Background: We evaluated the frequency of subnormal erythropoietin levels, JAK2V617F positivity and polycythemia vera (PV) in patients who did not meet 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.
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 3 rd , 6 th , 12 th , and 24 th months did not exhibit a statistically significant difference between groups; nor did total duration of response and adverse events. Response rates at the 1 st , 3 rd , 6 th , 12 th , and 24 th 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 2 nd 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.
Background Multiple myeloma (MM) is characterized by infiltration of neoplastic plasma cells in the bone marrow. Although many novel agents have been developed in the last decade, MM remains a non-curable disease. The association between bone marrow fibrosis (BMF) and MM survival is unknown, and the considerable changes in patient survival during the last few decades necessitates new studies to examine survival and associated factors in patients with MM. Results A total of 72 patients with MM, 39 (54.17%) males and 33 (45.83%) females, were included in this retrospective study. Fifteen patients did not have BMF, 55 had BMF (grades 1–4); there were no significant differences between these groups in terms of any of the parameters examined. The 5-year overall survival (OS) rate was 56.5 ± 7.4%. Mean OS was 81.54 ± 7.01 months, mean progression-free survival (PFS) after first-line treatment was 14.07 ± 2.54 months, and mean PFS after autologous stem cell transplantation (ASCT) was 25.92 ± 3.66 months. Survival times or mortality risk were not found to be associated with BMF in any of the analyses (HR 1.208, [95% CI 0.408–3.578], p = 0.733). Mortality risk was increased by 8.163-fold in patients with hypercalcemia (HR 8.163, 95% CI 2.413–27.617, p = 0.001), while it was decreased by 0.243-fold in patients with favourable response to first-line treatment (HR 0.243, 95% CI 0.078–0.756, p = 0.015). Younger patients (< 60 years) had a 1.981-fold greater risk of progression after first-line treatment (HR 1.981, 95% CI 1.111–3.532, p = 0.021), while those with hypercalcemia had a 3.160-fold greater risk of progression after ASCT (HR 3.160, 95% CI 1.103–9.052, p = 0.032). Low haemoglobin levels were also associated with increased mortality risk (p = 0.024). Conclusion Although hypercalcemia, unfavourable treatment response, young age and a low haemoglobin level were found to be indicators of poor prognosis in patients with MM, no relationship was found between BMF and survival.
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