Effects of Splenectomy on Natural Killer Cell Levels in ß-Thalassemia Major Patients.

AIM: In this study, we investigated how splenectomy affects natural killer (NK) cell levels in patients with ß-thalassemia major (ß-TM). MATERIALS AND METHODS: Seventy patients with ß-TM (38 splenectomized and 32 nonsplenectomized) and 25 healthy controls were included in this study. The hemogram parameters, ferritin, T lymphocyte, T-helper cell, T-suppressor cell, and NK cell numbers, were measured. RESULTS: The T lymphocyte (CD3+) level was found to be significantly higher in the patient group (p < 0.05). CD3+/CD4+ T lymphocytes were detected to be significantly higher in the patient group (p < 0.05). Although the CD3+/CD4+ T lymphocyte level was significantly higher in the nonsplenectomy group (p < 0.05), this was not the case in the splenectomy group. When the patient and control groups were compared, no significant difference was detected regarding CD3+/CD8+ T lymphocyte levels. CD3-/CD16+CD56+ NK cell level was found to be significantly lower only in the splenectomy group than in the control group (p < 0.05). We found that there was a significant negative correlation between serum ferritin levels and both total lymphocyte (r = -0.617) and CD3+ lymphocyte (r = -0.718) levels in the control group (p < 0.05). A significant negative correlation was detected between serum ferritin levels and CD3-/CD16+CD56+ NK cell levels in the patient group (r = -0.410) (p < 0.05). CONCLUSION: Splenectomy reduces NK cell levels in patients with ß-TM. The negative relationship between ferritin levels and NK cells indicates that ferritin levels should be kept under control in patients with ß-TM.

Is absence of CD56 a predictive factor for peripheral blood stem cell mobilization failure in patients with multiple myeloma?

BACKGROUND: CD56 is believed to play a major role in MM pathogenesis with a 70% to 80% expression rate in malignant plasma cells at the time of diagnosis. Our objective in this study was to investigate the relationship between the characteristics of CD56 expression in bone marrow aspiration material at the time of diagnosis and the success of stem cell mobilization in patients diagnosed with MM. METHODS: This monocenter study included 94 patients who were diagnosed with MM and had a stem cell mobilization procedure for autologous hematopoietic stem cell transplantation. The primary endpoint of the study was to compare the mobilization success between the groups with and without CD56 expression. The secondary endpoint was to identify other factors affecting mobilization failure outside CD56. RESULTS: At the time of diagnosis, 49 (52.1%) patients had CD56 expression and 45 (47.9%) did not. Mobilization failed in 11 (11.7%) patients. Age, gender, ISS stage and the number of premobilization treatment regimens were not found predictive of mobilization failure. CD56 negativity was 42.2% in the group that had mobilization success and 90.9% in the group that had mobilization failure (P = .001). CONCLUSIONS: The fact that CD56 residing on the membrane enables interaction between bone marrow cells and ECM and functions as a signal molecule increases sensitivity to the chemotherapy and G-CSF that are used for mobilization. We found that absence of CD56 can be used as a predictive factor for mobilization failure at the time of diagnosis.

Thrombotic Thrombocytopenic Purpura Cases and Consumption of Large Quantities of Energy Drink: Related or Coincidence?

The vast majority of cases of thrombotic thrombocytopenic purpura (TTP) are the result of acquired antibodies which inhibit the activity of the ADAMTS13 enzyme. Acquired TTP is more frequently seen in young females or in individuals with autoimmune disease. The development of antibodies against ADAMTS13 may also result from the administration or consumption of drugs and other substances. However, specific laboratory tests to identify the pathogenic mechanism of a particular drug may not be available, and the role of a potentially implicated drug or other ingested substance may not be clear. In this report we present 2 acquired TTP cases involving the consumption of a large amount of energy drink.

Impact of COVID-19 on Outcomes of Patients with Hematologic Malignancies: A Multicenter, Retrospective Study.

Objectives: Patients with hematological malignancies have a high risk of mortality from coronavirus disease 2019 (COVID-19). This study aimed to investigate the impact of COVID-19 on mortality rates in patients with various hematological malignancies and to determine risk factors associated with all-cause mortality. Methods: A multicenter, observational retrospective analysis of patients with hematological 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 hematological 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). In addition, 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. Age ≥ 65years (p=0.017), cardiac comorbidities (p=0.041), and continuation of ongoing active therapy for hematological cancer (p<0.001) were the independent risk factors for the prediction of mortality. Conclusions: In patients with hematological 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. Older age, cardiac diseases, and continuation of ongoing active therapy for hematological cancer are the independent risk factors for mortality in hematological malignancy patients with COVID-19.

Turkey real-life data: demographic features, treatment results and effects of comorbidities in chronic myeloid leukemia.

Aim: This study aimed to identify patient characteristics, treatment patterns and outcomes and to evaluate the effects of presence of comorbidities at diagnosis in chronic phase (CP)-chronic myeloid leukemia (CML) patients in Turkey. Materials & methods: Hospital records between 2005 and 2018 were retrospectively reviewed. Results: Of 861 CP-CML patients included, 31% had at least one comorbidity at diagnosis. Sex, cardiovascular disease status at diagnosis and molecular (at least major) and cytogenetic (partial and complete) responses were the independent predictors of survival. Conclusion: The response rates of CP-CML patients to the tyrosine kinase inhibitors were satisfactory. In addition to tolerability and side effect profiles of drugs, comorbidity status of patients should also be considered in treatment choice in CML patients.

This study aimed to identify patient characteristics, treatment patterns and outcomes and to evaluate the effects of presence of comorbidities at diagnosis in chronic phase (CP)-chronic myeloid leukemia (CML) patients in Turkey. Hospital records of patients between 2005 and 2018 were retrospectively reviewed. Of the included 861 CP-CML patients, 31% had at least one comorbidity at diagnosis. The survival of the patients was affected by sex, cardiovascular disease status at diagnosis, and molecular (at least major) and cytogenetic (partial and complete) responses. The response rates of CP-CML patients to the tyrosine kinase inhibitors were satisfactory. In addition to tolerability and side effect profiles of drugs, comorbidity status of patients should also be considered in treatment choice in CML patients.

Effect of the ABO blood groups on the development, clinical features and survival of multiple myeloma.

Background: Multiple myeloma (MM) is a cytogenetically heterogeneous and incurable plasma cell disease with unknown etiology. It is thought that the ABO blood groups may play a role in the etiology of many diseases. The purpose of this study is to determine whether there is a relationship between the ABO blood groups and the development of MM, clinical findings and overall survival. Methods: In this single-center, retrospective and observational study, 198 patients with known blood types who diagnosed with MM between January 2012 and June 2020 were included. Results: It was shown that individuals with blood group 0 had a significantly lower risk of MM (OR = 0.575, 95% confidence interval 0.416-0.794, P = 0.001). The incidence of extramedullary lesion was significantly higher in those with 0 blood group compared to other blood groups (P = 0.000). Overall survival was significantly shorter in patients with 0 blood group than those without 0 blood group (P = 0.007). Conclusion: Individuals with 0 blood group had a lower risk of developing MM. It was determined that having 0 blood group is a predisposing factor for the development of extramedullary lesion in MM patients. However, it was shown that having a blood group of 0 was a very significant prognostic factor for MM patients and was associated with short OS.

Comparison of Bone Marrow Involvement with Bone Marrow Biopsy and PET-CT and Evaluation of Any Effects on Survival in Patients Diagnosed with Hodgkin and Non-Hodgkin Lymphoma.

We aimed to demonstrate whether PET-CT can replace bone marrow biopsy in detecting bone marrow involvement in subtypes of lymphoma. In addition, we aimed to also reveal whether there is a difference between the mean survival of patients with bone marrow involvement via PET-CT or biopsy. A total of 276 newly diagnosed lymphoma patients who underwent bone marrow biopsy and PET-CT prior to the treatment were scanned retrospectively. Bone marrow biopsy was used as the standard method to investigate the presence of bone marrow involvement in PET-CT. The relationship between bone marrow involvement and mean survival was compared using both methods. Out of the 276 patients, bone marrow involvement was detected with PET-CT and with biopsy, respectively in 56 patients (20.2%) and in 78 patients (28.2%). In terms of PET-CT's accuracy with respect to revealing bone marrow involvement, the highest rates were achieved respectively in diffuse large B cell lymphoma (DLBCL) (87.4%) and Hodgkin lymphoma (HL) (77.7%). In both the PET-CT and bone marrow biopsy methods, Overall Survival (OS) was found to be significantly shorter in patients with involvement than in patients without involvement (P: 0.001). PET-CT may replace bone marrow (BM) biopsy in detecting the bone marrow involvement in aggressive lymphoma subtypes such as DLBCL and HL. The presence of BM involvement at the time of diagnosis in both PET-CT and BM biopsy is associated with poor prognosis, and OS is short in this group.

Prognostic significance of hematogone presence after autologous stem cell transplantation in patients with multiple myeloma.

BACKGROUND: Morphologically and immunophenotypically, the number of B-lymphocyte progenitor cells, so-called hematogones increases after chemotherapy and allogeneic stem cell transplantions. It is thought that hematogones can be used as a prognostic marker in these patients. It is aimed to determine the prognostic significance and factors affecting the development of hematogones, which can be seen after autologous stem cell transplantation in multiple myeloma (MM) patients. METHODS: This retrospective and single center study includes 80 patients who underwent autologous stem cell transplantation with the diagnosis of MM in our clinic between January 2013 and December 2019. The primary endpoint of the study was the relationship between the presence and rate of hematogone (HG) and progression free survival (PFS) and overall survival (OS). The secondary endpoint was to identify the factors affecting the development of HG. RESULTS: HG was detected in 61.2% of the patients. There was a moderate and positive linear correlation between the amount of stem cells given and HG ratio (r = 0.387, p = 0.000). PFS and OS were significantly shorter in the group with HG (p = 0.000 and p = 0.012). CONCLUSIONS: HG positivity after autologous stem cell transplantation was found to be an independent prognostic marker for PFS and OS in patients with MM. There is a positive relationship between the amount of stem cells used during transplantation and the ratio of HG. As the amount of stem cells increases, the ratio of HG increases and when the ratio of HG increases, PFS and OS become shorter.

Effect of prophylactic leukapheresis on early mortality and overall survival in acute leukemia patients with hyperleukocytosis.

Hyperleukocytosis has been associated with early mortality owing to the presence of complications including leukostasis, tumor lysis syndrome (TLS), and disseminated intravascular coagulation (DIC). Leukapheresis is a fast and effective cytoreductive procedure that removes leukocytes from the peripheral circulation. This single-center, retrospective, and observational study included 32 patients diagnosed with acute leukemia who underwent leukapheresis due to hyperleukocytosis between 2014 and 2020. This study primarily aimed to investigate the effect of prophylactic leukapheresis on early mortality and overall survival (OS). In the symptomatic group, seven and two patients died in the first and second weeks, respectively. In the prophylactic leukapheresis group, two and one patients died in the first and second weeks (p = 0.792), respectively. OS was significantly longer in the prophylactic leukapheresis group (p = 0.004). The leukapheresis procedure appears to be effective on early mortality and OS. Initiation of prophylactic leukapheresis before the appearance of leukostasis symptoms is effective on OS and possibly early mortality.

Clinical Significance of Increased FDG Uptake in the Waldeyer Ring and the Nasopharynx Region Identified by PET-CT in Postchemotherapy Follow-up in Patients With Lymphoma: When Should We Perform Biopsy?

PURPOSE: To identify whether fluorodeoxyglucose (FDG) uptake in the Waldeyer ring (WR)/nasopharyngeal (NP) region by positron emission tomography-computed tomography (PET-CT) was physiologic or pathologic in the follow-up of lymphoma patients receiving postchemotherapy treatment. PATIENTS AND METHODS: We retrospectively examined FDG uptake in the WR/NP region in 534 patients with lymphoma as assessed by PET-CT used for both diagnosis and follow-up. RESULTS: Forty-nine patients had FDG uptake in the WR/NP region by PET-CT performed after completion of a chemotherapy regimen. Biopsy was performed for 11 of these patients in whom the uptake was considered to be pathologic, and results indicated the presence of reactive follicular hyperplasia. It was considered to be physiologic in 38 patients. PET-CT was repeated after 1 year, and no significant difference was identified between the standardized maximum uptake values (SUVmax; P = .107). The initial diagnosis of 20 patients was made via biopsy performed in the WR/NP region. The SUVmax for the FDG uptake in these patients, asymmetry, SUVmax of the coexisting lymphadenopathies in the neck, and FDG uptake with a counterpart finding by CT were significantly higher than other groups (P = .047, .001, and .005). CONCLUSION: When deciding whether to resample after treatment completion, it should be taken into account that, in addition to the SUVmax of the lesion, asymmetry, and the SUVmax of the coexisting lymphadenopathy in the neck, a crucial criterion is whether the FDG uptake has a counterpart finding by CT.

Safety and efficacy analysis of ibrutinib in 32 patients with CLL and various B-cell lymphomas: real-world data from a single-center study in Turkey.

BACKGROUND: Ibrutinib is an oral irreversible Bruton's tyrosine kinase inhibitor. Here, we demonstrate the efficacy and safety of ibrutinib using real-life data from patients with marginal zone lymphoma (MZL), diffuse large B-cell lymphoma (DLBCL), Waldenstr?m macroglobulinemia (WM), and follicular lymphoma (FL), especially in chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL). METHODS: This is a retrospective, observational, non-interventional, and single-center study on 32 patients who received ibrutinib treatment between January 2017 and March 2020 regardless of their diagnosis. RESULTS: Of the 32 patients, 11 had CLL and 21 had other B-cell lymphomas. Patients with CLL were prescribed ibrutinib for a median of 4 months (range, 2?18). In this group, diarrhea was observed in 3 (27.3%), pneumonia in 3 (27.3%), and thrombocytopenia and/or neutropenia in 2 (18.2%) patients. The overall response rate (ORR) was 85.6 % [28.5 % complete response (CR) and 57.1 % partial response (PR)] in the final response assessment during treatment with ibrutinib. Among other types of B-cell lymphoma, seven (33.4%) of the 21 patients were diagnosed with MCL, 5 (23.8%) with DLBCL, 4 (19.0%) with MZL, 3 (14.3%) with WM, and 2 (9.5%) with FL, upon follow-up. The median treatment duration was 4 months (range, 1?28) in this group. The most common adverse event was diarrhea: 8 (38.1%) patients. The ORR was 66.6% (20.0% CR and 46.6% PR) in the final response assessment during the treatment. CONCLUSION: Ibrutinib is a good treatment option for CLL and other B-cell lymphomas, with an acceptable side effect profile, and high and promising CR/PR response rates. Ibrutinib treatment at an early stage decreases the burden of cytotoxic therapy in fragile patients, thereby, increasing their quality of life.

Survival analysis of patients with Sjögren's syndrome in Turkey: a tertiary hospital-based study.

OBJECTIVES: This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients. METHODS: All patients diagnosed with pSS between 2004 and 2014 were included in this study. By January 2019, all subjects still living by the end of the study, as well as any death, were identified. Survival rates and standard mortality rates (SMRs) using general population mortality data were calculated. Mortality-related factors were determined by univariate and multivariate analysis. RESULTS: During follow-up, 33 cases of 372 pSS patients resulted in death (8.9%). Of those patients, they were typically older at disease onset, at recruitment, and had shorter follow-up times (p < 0.001 for all). The overall SMR of all pSS patients compared with the general population was 2.11 (95% confidence interval (CI) 1.39-2.83). Male pSS patients had a higher SMR than that of general male patients. Overall survival rates were 97.8% at five years, 90.2% at 10 years, and 87.1% at 15 years in patients with pSS. The survival rate of pSS patients was significantly lower than the general Turkish population (p = 0.011). Multivariate Cox regression analysis showed that older age at disease onset and the presence of interstitial lung disease (ILD) were independent risk factors for mortality. CONCLUSIONS: Based on these data, mortality rates of Turkish pSS patients are higher compared with the general population. Survival significantly decreased in the pSS patients with ILD, especially in older male patients at disease onset. Male gender and malignancy may also be associated with a worse prognosis in pSS patients.Key Point• Mortality in Sjögren's syndrome.