In this study, we aimed to compare the engraftment days, graft-versus-host disease (GVHD) development, relapse and overall survival rates in patients using myeloablative/reduced intensity conditioning regimens with posttransplant cyclophosphamide (PTCy) 25 mg/kg x2 with Anti-T lymphocyte Globulin (ATLG) (n = 29) and PTCy 50 mg/kg x2 doses (n = 41) in patients with acute leukemias. Matched related, matched unrelated, 1 mismatched unrelated, and haploidentical donors were selected for the patients. Platelet (median 11 vs 17 days) and neutrophil (median 14 vs 15 days) engraftment times were shorter in ATLG+ PTCy25 treated patients (both p < 0.05); veno-occlusive disease rates, graft failure and poor graft functions were similar between the two approaches (all p > 0.05); cumulative incidences of grade II-IV aGVHD at +100 days, grade III-IV aGVHD at +100 days, and grade II-IV cGVHD at 1-year were comparable between ATLG+PTCy25 and PTCy50 groups (all p > 0.05). Cumulative incidences of relapse and non-relapse mortality at 1-year were similar in two cohorts (both p > 0.05). PTCy50 was associated with a statistically significant benefit in terms of GVHD-free/relapse-free survival (GRFS) at 1-year (p = 0.03). Median GRFS was 115 (95% CI: 42–214) days and 248 (95% CI: 151-not reached) days, respectively [HR was 0.51 (0.28–0.95), p = 0.03; GRFS at 1-year was 20.7% vs 44.3%, respectively]. However, the groups were comparable in terms of PFS and OS. Median PFS was 332 days (95% CI: 182 days-not reached) for ATLG+PTCy25 group. It was not reached (95% CI: 210 days-not reached) for the patients who received PTCy50. Median OS was not reached in either ATLG+PTCy25 (95% CI: 191 days-not reached) or PTCy50 groups (Log rank = 0.42). Our study showed that lowering PTCy dose with ATLG seems to accelerate platelet and neutrophil engraftment rates; confers similar survival and relapse rates, similar acute and chronic GVHD frequency despite increased GRFS at 1-year.
Introduction: Central nervous system (CNS) involvement is reported to represent 5% of extranodal diffuse large B cell lymphoma (DLBCL) at diagnosis. To stratify patients’ risk of CNS involvement, the CNS-International Prognostic Index (CNS-IPI) score was developed. Serum uric acid level has not been incorporated into IPI or CNS-IPI score and its correlation with CNS-IPI risk groups and the CNS relapse has not been studied to date. Therefore, we aimed to retrospectively analyze the relationship between uric acid levels and CNS relapse risk in patients with newly diagnosed adult DLBCL. Methods: Ninety-four adult newly diagnosed DLBCL patients were retrospectively assessed via electronic records and patient files. Demographic data, IPI and CNS-IPI scores, hematologic parameters, serum lactate dehydrogenase, beta-2 microglobulin, serum uric acid and creatinine levels at diagnosis were recorded for analysis. Uric acid levels were compared between CNS-IPI low-intermediate and high groups and cumulative incidence of CNS relapses were analyzed between normal and elevated uric acid levels. Results: Uric acid levels were found to be significantly higher in CNS-IPI high-risk patients (p = 0.008). Elevated serum uric acid levels at diagnosis were significantly associated with high CNS-IPI risk in the logistic regression analysis (OR 1.34, 95% CI 1.05–1.78, p = 0.047). Uric acid also higher than 5.39 mg/dL showed a discriminatory ability in ROC analyses (AUC 0.633, 95% CI 0.495–0.771, p = 0.05). In the competing risks regression analysis, accounting for non-CNS-related death and progression as the competing events, CNS-IPI subgroups and uric acid levels were not significantly associated with the cumulative incidence of CNS relapse (SHR 0.81, 95% CI 0.12–5.59; Fine–Gray, p = 0.834), (SHR 0.55, 95% CI 0.09–3.47; Fine–Gray, p = 0.526), respectively. Conclusions: Even though uric acid levels are significantly higher and showed a discriminatory ability to detect the CNS-IPI high subgroup, elevated uric acid levels could not predict the CNS relapses.
This study aimed to investigate the clinical manifestations, treatment patterns and clinical outcomes in patients with iTTP across Türkiye via an interim analysis of the Turkish iTTP registry. A total of 215 patients with iTTP (median age at diagnosis 41 years, 58.6% female) diagnosed between 2001 and 2023 were retrospectively analyzed in the interim analysis of the prospective noninterventional observational multicenter iTTP registry study conducted at 19 tertiary hematology centers. Data on patient demographics, disease characteristics on initial admission, treatment characteristics, and responses, exacerbations/relapses, and the survival outcome were registered via electronic case report forms. Infection (15.0%), new drug initiation (9.7%), and pregnancy/postpartum (6.3%) within 3 weeks before diagnosis were the most prevalent potential triggers. Patients presented more commonly with systemic/constitutional (fatigue 68.8%; fever 18.1%) and neurologic (headache 40.0%, vertigo 32.1%), followed by hemorrhagic, gastrointestinal, renal, and cardiovascular manifestations. Based on the PLASMIC risk scoring, 77.8% of patients were initially at high risk for TTP. The initial treatment was commenced within the first 48 hours of hospital admission in 64.1% of patients (36.2% on the day of admission). Treatment was mainly based on TPE exchange (92.1%) and steroids (63.7%), while rituximab was used in 15.8% of patients. The clinical response rate was 79.9%, and clinical remission was achieved in 68.2% of patients. As for the ADAMTS13 levels, partial and complete responses were achieved in 17.7% and 14.6%, respectively. During a median of 30 months (range, 0.1 to 262.4 months) follow-up, 35 patients experienced exacerbations/relapses. Mortality occurred in 11 (5.5%) patients and was found to be disease-related in 6 (3.0%). This interim analysis of the nationwide Turkish iTTP registry study provided valuable data on the real-world clinical practice in the diagnosis and management of iTTP at different hematology clinics across the country.
18 May 2026 in Journal of Current Hematology & Oncology Research
Aims: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by severe ADAMTS13 deficiency, leading to widespread microvascular thrombosis and requiring urgent therapeutic plasma exchange (TPE) and immunosuppression. Although most patients achieve remission with first-line TPE and corticosteroids, approximately 10–40% develop refractory disease requiring additional therapies. Evidence supporting alternative salvage therapies, including vincristine, remains limited. Methods: We conducted a retrospective single-center study of adult patients diagnosed with TTP between 2020 and 2025. All patients received first-line daily TPE plus corticosteroids. Refractoriness was defined as failure to achieve platelet recovery or biochemical improvement after 5–7 days of therapy. Patients laboratory results were obtained from the hospital's record system. Results: A total of 25 patients were included. First-line therapy achieved remission in 48% of patients (12/25). Among 13 refractory patients, rituximab induced remission in 2 (15%). Eleven patients subsequently received vincristine salvage therapy, of whom 9 (81,8%) achieved complete remission, accompanied by rapid platelet recovery and normalization of lactate dehydrogenase levels. Two elderly patients with severe multisystem involvement died despite salvage therapy. Overall survival for the cohort was 92%. Conclusion: Vincristine was highly effective and well tolerated as a salvage therapy in patients with refractory TTP who failed both TPE and rituximab, achieving remission in more than 80% of cases. These findings support vincristine as a valuable therapeutic option, particularly in settings where caplacizumab or other advanced biologics are unavailable. Larger prospective studies are warranted to better define its optimal timing and comparative efficacy among emerging salvage therapies.