Objective: Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative diseases that can transform to secondary myelofibrosis (SMF). In this study, we evaluated spleen stiffness using shear-wave elastography (SWE) as a predictor of progression to SMF. Methods: Participants were grouped as healthy volunteers (HVs), PV/ET patients, and SMF patients. Participants' spleen sizes, spleen stiffness values, bone marrow fibrosis degrees, and the other parameters were evaluated. Spleen stiffness values and spleen sizes were compared between groups. Results: Of the 121 participants included in this study, 52 patients were HVs, 52 patients were PV and/or ET patients, and 17 patients were SMF patients. In terms of age and sex, there was no difference between groups. Splenic parenchymal stiffness median values by using SWE were found to be 0.82 m/s in HVs, 1.41 m/s in PV/ET patients, and 2.32 m/s in SMF patients (P < 0.001). In terms of median length of the spleen, the difference between groups was significant (P < 0.001). In addition, we found a significant positive correlation between spleen stiffness and bone marrow fibrosis degree (P < 0.001, r = 0.757). However, in multivariate analysis, there was no strong independent risk factor for spleen stiffness. Conclusion: In this study, we showed that measurement of spleen stiffness using SWE can distinguish SMF from PV/ET patients and HVs. Therefore, we believe that SWE may be used as a noninvasive and easily accessible method to check the fibrotic progression of bone marrow in PV and ET patients to monitor the transformation to SMF, and enables to detect fibrosis in early phase.
1 November 2022 in Mediterranean Journal of Hematology and Infectious Diseases
Objectives: Patients with hematologic malignancies have a high risk of coronavirus disease 2019 (COVID-19) mortality. This study aimed to investigate the impact of COVID-19 on mortality rates in patients with various hematologic malignancies and to determine risk factors associated with all-cause mortality. Methods: A multi-center, observational retrospective analysis of patients with hematologic malignancies infected with COVID-19 between July 2020 and December 2021 was performed. Demographic data, clinical characteristics, and laboratory parameters were recorded. Patients were grouped as non-survivors and survivors. All-cause mortality was the primary outcome of the study. Results: There were 569 patients with a median age of 59 years. Non-Hodgkin lymphoma (22.0%) and multiple myelomas (18.1%) were the two most frequent hematologic malignancies. The all-cause mortality rate was 29.3%. The highest mortality rates were seen in patients with acute myeloid leukemia (44.3%), acute lymphoid leukemia (40.5%), and non-Hodgkin lymphoma (36.8%). The non-survivors were significantly older (p<0.001) and had more comorbidities (p<0.05). There were significantly more patients with low lymphocyte percentage (p<0.001), thrombocytopenia (p<0.001), and high CRP (p<0.001) in the non-survived patients. Cardiac comorbidities, (p=0.016), cytotoxic chemotherapy (p=0.024), low lymphocyte percentage (p=0.025), thrombocytopenia (p<0.0001), and high CRP values (p=0.017) were the independent risk factors for the prediction of mortality. Conclusions: In patients with hematologic malignancies, coexistent COVID-19 leads to a higher mortality rate in elderly patients with more comorbidities. Acute myeloid and lymphoid leukemia and non-Hodgkin lymphoma have the highest mortality rates. Cardiac diseases, cytotoxic chemotherapy, lymphopenia, thrombocytopenia, and high CRP are the independent risk factors for mortality in hematologic malignancy patients with COVID-19. Keywords: Covid; Hematologic Malignancy; Cytotoxic chemotherapy.
Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare clonal hematopoietic stem cell disease characterized by chronic hemolytic anemia and thrombosis. We report data from a study of the occurrence of PNH among patients with idiopathic portal vein thrombosis (PVT). Patients who were followed up with the diagnosis of idiopathic PVT were enrolled into this study. Those with laboratory and/or imaging evidence of any local or systemic factor that could lead to PVT were excluded. PNH clone was examined in all patients using established FLAER methodology. A total of 112 patients (42 males and 70 females), none of them had a markedly PNH clone, but 4 patients (3.6%) with confirmed tests two times had small PNH clones (size between 3.02% and 4.62%). The median ages of PNH clone (-) and PNH clone (+) patients were 42 (range; 24-59) vs 39 (range; 36-42) years, respectively. The median hemoglobin concentration, platelet count and leukocyte count were lower in the PNH clone (+) group than the PNH clone (-) group. Anemia, thrombocytopenia, and leukopenia were detected in all PNH clone (+) patients. In addition, the PNH clone positivity size in monocytes was higher than erythrocytes in all of 4 patients. PNH should be considered during differential diagnosis among patients with idiopathic PVT. Small PNH clones can be detected in PVT patients that we cannot clearly determine its relationship with PVT. We need furthermore studies to explore the potential role of this finding.
Purpose: This study aims to evaluate the epidemiological, demographic and clinical characteristics, and prognostic factors of the patients with non-Hodgkin lymphomas (NHL). Materials and Methods: All patients diagnosed with NHL at the Department of Hematology of Inonu University Faculty of Medicine (Turkey) between 2000 and 2016 were evaluated for this study. Only patients older than 18 years were included in the study. Characteristics were evaluated by reviewing patients' records retrospectively. Results: Of the 386 patients studied, 242 (62.7%) were male and 144 (37.3%) were female and the overall median age was 53 years (range: 18-92). The most common histological subtype of NHL was diffuse large B-cell lymphoma (DLBCL) (46.9%). In univariate analyses, advanced stage (III-IV), high-intermediate to high risk category disease based on IPI score, bone marrow involvement at diagnosis, haemoglobin levels below 10 g/dL, increased LDH levels, primary nodal involvement, the presence of B symptoms, the exigency of autologous bone marrow transplant, and not receiving rituximab-based chemotherapy regimens as the primary treatment were all associated with shorter overall survival. Conclusion: The prevalence, clinical characteristics, histopathological subtypes, treatment responses, and overall survival rates may differ because NHL is a heterogeneous disease group and may vary according to a geographical area. Therefore, treatment should be individualized according to disease subtype.