Unexpected response of extramedullary plasmacytoma in patients with lung cancer who received nivolumab

Correction to: prediction of thrombosis risk in patients with paroxysmal nocturnal hemoglobinuria The authors determined an error in the affiliation section; it was captured as Department of Hematology, Peking Union Hospital, CAMS & PUMC, Beijing 100,730, China. The correct affiliation should be Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China.

Acute myeloid leukemia with a cryptic NUP98/PRRX2 rearrangement developing after low-dose methotrexate therapy for rheumatoid arthritis

Outcomes of allogeneic stem cell transplantation for DLBCL: a multi-center study from the Kyoto Stem Cell Transplantation Group Abstract Allogeneic hematopoietic stem cell transplantation (allo-SCT) has been considered as a potentially curative treatment option for refractory or relapsed diffuse large B cell lymphoma (DLBCL) patients. However, there is little information available, especially for Japanese patients and in cord blood transplantation (CBT). We aimed to determine treatment outcomes of allo-SCT for DLBCL in the Kyoto Stem Cell Transplantation Group, a multi-institutional joint research group. Sixty-eight DLBCL patients who underwent their first allo-SCT between 2003 and 2016 were included. The median time from diagnosis to transplantation was 13.5 months. Thirty-one patients were in CR/PR at transplantation. Twenty-seven patients underwent CBT. The median follow-up for survivors was 44.2 months. Four-year overall survival (OS) and relapse-free survival (RFS) rates were 23% (95% CI, 13–35%) and 20% (95% CI, 11–31%), respectively. Cumulative incidences of non-relapse mortality and relapse were 23% and 57%, respectively. Patients in CR/PR at allo-SCT had better OS (4-year, 46% vs 4%, P < 0.001) and RFS (4-year, 36% vs 7%, P = 0.005). The source of the stem cell did not significantly affect OS (4-year, bone marrow vs cord blood vs peripheral blood, 28.6% vs 27.2% vs 6.5%, P = 0.193). In multivariate analysis, non-remission status at SCT associated with inferior OS and RFS. Duration from diagnosis to transplantation of less than 1 year associated with inferior RFS. Allo-SCT, including CBT, may be a promising therapeutic modality for DLBCL patients who have good disease control at transplantation.

Double hit B cell precursor leukemia/lymphoma in a patient with a prior diagnosis of follicular lymphoma: a diagnostic and therapeutic dilemma

Hemoglobin Kirklareli [Α 2 59(E7) His>Leu; HBA2:c.176A>T] in a Brazilian child with severe dyspnea and low O 2 saturation

Early risk stratification for diffuse large B-cell lymphoma integrating interim Deauville score and International Prognostic Index Abstract The aim of this study was to evaluate the prognostic relevance of early risk stratification in diffuse large B-cell lymphoma (DLBCL) using interim Deauville score on positron emission tomography-computed tomography (PET-CT) scan and baseline International Prognostic Index (IPI). This retrospective study included 220 patients (median age, 64 years; men, 60%) diagnosed with DLBCL between 2007 and 2016 at our institution, treated with rituximab-based chemotherapy. Interim PET-CT was performed after three cycles of immuno-chemotherapy. Interim Deauville score was assessed as 4 or 5 in 49 patients (22.3%), and 94 patients (42.7%) had high-intermediate or high-risk IPI scores. In multivariate analysis, interim Deauville score (1–3 and 4–5) and baseline IPI (low/low-intermediate and high-intermediate/high) were independently associated with progression-free survival (for Deauville score, hazard ratio [HR], 1.00 vs. 2.96 [95% confidence interval (CI), 1.83–4.78], P < 0.001; for IPI, HR, 1.00 vs. 4.84 [95% CI, 2.84–8.24], P < 0.001). We stratified patients into three groups: low-risk (interim Deauville scores 1–3 and low/low-intermediate IPI), intermediate-risk (Deauville scores 1–3 with high-intermediate/high IPI or Deauville scores 4–5 with low/low-intermediate IPI), and high-risk (Deauville scores 4–5 and high-intermediate/high IPI). This early risk stratification showed a strong association with progression-free survival (HR, 1.00 vs. 3.98 [95% CI 2.10–7.54] vs. 13.97 [95% CI 7.02–27.83], P < 0.001). Early risk stratification using interim Deauville score and baseline IPI predicts the risk of disease progression or death in patients with DLBCL. Our results provide guidance with interim PET-driven treatment intensification strategies.

Aggressive systemic mastocytosis: a diagnostic challenge in a patient with myotonic dystrophy type 2: a case report

Is bendamustine-rituximab a reasonable treatment in selected older patients with diffuse large B cell lymphoma? Results from a multicentre, retrospective study Abstract Despite bendamustine-rituximab (BR) showed disappointing efficacy in diffuse large B cell lymphoma (DLBCL), it is still occasionally used as first-line treatment in older DLBCL patients instead of recommended R-CHOP. This multicentre, retrospective study was aimed to clarify circumstances in which BR may be justified in this setting. Patients ≥ 65 years with ECOG performance status (PS) ≥ 2 or ≥ 75 years regardless of PS were included. A total of 140 patients were analysed (BR, 68; R-CHOP, 72). BR patients were older (p < 0.001) and were diagnosed more often with high-risk disease (p = 0.03); no difference regarding comorbidities or PS was seen. Compared with R-CHOP, BR was associated with marked inferior overall survival (OS) (16.3 vs. 75.4 months; p = 0.006) and progression-free survival (PFS) (11.0 vs. 62.3 months; p < 0.001). In multivariate analysis, only high age-adjusted Charlson Comorbidity Index (aaCCI) was associated with inferior PFS in R-CHOP patients (hazard ratio 2.67; p = 0.012). Comparing the subgroup of BR and R-CHOP patients with high aaCCI, there was no difference in OS (p = 0.73) or PFS (p = 0.75). Due to the observed non-superiority of R-CHOP in older DLBCL patients with comorbidities, we propose that this subgroup may be treated alternatively with BR, whereas all other older patients are clearly R-CHOP candidates.

Socioeconomic Position is Positively Associated with Monoclonal Gammopathy of Undetermined Significance in a Population-based Cohort Study Abstract Knowledge of social inequalities in monoclonal gammopathy of undetermined significance (MGUS) will contribute to understanding multiple myeloma (MM) etiology, as MGUS consistently precedes MM. The aim of the present study was to examine whether socioeconomic position (SEP) is associated with MGUS in a population-based cohort including information on potential MGUS risk factors. Overall, 4787 study participants aged 45–75 years with information on MGUS were included. SEP indicators (education, income) and potential risk factors (i.e., body mass index, diabetes, smoking, dietary factors) were assessed at baseline. Overall, 260 MGUS cases were detected at baseline and prospectively over a 10-year follow-up. In age-adjusted logistic regression models, a lower chance of having MGUS at baseline or developing MGUS during 10 years of follow-up was indicated for groups of low SEP with odds ratios (OR) of 0.39 (95% confidence interval [95%-CI] 0.19–0.76) for women and 0.48 (95% CI 0.10–1.16) for men in the lowest compared to the highest educational group. After additionally including potential mediating risk factors in the regression models, the estimated ORs changed only slightly in magnitude. Similar results were obtained for income. Current smoking and low fruit consumption were associated with MGUS independently of SEP in women, but not in men. The present study indicates a lower MGUS risk in lower SEP groups. Supporting evidence is given that smoking and diet play a role in the development of MGUS independently of SEP, while it has to be assumed that risk factors unknown to date are responsible for the observed social inequalities in MGUS.