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Primary plasma cell leukemia (pPCL) is a rare and aggressive variant of multiple myeloma (MM) with poor long-term survival after cytotoxic chemotherapy. Many novel drugs have revolutionized the treatment algorithms for MM. The impact of targeted therapy, both pre- and post-autologous stem cell transplant (auto-HCT) remains an area of ongoing interest. In this study, we report outcomes post auto-HCT for pPCL and the impact of maintenance therapy posttransplant with novel agents.

Abstract Introduction Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is a lymphoid neoplasm of with heterogeneous cytogenetic and molecular abnormalities that are used for prognostication. We aimed to do a clinic-pathological study of CLL/SLL with complex karyotype (≥3 abnormalities) and assess risk of Richter transformation. Methods We identified 333 patients with CLL/SLL having complex karyotype at a single institution from January 1969 to December 31, 2015. Clinicopathological parameters were extracted from institutional databases. Logistic regression (SAS 9.4) was applied to test association between parameters and transformation. Results Median age was 64 years. Patients included 250 men (75.01%) and 83 women (83%). Laboratory parameters were as follows (median [range]): Hb 11.1 (9.6–12.7) g/dL, WBC 20.6 (7.2–70.9) K/uL, platelets 100 (50–160) K/uL, absolute lymphocyte count 14.96 (3.93–61.11) K/uL, LDH 698 (547–939) IU/L, B2 microglobulin 4.7 (3.2–6.6). Splenomegaly was present in 97 (29.13%) patients, bulky lymphadenopathy in 20 (6.01%). In patients that had results available, CLL cells were positive for ZAP70 in 71% and CD38 in 182 (64.76%) patients. VH mutation was present in 42 (14%). Cytogenetic abnormalities were as follows: -17 in 20 (5.70%), trisomy 12 in 56 (16.82%). A total of 39 patients developed Richter transformation; of these 24 (61.54%) were men and 15(38.46%) women, and median age at time of transformation was 59.5 years. Of 31 patients with survival data, median survival time for Richter transformation was 6.91 years as compared to 7.9 years for 147 subjects without. Only Hb was associated with increased risk of developing Richter transformation. Conclusion CLL/SLL with complex karyotype is a heterogeneous disease at the genetic level. Only Hb was associated with risk for Richter transformation.

The impact of bone marrow fibrosis grade on the prognosis of patients with chronic myelomonocytic leukemia (CMML) remains controversial. Therefore, we examined the records of 82 patients diagnosed with CMML at our institution and summarized baseline characteristics and molecular profiles by subgroups of absent or mild (grades 0/1) and moderate (grade 2) fibrosis. Cox proportional hazards models were constructed to assess the prognostic significance of fibrosis grade. Grade 2 fibrosis was identified in 63 patients (76.8%), grade 1 in 16 patients (19.5%), and grade 0 in 3 patients (3.7%). Grade 2 fibrosis was associated with reduced hemoglobin levels (median 9.75 vs 11.0 g/dL in grade 0/1; p = 0.04) and increased percentages of ringed sideroblasts (7.5 vs 0%; p = 0.008). In multivariable analysis, grade 2 fibrosis was an independent predictor of poor overall survival (OS; 95% CI 1.32–6.35; HR 2.90; p = 0.008), but not event-free survival (EFS; 95% CI 0.62–2.67; HR 1.28; p = 0.50). Absolute neutrophil count (ANC) was found to impact OS (95% CI 1.01–1.09; HR 1.05; p = 0.009), while both ANC (95% CI 1.00–1.07; HR 1.04; p = 0.04) and peripheral blood blast percentage (95% CI 1.02–1.32; HR 1.16; p = 0.02) impacted EFS. These results implicate fibrosis grade is an important indicator of prognosis, with high-grade fibrosis predicting inferior survival. Given the prevalence of marrow fibrosis in CMML, fibrosis grading should be incorporated into prognostic assessment and therapeutic decision-making.