The assessment of health-related quality of life in patients with polycythemia vera.

Previous studies have shown that patients with polycythemia vera (PV) have poor quality of life (QoL). Similarly, it has been shown that survival is influenced by QoL. We aimed to evaluate QoL in 88 Turkish patients with PV. This cross-sectional study included cases diagnosed with PV between January 1995 and August 2019 who attended follow-up studies in the hematology department of a tertiary hospital in Türkiye between August 2019 and July 2020. Beginning in August 2019, subjects who approved study participation applied the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30) questionnaire during their routine follow-up-given that they met inclusion/exclusion criteria. Individuals with comorbidities or factors influencing QoL and those with secondary PV-related conditions were excluded. Recorded data included age, sex, history of bleeding, thrombosis, erythrocytosis, leukocytosis, thrombocytosis, obesity or splenomegaly, and cytogenetic mutation profiles such as JAK2, BCR and MPL. We also assessed whether they needed phlebotomy or erythrocyte suspensions. Data concerning comorbidities and medication use were obtained from medical records. The median age of patients was 52 (44-61) years. The majority of participants were male (67.05%). Global health status score was 75 (66.67-83.33). PV patients who had required phlebotomy demonstrated higher social functioning scores (P = .004) and lower scores for loss of appetite (P = .013) and financial difficulties (P = .020) than patients without phlebotomy. PV patients who had suffered from leukocytosis demonstrated lower physical functioning scores compared to those without leukocytosis (P = .001). Patients without JAK2 exon 14 mutations had better physical (P = .016) and cognitive functioning scores (P = .048). It was found that PV patients with splenomegaly demonstrated lower physical functioning (P = .019) and higher appetite loss scores (P = .005) than those without splenomegaly. Higher leucocyte counts were associated with decreased physical functioning and greater fatigue. In conclusion, we demonstrated deterioration of physical and emotional QoL in patients diagnosed with PV. Patients with PV require individualized, patient-specific and integrated approaches in order to minimize symptoms, improve QoL, and increase survival.

The Obesity Controversy: Does It Impact Treatment Response in Diffuse Large B-Cell Lymphoma?

Background: We aimed to investigate the association of body mass index (BMI) with treatment response in patients with DLBCL. Material and Methods: Seventy-nine DLBCL subjects were included in this study. Data about patient age, sex, serum LDH level, presence of B symptoms, IPI score, ECOG performance score, disease stage, extranodal involvement, and BMI values at diagnosis were retrieved by retrospective patient record review. Patients were staged according to Ann Arbor classification using CT and/or PET/CT findings, and the presence of B symptoms. Body mass index was calculated by dividing weight in kilograms by height in meters squared (kg/m2). Patients were divided into groups according to their BMI as underweight (BMI≤ 18.5 kg/m2), normal weight (BMI 18.5-25 kg/m2), overweight (BMI 25-30 kg/m2), and obese (BMI≥ 30 kg/m2), as defined by the World Health Organization. Results: Patients were divided into four groups according to their BMIs, but because there was only one patient in the underweight group, comparisons were performed between normal-weight, overweight, and obese patients. There was no statistically significant difference between these groups in terms of age, sex, serum LDH level, disease stage, presence of B symptoms, extranodal involvement, ECOG performance score, IPI score and treatment response (p= 0.070, 0.704, 0.325, 0.464, 0.254, 0.152, 0.658, 0.620, and 0.947, respectively). Conclusion: In our study, we showed that BMI has no significant impact on treatment response in patients with DLBCL.

Charlson Comorbidity Index (CCI) in Diffuse Large B-cell Lymphoma: A New Approach in a Multicenter Study.

Purpose: Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of adult lymphomas. The incidence of DLBCL increases with age and has a fairly rapid fatal course without treatment. Patients often have difficulty tolerating standard chemotherapy regimens due to their comorbidities. Charlson Comorbidity Index (CCI), which is calculated by considering 19 different comorbidities, was developed in 1987 and is widely used for mortality prediction in cancer patients. Literature data on CCI and hematological malignancies are limited. Main aim in this study is to evaluate the effectiveness of CCI and compare to the International Prognostic Index (IPI) scoring system in the DLBCL patient group. Methods: A total of 170 patients diagnosed with DLBCL between 1.1.2002- 1.12.2020 were included in the study. Statistical analyzes were performed among patients whose IPI and CCI scores were recorded by considering baseline data. Results: The median age of patients was 58 (range: 17-84). Thirty-five (20.6%) patients had stage III and 76 (44.7%) had stage IV disease. When the CCI, IPI and ECOG scores were compared with the mortality status of the patients as a reference, AUCs were resulted as 0.628 (95% CI: 0.506-0.749), 0.563 (95% CI: 0.484-0.639) and 0.672 (95% CI: 0.596-0.743), respectively. There was no significant difference between the ROC curves of CCI, IPI and ECOG scores. Patients with a CCI score of ≥ 4 had shorter OS comperad to those with a score of < 4. Conclusion: Rather than claiming that CCI is superior to IPI, ECOG or another scoring system in a single-center patient population, it should be stated that CCI is also an effective scoring system in patients diagnosed with DLBCL. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-022-01567-5.

Eltrombopag in the Treatment of Immune Thrombocytopenia: Two-Center Experience from Istanbul.

Immune Thrombocytopenia (ITP) is a disease caused by autoantibodies forming against platelets and T cell dysregulation and is characterized by platelet count falling below 100 × 109/L. Corticosteroids remain as the first-line of treatment, but in the light of recent developments, thrombopoietin receptor agonists are gradually replacing splenectomy in steroid-dependent or refractory patients. In this study, it was aimed to retrospectively evaluate the efficacy, safety and side-effect profile of eltrombopag treatment for chronic ITP. A total of 23 chronic ITP patients treated with eltrombopag from two health institutions in Istanbul were evaluated retrospectively. Overall response rate (partial or complete) was 87%, complete response rate was 78.3%, and the median time from treatment until reaching platelet counts above 50 × 109/L was 14 days (min-max: 4-126). Treatment was discontinued in four patients due to persistent response, two of these were still fully responsive. During treatment, one patient developed basal cell carcinoma, and another developed chronic myelomonocytic leukemia. Although its long-term side effects are not yet known, eltrombopag is a very effective treatment option in ITP and may provide favorable outcomes in patients.

Comparison of Splenectomy and Eltrombopag Treatment in the Second-Line Treatment of Immune Thrombocytopenic Purpura

Objective: Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by isolated thrombocytopenia. While first-line treatments focus on inhibiting autoantibodies and platelet destruction, second- and third-line treatments include splenectomy and thrombopoietin receptor agonists. In this study, we aimed to compare the efficiency and toxicities of splenectomy and eltrombopag as second-line treatments in ITP. Materials and Methods: We retrospectively analyzed patients who were diagnosed with ITP and followed between 2015 and 2020. Patients who underwent splenectomy or received eltrombopag treatment as second-line or further therapy were included. For subgroup analyses, patients were further stratified according to whether they received eltrombopag in the second or third line of treatment. Results: There were 38 patients in the splenectomy group and 47 patients in the eltrombopag group. The mean age of patients in the splenectomy and eltrombopag groups was 43.2 and 50.5 years, respectively. Time to response was significantly shorter in the splenectomy arm (p=0.001). However, response rates at the 3rd, 6th, 12th, and 24th months did not exhibit a statistically significant difference between groups; nor did total duration of response and adverse events. Response rates at the 1st, 3rd, 6th, 12th, and 24th months and the total duration of response did not exhibit a statistically significant difference between eltrombopag subgroups. Eltrombopag treatment was ceased for 20 patients after a median of 54.1 months (range: 1-151). Among them, 12 patients (60%) did not experience a loss of response. Conclusion: Comparing the splenectomy and eltrombopag arms, even though time to achieve response was in favor of the splenectomy group, this advantage disappeared when overall response rates and response rate at the 2nd year were considered. Using eltrombopag in the second or third line of therapy does not yield any difference in terms of time to achieving response.

A Real-life Turkish Experience of Venetoclax Treatment in High-risk Myelodysplastic Syndrome and Acute Myeloid Leukemia.

INTRODUCTION: Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS) in combination with either low-dose cytarabine (ARA-C) or hypomethylating agents. We aimed to collect and share data among the efficacy and safety of venetoclax both as a monotherapy or in combination with other drugs used to treat high-risk MDS or AML. MATERIALS AND METHODS: A total of 60 patients with a median age of 67 (30-83) years from 14 different centers were included in the final analysis. Thirty (50%) of the patients were women; 6 (10%) of the 60 patients were diagnosed with high-risk MDS and the remaining were diagnosed with AML. RESULTS: The best objective response rate (complete remission [CR], complete remission with incomplete hematological recovery (CRi), morphological leukemia-free state [MLFS], partial response [PR]) was 35% in the entire cohort. Best responses achieved during venetoclax per patient number were as follows: 7 CR, 1 CRi, 8 MLFS, 5 PR, and stable disease. Median overall survival achieved with venetoclax was 5 months in patients who relapsed and not achieved in patients who were initially treated with venetoclax. Nearly all patients (86.7%) had experienced a grade 2 or more hematologic toxicity. Some 36.7% of these patients had received granulocyte colony stimulating factor (GCSF) support. Infection, mainly pneumonia (26.7%), was the leading nonhematologic toxicity, and fatigue, diarrhea, and skin reactions were the others reported. CONCLUSION: Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML.

Beta Thalassemia Minor: Patients Are Not Tired but Depressed and Anxious.

OBJECTIVE: We aimed to investigate whether the severity of fatigue and the incidences of depression and anxiety of patients with beta thalassemia minor (BTm) are different from healthy individuals using the Fatigue Severity Scale (FSS) and Hospital Anxiety and Depression Scale (HADS). SUBJECTS AND METHODS: BTm patients who were followed at the University of Health Sciences Istanbul Training and Research Hospital Hematology Clinic between 2016 and 2017 and who had normal biochemical parameters, thyroid function tests and C-reactive protein levels, and did not use any medications, consume alcohol or tobacco, have any chronic diseases or sleep disturbances were included in the study. Healthy control subjects who were matched with age, sex, marital status, educational status, and body mass index (BMI) were also included for comparison. RESULTS: Thirty-nine BTm patients and 25 healthy controls were included in the study. The BTm and the control groups were comparable in terms of gender, age, BMI, educational status and marital status (p = 0.368, 0.755, 0.851, 0.785, and 0.709, respectively). FSS score was ≥4 in 23 (59.0%) BTm subjects and in 15 (60%) control subjects (p = 1.0). HADS anxiety score was ≥10 in 20 (51.3%) BTm subjects and in 5 (20.0%) control subjects (p = 0.018), and HADS depression score was ≥7 in 20 (51.3%) BTm subjects and in 6 (24.0%) healthy control subjects (p = 0.039).There was no correlation of hemoglobin with FSS score (p = 0.526, r = -0.105), HADS anxiety score (p = 0.703, r = -0.063), or HADS depression score (p = 0.718, r = -0.06) in the BTm group. CONCLUSION: We found that both depression and anxiety were higher in BTm patients than in healthy individuals, but this difference was not feasible for fatigue.

Lenalidomide Plus Decitabine Treatment in a Myelodysplastic Syndrome Patient With Deletion 5q and Excess Blasts.

This is the first case of decitabine plus lenalidomide treatment for a myelodysplastic syndrome (MDS) patient with 5q deletion (del(5q)) and elevated number of blasts. Upon bone marrow aspiration and biopsy with conventional cytogenetical studies she was diagnosed with MDS with del(5q) and refractory anemia with excess blasts (RAEB-1). Decitabine was started at a daily dose of 20 mg/m2 1 - 5 days and lenalidomide was started at daily doses of 10 mg 6 - 20 days a month. After two cycles, her hemoglobin level increased and transfusion dependency ceased. After four cycles, bone marrow aspiration showed blast ratio of < 5%. Decitabine and lenalidomide were applied for three more cycles. Decitabine was terminated after seven cycles and lenalidomide has been continued for 12 months. Latest blood values (February 2020) were as follows: white blood cells (WBCs) of 8,670/mm3, neutrophil count of 3,470/mm3, hemoglobin (Hb) level of 11.7 g/dL and platelet count of 203,000/mm3, and the patient continues to follow-up without treatment. In conclusion, combination of lenalidomide and decitabine seems to be an effective treatment modality without notable side effects in MDS patients with del(5q) and excess blasts. The efficacy of this combination should be validated with studies including large patient groups and with longer follow-up periods.

Evaluation of the Reliability of Interim PET/CT in the Hodgkin Lymphoma.

INTRODUCTION: Positron-emission tomography (PET)/computerized tomography (CT) with 18F-fludeoxyglucose (FDG) has been come into use for risk assessment of Hodgkin lymphoma (HL) patients in recent years. The aim of our study is to evaluate the reliability of interim PET results according to Deauville score (DS), and also to compared PET findings with tumor reduction on CT. METHODS: Forty-two HL patients (median 39, range 19-75 y, 27 M, 15 F) were retrospectively evaluated with pre, interim and post-treatment PET/CT imaging. PET/CT imaging was obtained 60 min after the intravenous administration of 3.7-5.2 MBq/kg 18F-FDG. RESULTS: The negative predictive value of the interim PET was 89%. Four (10.5%) of the 38 interim PET-negative patients became post-treatment PET-positive. According to CT, 15 patients were in complete remission (CR), 27 (64.6%) patients were in partial remission (PR) or stable disease (SD). CONCLUSION: The negative predictive value of interim PET was not satisfactory considering the treatment rate of over 80% of HL. Additionally, high rate of interim PET-negative patients' conversion to PET-positive post-treatment state was considered as unexpected.

How Does 2016 WHO Criteria for Polycythemia Vera Contribute to Our Daily Practice? A Single-Center Study from Turkey.

Background: We evaluated the frequency of subnormal erythropoietin levels, Janus kinase 2 (JAK2) V617F positivity and polycythemia vera (PV) in patients who did not meet World Health Organization (WHO) 2008 criterion for hemoglobin levels but were suggested to be investigated for PV in 2016 revision. Materials and Methods: We assessed the data of 92 patients who were further evaluated with JAK2V617F mutation and serum erythropoietin (EPO) levels and bone marrow biopsy, if necessary. We also compared this patient group with 20 patients whose Hgb>18.5 g/dL for men and >16.5 g/dL for women. Results: Nine patients (45%) in the higher hemoglobin group were JAK2V617F positive, while 4 patients (4.3%) in the lower hemoglobin group were JAK2V617F positive (p<0.001). The number of patients with serum EPO levels <4.3 mIU/mL was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=7, 7.6%) (p<0.001). Finally, the number of patients who received a diagnosis of PV was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=9, 9.8%) (p<0.001). Conclusion: We found a substantial increase in patients who were candidates for testing for PV with the introduction of WHO 2016 criteria; these patients were diagnosed with PV with a rate (9.8%) that cannot be underestimated.

Comparison of clinical features and survival between young and old chronic lymphocytic leukemia patients: a single center study from Turkey.

We aimed to investigate whether the clinical characteristics, the rate of treatment demand and survival differ between chronic lymphocytic leukemia (CLL) patients <65 years (y) and ≥65 y. Sixty three (46%) patients were <65 y and 74 (54%) were ≥65 y. 28.6% (18/63) of the patients <65 required treatment, while this rate was 44.6% (33/74) for patients ≥65 y (p > .05). The probability of overall survival (OS) was 90 vs. 51% in patients <65 y and ≥65 y (p = .016). In univariate analysis, the age affected OS (p = .04, HR: 0.212, CI: 0.047-0.946). In subgroup analysis, in treated patients, the probability of OS was 73 vs. 49% in patients <65 y and ≥65 y (p = .389). The survival difference between the young and old patients is too high to be ignored and age seems to affect the outcome of CLL patients.

Prevalence of inflammatory back pain and radiologic sacroiliitis is increased in patients with primary Sjögren's syndrome.

INTRODUCTION: The prevalence of Sjögren's syndrome (SS) in patients with the diagnosis of SpA has been reported to be higher than normal population. Yet, the vice-versa is unclear. In this study, we aimed to investigate the prevalence of IBP, radiologic sacroiliitis and SpA in patients with primary SS. METHODS: 85 patients followed at the rheumatology clinics of the Marmara and Kocaeli Universities with the diagnosis of primary SS between November 2011 and August 2012 were included in this study. The control group consisted of 100 age-and gender-matched patients. Inflammatory back pain and axial SpA were diagnosed according to the assessment of spondylo arthritis International Society (ASAS) criteria. RESULTS: 83 patients were (97%) female and 2 (3%) were male. Mean age of the patients was 49.1 (±11) years. Mean disease duration was 7.3 (±4) years. The patient and control groups were comparable in terms of age and gender (p > 0.05). Inflammatory back pain was observed in 21 (24.7%) of 85 primary SS patients and in 4 (4%) of 100 control subjects (p < 0.001), radiographic sacroiliitis was demonstrated in 9 (10.5%) of primary SS patients and 2 (2%) of the control subjects (p = 0.025). Remaining SpA findings were not encountered in either group. CONCLUSION: inflammatory back pain and radiologic sacroiliitis is increased in patients with primary SS. Whether IBP, SI joint inflammation and radiologic sacroiliitis is due to the co-existence of SpA and primary SS or IBP is an underdiagnosed clinical feature of SS deserves further studies of large patient numbers.

Stem cell mobilization kinetics in elderly patients with multiple myeloma.

In this study, we aimed to investigate whether the procedure and product kinetics differ according to age groups in advanced-age MM patients who underwent autologous HSCT. 59 patients who underwent autologous HSCT were retrospectively analyzed. Then, the patients were divided into two groups as 60-65 years and ≥65 years. It was significantly lower in ≥65 years group (p = 0.008) and proportionally, the procedure duration was also significantly shortened in this group (p = 0.013). Total number of collected CD34 positive stem cells was 6.20 × 106 (±3.83) in 60-65 years group while it was 5.51 × 106 (±2.48) in ≥65 years group with no statistically significant difference (p = 0.825). In conclusion, there was no significant difference in terms of the number of collected CD34-positive stem cells in this study that investigates the mobilization data, procedure and product kinetics, we think that successful stem cell mobilization can be performed in appropriately selected patients regardless of age.

Autologous stem cell transplantation and stem cell mobilization kinetics in elderly patients with B cell non-Hodgkin lymphoma.

As known, the world population is aging and as the life span increases the number of advanced-age lymphomas also shows an upward trend. Autologous hematopoietic stem cell transplantation (HSCT) is the standard treatment modality in chemotherapy-sensitive relapsed or refractory aggressive lymphomas. Increased morbidity and mortality related to both the transplant itself and comorbid diseases can be observed in elderly lymphoma patients. Patients who are 65 years or older and underwent autologous HSCT with B-cell non-Hodgkin lymphoma were retrospectively included in our study. In terms of survival analysis, median follow-up was 34.5 months (8-159) while the overall survival (OS) was 58%. In the univariate analysis of prognostic data in OS, patients who were referred to transplantation with complete response had a statistically significant survival advantage (p=0.043). In terms of the effect of pre-transplant conditioning regimens on survival, BEAM regimen yielded better results, though not statistically significant. Age, number of chemotherapy cycles received before mobilization and radiation therapy had no significant effect on the CD34 (+) cell count in the final product (p=0.492, 0.746 and 0.078 respectively). In conclusion, autologous HSCT is a practicable treatment modality that provides survival advantage in suitable advanced-age patients with a diagnosis of B-cell non-Hodgkin lymphoma.

Rethinking the usefulness of bone marrow biopsy on treatment decision in CLL patients at diagnosis.

We aimed to investigate the role of bone marrow infiltration pattern (BMIP) and bone marrow reticulin fibrosis (BMRF) in determining treatment demand in patients with diagnosis of chronic lymphocytic leukemia (CLL). We retrospectively evaluated the data of 65 patients, who were followed with the diagnosis of CLL at Istanbul Training and Research Hospital, Department of Hematology, between July 2007 and June 2016. The median age of the patients was 64 years (range, 32-83). Twenty-three (35.4%) patients were female, and 42 (64.6%) were male. Early/mild grade BMRF was observed in 46 (70.8%) patients and advanced grade BMRF in 19 (29.2%) patients. Eleven (23.9%) of 46 patients with early/mild grade BMRF and 10 (52.9%) of 19 patients with advanced grade BMRF required treatment during follow-up (p = 0.04). According to the BMIP, 14 (21.5%) patients had diffuse and 51 (78.5%) patients had non-diffuse BMIP. Eleven (78.6%) of 14 patients with diffuse BMIP and 10 (19.6%) of 51 patients with non-diffuse BMIP required treatment during follow-up (p < 0.001). In univariate analysis, both advanced grade BMRF and diffuse BMIP had an impact on occurrence of treatment demand (p = 0.028, HR = 3.535 vs. p < 0.01 HR = 15.033). Multivariate analysis also revealed diffuse BMIP to be effective (p < 0.001, HR 13.089), while advanced grade BMRF failed to significantly influence treatment demand (p = 0.140, HR 2.664). In conclusion, in the light of our findings, it is reasonable to consider that bone marrow biopsy at the time of diagnosis might provide a preliminary information about treatment demand in patients with CLL.

Evaluation of neutrophil-lymphocyte ratio in patients with early-stage mycosis fungoides.

Neutrophil-lymphocyte ratio (NLR), an indicator of inflammation, has been lately demonstrated as a prognostic factor and an indicator of disease activity in various diseases. However, the effects of NLR have not been investigated in mycosis fungoides (MF) patients yet. The aim of this study is to investigate the relationship between the NLR and treatment demand (systemic PUVA and/or chemotherapy), time to treatment, progression in stage, and time to progression in stage in MF patients. The data of 117 patients, who were followed with the diagnosis of MF at the Department of Dermatology in Istanbul Training and Research Hospital between April 2006 and January 2016, were analyzed retrospectively. The cutoff score for NLR was determined as 2 according to the median NLR level which was 1.96. At the time of diagnosis, the median age of patients was 54 years (range, 21-90) with 62 (53 %) female and 55 (47 %) male. Seventy-seven (65.8 %) patients required treatment during follow-up. Sixty-three (53.8 %) patients showed progression in disease stage. There was no significant difference in treatment demand, time to treatment, progression in stage, and time to progression in stage in patients with a NLR ≥ 2 and NLR < 2 (p = 0.331, 0.987, 0.065, and 0.119, respectively). It seems that there is no association between the NLR and treatment demand, time to treatment, progression in stage, and time to progression in stage in MF patients.

T-cell acute lymphoblastic leukemia with co-expression of CD56, CD34, CD117 and CD33: A case with poor prognosis.

T-cell acute lymphoblastic leukemia (ALL) is an aggressive hematological malignancy, accounting for ~25% of all adult cases of ALL. We herein report a case of T-cell ALL exhibiting aberrant CD34, CD56, CD33 and CD117 expression in addition to T-cell markers, which did not respond to induction treatment. A 55-year-old woman was admitted to our hospital with a sore throat unresponsive to medication for 1 month. The laboratory examination revealed pancytopenia and the peripheral blood smear examination revealed blast cells. On flow cytometric analysis, the blast cells were found to be positive for cytoplasmic CD3, CD2, CD5, CD7, CD34, CD56, CD33 and CD117, and negative for myeloperoxidase, CD13, CD11b, CD15, CD19, CD79a, CD22 and CD10. The patient was diagnosed with T-cell ALL according to the 2008 World Health Organisation classification. The patient did not respond to Hyper-cyclophosphamide, vincristine, adriamycin and dexamethasone (CVAD) course A treatment and succumbed to the disease during Hyper-CVAD course B treatment. To the best of our knowledge, this is the first report of aberrant co-expression of the natural killer cell marker CD56, myeloid cell markers CD117 and CD33 and stem cell marker CD34 in a patient with T-cell ALL. This appears to be associated with an unfavorable outcome, despite the use of intensive chemotherapy.