BackgroundWaldenström Macroglobulinemia (WM) is a rare subtype of indolent B-cell lymphoma, and prospective randomized studies on WM are scarce. The R-CHOP therapy [rituximab (R), cyclophosphamide, hydroxy-doxorubicin, vincristine, and prednisone] is a popular and recommended regimen for primary therapy, prescribed by several treatment guidelines for WM. However, treatment with R-CHOP is accompanied by severe myelosuppression and high rates of peripheral neuropathy. Therefore, we retrospectively evaluated the efficacy and toxicity of half-dose CHOP combined with R as a primary therapy for WM.MethodsPatients with untreated symptomatic WM, treated at the Disaster Medical Center between April 2011 and September 2016, were retrospectively analyzed after administration of 6 cycles of half-dose R-CHOP for every 3 weeks. The response, median time to response, best response, progression-free survival, overall survival, and toxicities were evaluated.ResultsOf the 20 WM patients analyzed, 16 (80%) received half-dose R-CHOP without vincristine, and 13 (65%) responded to the treatment. With a median follow-up duration of 26.3 months, the 2-year progression-free survival and 2-year overall survival rates were 70 and 93.3%, respectively. The median time to response and best response were 6 and 9.9 weeks, respectively. Grade 3/4 leukocytopenia, neutropenia, febrile neutropenia, and Grade 1 peripheral neuropathy developed in 32, 37, 0, and 21% of patients, respectively.ConclusionThe half-dose R-CHOP is an effective and well-tolerated primary therapy for WM. To the best of our knowledge, this is the first study reporting the use of a reduced-dose R-CHOP regimen for the primary treatment of WM.
To the Editor, Rearrangements of PDGFRA, PDGFRB, FGFR1 or JAK2 are established features of myeloid/lymphoid neoplasms with eosinophilia (MLN-Eo). 1 The rearrangement of the fms-related tyrosine kinase 3 (FLT3) gene should also be associated with MLN-Eo, and ETV6, SPTBN1, GOLGB1 and TRIP11 have been identified as FLT3 rearrangement partner genes 2 (Figure S1). Cases of MLN-Eo with FLT3 rearrangement are rare but have a poor outcome.We encountered a patient who achieved a favourable long-term outcome by allogeneic haematopoietic stem cell transplantation (allo-HSCT) and without using tyrosine kinase inhibitors, despite being refractory to conventional chemotherapy. The coiled-coil domain containing an 88C (CCDC88C)-FLT3 translocation was identified in this patient who was diagnosed with myeloid neoplasm with T-cell lymphoblastic lymphoma (T-LBL). Chronic myelomonocytic leukaemia (CMML) was one of the differential diagnoses for the current patient; the criteria of chronic myelomonocytic leukaemia included not having the specific genes, such as PDGFRA, if eosinophilia was present. 1 The current case showed a FLT3 rearrangement, and therefore we considered a diagnosis of MLN-Eo as reasonable. The CCDC88C-FLT3 translocation was identified in T-LBL, CD34-positive haematopoietic stem and multilineage cells.We speculated that FLT3 was associated with the t(13;14)(q12;q32) translocation. Previous reports indicated FLT3 breakpoints in theThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Secondary immune thrombocytopenic purpura (ITP) with non-Hodgkin lymphoma (NHL) is a rare disease. Although some treatment regimens are available for primary ITP, the treatment strategy for secondary ITP remains unconfirmed. We herein report a 79-year-old man who was diagnosed with secondary ITP with mantle cell lymphoma. Although intravenous immunoglobulin (IVIG) has been considered an effective option for secondary ITP, similar to the treatment of primary ITP, our patient did not benefit from IVIG. A literature review including the current report revealed that IVIG was ineffective in all treated patients. Secondary ITP with NHL should be treated differently from primary ITP.
Background: According to VISTA study, the prognosis of elderly multiple myeloma (MM) patients has been improved with VMP therapy. However, intensive chemotherapy has induced severe adverse events (AEs), resulting in highly discontinued rate, especially often seen in unfit or frail patients. Several studies have demonstrated the potent of weekly bortezomib administration in induction therapy (GIMEMA, GEM2005MAS65), and maintenance therapy with bortezomib. Based on these fact, we designed phase 2 clinical study, BoRtezomib-based Optimized therapy Aiming Disease control in Japan (BROAD-J study), based on a weekly VMP as induction therapy followed by maintenance therapy with bortezomib (Bor-MT) with every two weeks administration for newly-diagnosed (ND) symptomatic non-transplant eligible MM patients. (UMIN 00007335) Method: From August 2011 to June 2016, according to the International Myeloma Working Group (IMWG) criteria, 87 patients older than 65 years with symptomatic MM were eligible for this trial (Table 1). The primary objectives were included maximum response and time to progression duration. Secondary objective was the continued treatment duration and adverse event rate. The induction phase consisted of VMP treatment: Melpharan: on days 1-4 (6 mg/m2), every 35-day cycle; prednisolone: on days 1-4 (40mg/d, every 35-day cycle; Bortezomib: 1.3mg/m2, d1, 8, 15, 22 every 35-day cycle. After 9 cycles of VMP therapy, the maintenance therapy starts. The maintenance phase was consisted of twice a month bortezomib administration (1.3 mg/m2) without dexamethasone until progressive disease. Dose reductions of bortezomib were allowed according to instructorfs recommendation. Patients could receive supportive therapy including bisphosphonates and transfusions as necessary. AEs were graded according to NCI-CTCAE v4.0. Response was assessed prior to every treatment cycle. Response categories were based on the International Myeloma Working Group uniform response criteria. Results: Mean age was 75 years (66-88), sex ratio was 44:43 (M: F), and ISS stage was I 12%, II 43%, III 43%. High risk cytogenetics abnormalities (i.e. del17p, t(14;16)) were observed in 13 patients (15%). Overall response rate was 87% and complete response was obtained in 22 patients (25%) (Table 1). Median progression free survival (PFS) was 36 months (Figure 1a) and median overall survival was 57 months (Figure 1b). Median PFS (Kaplan-Meier estimate) was significantly higher with patients who obtained CR or VGPR than PR or SD (P<0.0001) (Figure 1c). Significantly higher ORR, rate of VGPR or better, and rate of CR or better were achieved in patients with revised ISS I stage, respectively. In induction phase, 32% of patients were suffered from greater than grade 3 AEs, whereas only 3% had grade 3 or 4 AEs in maintenance phase. Discussion: This trial reveals that VMP regimen with weekly bortezomib administration is effective and tolerablefor unfit or frail elderly MM patients. In addition, maintenance therapy of twice a month bortezomib administration was effective in patients who obtained VGPR or more better. These results are no way inferior to results of VISTA trial. However, high R-ISS stage patients or poor treatment responders did not obtain the benefit of this setting, so, another setting with anti-myeloma antibody (i.e. elotuzumab or daratumumab) may be warranted in the future for such patients. Conclusion: BROAD-J study is conducted to yield the possibility of a careful treatment toward at least PR state and the efficacy of VMP followed by bortezomib maintenance in unfit or frail MM patients. The excellent results we demonstrated such as a good OS, PFS, and a low incidence of AEs might be suggested a superiority of this strategy. Figure 1 Figure 1. Disclosures Tokuhira: Bristol Myers Squibb Co., Ltd: Honoraria; Eizai Co., Ltd: Honoraria; Pfizer Co., Ltd: Honoraria.
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