many hematological malignancies. Several adverse effects can be seen in HSCT due to the infusion and damage caused by the conditioning regimens. Cardiovascular adverse effects are relatively common during HSCT, and they have the potential to cause devastating complications. The aim of present study was to evaluate the transplantation-related cardiac adverse effects and determine the risk factors in patients undergoing HSCT at our institution. A retrospective analysis has been performed in 662 patients who was treated at Hacettepe University Stem Cell Transplantation Unit. Amongst the 622 patients, 318 (51.1 %) underwent autologous and 304 (48.9 %) underwent allogeneic HSCT. The frequency of the cardiac adverse effects was found to be 10.8 % in all the study population. The most common adverse effect was tachyarrhythmia, constituting 7.9 % of all population. These adverse effects were mostly occurred in lymphoma patients (14 %). Nineteen (3.0 %) of all patients developed atrial fibrillation mostly on the 4th day (range of 1-9 days) after transplantation. Life-threatening events are extremely rare. These adverse effects appear to be related to the type of transplantation rather than the underlying disease. Therefore, close follow-up of patients is important during the peri-transplantation period.

Predictive factors for stem cell mobilization failure in multiple myeloma patients: A single center experience.

BACKGROUND AND AIM: High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has been considered the standard of treatment care for patients with multiple myeloma (MM). Insufficient mobilization and harvest of peripheral stem cells can be a major obstacle for performing ASCT. This is resulting in a lacking opportunity of cure in patients with MM. The aim of this study was to evaluate the factors which influence mobilization failure in patients with MM. MATERIALS AND METHODS: This study has been performed in a retrospective manner. Two hundred and thirty-four patients with diagnosed MM who underwent stem cell mobilization after induction chemotherapy at Hacettepe University Hospital between the years of 2003 and 2018 were evaluated. RESULTS: A total of 234 patients were included in this study. The median age was 54 (32-76) years at the time of diagnosis. In 209 of 234 patients (89.3%) first mobilization trial was successful. At univariate analysis, among parameters identifiable before mobilization, male gender (p = 0.03), number of chemotherapy cycle before stem cell mobilization (p < 0.001), second ASCT (p < 0.001) and immunomodulatory treatment before stem cell mobilization (p < 0.001) predicted mobilization failure. At multivariate analysis, number of chemotherapy cycle before stem cell mobilization (p = 0.03), second ASCT (p < 0.001) and immunomodulatory treatment before stem cell mobilization (p = 0.02) retained independent predictive power. CONCLUSION: Detectable different clinical characteristics of MM patients before initiating mobilization may be predictors of poor mobilization. Therefore, the mobilization protocol should be evaluated on a patient basis. Minimization of exposure to chemotheraputic agents in MM patients, especially immunomodulatory agents, may increase CD34+ cell harvest yields.

The comparison of bone marrow kinetics between patients with acute myeloid leukemia and acute promyelocytic leukemia after induction chemotherapy.

Background and Aim: Recently, acute promyelocytic leukemia (APL) has shifted from the most hazardous to the best curable type of acute myeloid leukemia. Anthracyclines, all-trans retinoic acid (ATRA) and arsenic derivatives are the most important developments for the treatment of APL. ATRA promotes the terminal differentiation of malignant promyelocytes to mature neutrophils. We aimed to compare platelet and neutrophil recovery time after induction chemotherapy in patients with acute myeloid leukemia (AML) and APL.Materials and Methods: Two hundred and fifteen patients with AML and APL, who were diagnosed and treated in our tertiary care center between the years of 2001 and 2018 were evaluated.Results: One hundred and eighty one AML patients (84.2%) and 34 (15.8%) APL patients were included in this study. The time between neutrophil nadir after induction chemotherapy and neutrophil recovery was longer in APL patients than in AML patients [30.5 (4-52) vs. 20 (5-58), p < 0.001]. The time between platelet nadir after induction chemotherapy and platelet recovery was longer in APL patients than in AML patients [21.5 (4-42) vs. 17 (4-45), p = 0.02].Conclusion: Neutrophil and platelet recovery times were longer in APL patients than in AML patients in our present study. In 60 days, mortality rate was higher in APL patients than AML patients. Non-relapse mortality (NRM) rate was similar between two groups. There was a significant difference between two groups in terms of NRM causes. Platelet and neutrophil recovery time is very important because infection is the most important cause of NRM.

CD34+ hematopoietic progenitor cell dose as a predictor of engraftment and survival in multiple myeloma patients undergoing autologous stem cell transplantation

Background/aim: High-dose melphalan and autologous hematopoietic stem cell transplantation (AHSCT) is the standard treatment strategy for multiple myeloma (MM) patients who are eligible for it. The recommended dose of CD34+ hematopoietic progenitor cells (HPCs) for adequate engraftment is above 2 × 106/kg. The aim of this study was to evaluate the relationship between the dose of CD34+HPCs and survival in MM patients who underwent AHSCT at a tertiary care center. Materials and methods: Enrolled in this study were 271 MM patients who underwent AHSCT between 2003 and 2019. Clinical characteristics of the patients, disease status pre-AHSCT, reinfused CD34+ cell doses, and neutrophil and platelet engraftment days were recorded, retrospectively. The patients were divided into 2 groups according to whether the dose of reinfused CD 34+ HPCs was <5 × 106/kg or ≥5 × 106/kg. The groups were compared in terms of engraftment and overall survival (OS) times. Results: The median age of the patients was 54.8 (33­76) years. The median dose of infused CD34+ HPCs was 5.94 × 106/kg (1.47­59.5 × 106/kg). The median follow-up period was 54 months (4­211). The median OS of the patients was 103 months (11­144). The median neutrophil and platelet engraftment time was 10 (8­24) and 11 (7­40) days. Doses of <5 × 106/kg and ≥5 × 106/kg CD34+ HPC were reinfused in 38.1% and 61.9% of the patients, respectively. There was a negative significant correlation between the reinfused CD34+cell level and neutrophil/platelet engraftment times (r = ­0.32, P < 0.001; r = ­0.27, P < 0.001, respectively). The median OS times were observed as 103 months (11­144) and 145 months (123­166) for patients who had been administered <5 × 106/kg and ≥5 × 106/kg of CD34+ HPCs, respectively (P = 0.009). Conclusion: The increased amount of CD34+ autologous hematopoietic stem cell dose after high dose melphalan chemotherapy in MM patients shortened the platelet and neutrophil engraftment time and increased OS. Early platelet engraftment and administration of a CD34+ HPC count that is ≥5 × 106/kg can be considered as predictors of better survival in patients.

The effects of blood group types on the risk of COVID-19 infection and its clinical outcome

Background/aim: COVID-19 (Coronavirus disease of 2019) is an infectious disease outbreak later on declared as a pandemic, caused by the SARS-CoV-2 (severe acute respiratory syndrome coronavirus-2). It spreads very rapidly and can result in severe acute respiratory failure. The clinical studies have shown that advanced age and chronic diseases increase the risk of infection. However, influence of the blood groups on COVID-19 infection and its outcome remains to be confirmed. The aim of this study is to investigate whether there exists a relationship between the blood groups of the patients and risk of SARS-CoV-2 infection and the clinical outcomes in COVID-19 patients Material and method: 186 patients with PCR confirmed diagnosis of COVID-19 were included in this study. Age, sex, blood groups, comorbidities, need for intubation and intensive care unit follow up and mortalities of the patients were analyzed retrospectively. 1881 healthy individuals, who presented to the Hacettepe University Blood Bank served as the controls. Results: The most frequently detected blood group was blood group A (57%) amongst the COVID-19 patients. This was followed by blood group O (24.8%). The blood group types did not affect the clinical outcomes. The blood group A was statistically significantly more frequent among those infected with COVID-19 compared to controls (57% vs. 38%, P < 0.001; OR: 2.1). On the other hand, the frequency of blood group O was significantly lower in the COVID-19 patients, compared to the control group (24.8% vs. 37.2%, P: 0.001; OR: 1.8). Conclusions: The results of the present study suggest that while the blood group A might have a role in increased susceptibility to the COVID-19 infection, the blood group O might be somewhat protective. However, once infected, blood group type does not seem to influence clinical outcome.

Ghosal hematodiaphyseal dysplasia with autoimmune anemia in two adult siblings.

Ghosal hematodiaphyseal dysplasia (GHDD) is an autosomal recessive inherited disorder associated with biallelic mutations in the TBXAS1 gene located on the chromosome 7q33-34, which encodes thromboxane-A-synthase. GHDD is characterized by defective hematopoiesis due to bone marrow fibrosis and metadiaphyseal dysplasia of long bones. The accurate diagnosis of this rare syndrome is critical since it reduces the need of blood transfusions by corticosteroid therapy, leading to a significant improvement in anemia and bone changes. The aim of this study is to report two adult siblings diagnosed as GHDD, who admitted with pancytopenia and treated with steroids treatment in adult hematology clinic.

Impact of bone marrow plasma cells percentage on survival at diagnosis and pre-transplant period in newly diagnosed multiple myeloma: Experience of a single center from Turkey.

BACKGROUND AND AIM: The current definition of complete remission (CR) in multiple myeloma (MM) includes negative serum and urine immunofixation (IFE) tests and <5% bone marrow plasma cells (BMPCs). The aim of this study was to examine the impact of BMPC percentage on survival, at diagnosis and pre-transplant period, in newly diagnosed multiple myeloma. MATERIALS AND METHODS: One hundred and fourty eight patients with newly diagnosed MM who had received autologous stem cell transplantation (ASCT) in our HSCT (hematopoietic stem cell transplant) center at Hacettepe University Hospital between the years of 2008 and 2018 were evaluated retrospectively. RESULTS: The median follow-up period was 27.4 months (range, 4.5-122) for the entire group. The 3-year OS was 87% in the pre-transplant BMPCs <5% group and 92% in the pre-transplant BMPCs ≥ 5% group, there was no statistically significant difference. The 5-year OS for the pre-transplant BMPCs <5% group and the pre-transplant BMPCs ≥ 5% group were 73% and 70%, respectively (p = 0.50). The 3-year PFS in the pre-transplant BMPCs <5% group and the pre-transplant BMPCs ≥ 5% group were 77% and 57%. The 5-year PFS in the pre-transplant BMPCs <5% group and the pre-transplant BMPCs ≥ 5% group were 43% and 13%, respectively. There was a statistically significant difference between the two groups with respect to PFS (p = 0.04). CONCLUSION: In conclusion, this study highlights the importance of reaching <5% BMPCs at pre-transplant period. OS and PFS were better in patients who had pre-transplant BMPCs <5% than pre-transplant BMPCs ≥ 5%.

Unclassifiable non-CML classical myeloproliferative diseases with microcytosis: findings indicating diagnosis of polycythemia vera masked by iron deficiency

Background/aim: Polycythemia Vera (PV) is a myeloproliferative disorder characterized by overproduction of morphologically normal red blood cells (RBCs), granulocytes, and platelets, a phenotype that is caused by a mutation (V617F) in Janus kinase 2 (JAK2). However, JAK2 V617F is also found in approximately 50% of patients with essential thrombocytosis and primary myelofibrosis, rendering its presence nonspecific as a diagnostic test. An increased red cell mass is a major criterion for the diagnosis of PV according to World Health Organization (WHO) 2016 criteria. High hemoglobin (Hgb) or Hematocrit (Hct) are universally used as indicators of an increased red cell mass for the diagnosis of PV. However, conditions such as iron deficiency (ID) with decreased mean cell volume may mask the diagnosis due to nonelevated Hct level. The aim of this study was to investigate the clinical characteristics of the patients with unclassifiable non-CML classical myeloproliferative disease with microcytosis (MPD/M) and nonelevated Hgb and Hct levels at diagnosis and to determine if some of these cases could be real PV cases masked due to ID-related microcytosis. Materials and methods: There were 23 MPD/M cases among 208 non-CML classical MPD cases (11%). Among 22 patients who had adequate test results related to the cause of microcytosis, ID and beta-thalassemia trait (TT) were the apparent causes of microcytosis in 15 and 1 cases, respectively. Results: Clinicopathological correlations revealed consistently positive JAK2 V617F mutation status (20/20, 100%), frequently elevated RBC count (17/23, 73.9%), and PV-compatible bone marrow findings (10/12, 83.3%). These findings are compatible with PV instead of essential thrombocytopenia or primary myelofibrosis. In spite of frequent cytoreductive treatment, 3 patients developed increased Hgb/Htc levels during median 58.2 (279­63) months' follow-up. Conclusion: These data show that the majority of MPD/M cases are PV patients masked due to ID-related microcytosis.