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Objective: Real-life studies on patients with primary CNS lymphoma (PCNSL) are scarce. Our objective was to analyze, in a nationwide population-based study, the current medical practice in the management of PCNSL., Methods: The French oculo-cerebral lymphoma network (LOC) database prospectively records all newly diagnosed PCNSL cases from 32 French centers. Data of patients diagnosed between 2011 and 2016 were retrospectively analyzed., Results: We identified 1,002 immunocompetent patients (43% aged >70 years, median Karnofsky Performance Status [KPS] 60). First-line treatment was high-dose methotrexate-based chemotherapy in 92% of cases, with an increasing use of rituximab over time (66%). Patients <60 years of age received consolidation treatment in 77% of cases, consisting of whole-brain radiotherapy (WBRT) (54%) or high-dose chemotherapy with autologous stem cell transplantation (HCT-ASCT) (23%). Among patients >60 years of age, WBRT and HCT-ASCT consolidation were administered in only 9% and 2%, respectively. The complete response rate to initial chemotherapy was 50%. Median progression-free survival was 10.5 months. For relapse, second-line chemotherapy, HCT-ASCT, WBRT, and palliative care were offered to 55%, 17%, 10%, and 18% of patients, respectively. The median, 2-year, and 5-year overall survival was 25.3 months, 51%, and 38%, respectively (<60 years: not reached [NR], 70%, and 61%; >60 years: 15.4 months, 44%, and 28%). Age, KPS, sex, and response to induction CT were independent prognostic factors in multivariate analysis., Conclusions: Our study confirms the increasing proportion of elderly within the PCNSL population and shows comparable outcome in this population-based study with those reported by clinical trials, reflecting a notable application of recent PCNSL advances in treatment., (© 2020 American Academy of Neurology.)

We studied the outcome of 213 patients who received allo-HSCT for hematological malignancies, 121 (57%) from HLA identical siblings, 63 (29%) from 10/10 HLA identical unrelated donors and 29 (14%) from 9/10 HLA mismatched unrelated donors. Engraftment was lower in the 9/10 HLA group (90%) than in the 10/10 HLA group (95%) than in the sibling group (99%); 3 months CI of aGVHD ≥ 2 was 32% (23-41), 20% (15-26) and 27% (23-32) respectively; the one year CI of extensive cGVHD was 21% (13-30), 9% (5-13) and 17% (14-21) respectively. The median OS was 10 months (5-21), 18 months (11-NR) and 60 months (31-NR) respectively with 2-years probability of 19% (8-44), 43% (31-59) and 63% (54-74) respectively. TRM was significantly higher in the 9/10 HLA group with 1 year CI of 45% (35-55), compared to 33% (27-39) in the unrelated 10/10 HLA group and 12% (9-15) in the identical siblings group (p < 0.001).

Background: Extranodal NK/T-cell lymphoma (ENKTL) is a rare form of non-Hodgkin's lymphoma and carries a poor prognosis. Depending on the primary sites of anatomical involvement, it is subcategorized into nasal or extra-nasal ENKTL. Cutaneous involvement is the second localization reported for these lymphomas., Patients and Methods: A woman was admitted for erythematous infiltrative patches on the breasts having an ulcerative course. Cutaneous histopathology showed a dense, diffuse infiltrate of atypical lymphocytes. Immunohistochemistry revealed expression of specific markers for NK-cells and of cytotoxic molecules (TIA-1, granzyme B and perforin), lack of expression of T-cell markers (except positivity of cytoplasmic CD3 and CD2), and the presence of EBV-DNA in lymphoma cells. Positron emission tomography-computed tomography revealed sub- and supra-diaphragmatic multi-organ involvement (kidneys, breasts, stomach, duodenum, lungs, pleural cavity, uterus, bones). No bone marrow infiltration was noted. PCR (polymerase chain reaction) showed high circulating levels of EBV-DNA in peripheral blood. A systemic nasal-type ENKTL was diagnosed. A chemotherapy regimen including high-dose methotrexate, oxaliplatin, gemcitabine, L-asparaginase and dexamethasone was started. Despite good initial therapeutic response, the outcome was rapidly fatal with bone marrow involvement and multi-organ failure., Discussion: Major cutaneous manifestations of ENKTL comprise erythematous infiltrative patches mimicking panniculitis or cellulitis and evolving towards ulceration or necrosis. Subcutaneous nodules may also be noted. Late diagnosis at an advanced stage accounts for the poorer prognosis in extra-nasal ENKTL. In the advanced stages, treatment is based on a chemotherapy regimen including L-asparaginase, possibly followed by autologous or allogeneic hematopoietic stem cell transplantation., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: Lenalidomide in combination with dexamethasone (Len/Dex) is indicated for patients with recurrent/refractory multiple myeloma (RRMM) who were treated with 1 prior therapy until evidence of disease progression. The objective of the current study was to determine the efficacy and safety profile of long-term exposure to Len/Dex., Methods: A total of 50 patients with RRMM who were treated with long-term Len for ≥ 2 years from 2 Intergroupe Francophone du Myélome (IFM) centers (Lille and Nancy) were included in the current study., Results: The median age of the patients was 58 years, with 30% of the patients aged >65 years, 49% having an International Staging System stage of 2 and 3, 12% having severe renal insufficiency, and 8% demonstrating an adverse result on fluorescence in situ hybridization. Approximately 56% of the patients received treatment with Len/Dex for ≥ 3 years. The median duration of treatment with Len/Dex was 3 years (range, 2 years-7 years). The response rates for partial response or better and very good partial response or better for the overall cohort were 96% and 74%, respectively, which is similar to patients exposed to Len for ≥ 3 years. With a median follow-up of 4 years, 19 (38%) patients had stopped treatment with Len/Dex. The time to disease progression rate at 37 months was 78% and 91%, respectively, in patients exposed to Len for 2 years to <3 years and for ≥ 3 years (P=025). The safety profile was manageable, similar to that of Len when administered for a shorter period of time; 16% of patients had grade 3 to 4 neutropenia, 6% had thrombopenia, 6% had anemia, and 20% experienced thromboembolic events, all of venous type. The annual incidence rate of second primary malignancy was 1.96% in the current series., Conclusions: The results of the current study confirmed that the Len/Dex combination is feasible for long-term use in patients with RRMM, with a significant benefit noted in terms of time to disease progression for prolonged treatment with Len/Dex., (Copyright © 2013 American Cancer Society.)

Port wine stains are mainly due to an increase in the number or size of dermal capillary vessels. They are a natural disgrace which, in their most common form (i.e. isolated and non-systematized), requires a cosmetic treatment. It is for this purpose that various therapies were formerly prescribed with inconstant results. Since the development of lasers, notably the argon laser and, most recently, the pulsed dye laser, these treatments have become obsolete. Before they fall into oblivion, it must be remembered that until the seventies radiotherapy was part of the old methods and that all cases of port wine stain now seen in adults must be followed up with particular attention if they have received radiotherapy in their childhood.