Une endoscopie redoutable [A terrible endoscopy].

International audienceNous exposons ici le cas d’un lymphome intra-vasculaire diagnostiqué sur biopsies duodénales réalisées dans le cadre du bilan d’une altération de l’état général et de douleurs épigastriques évoluant depuis 3 mois chez un homme de 77 ans. Cette entité très rare, appartient au sous-groupe des lymphomes B diffus à grandes cellules dans la classification OMS 2008. Il se caractérise par une localisation quasi exclusive des cellules lymphomateuses dans les capillaires et les vaisseaux de petit calibre. Le pronostic de cette hémopathie généralement diagnostiquée post mortem reste de nos jours très péjoratif, notamment du fait du retard diagnostic en lien avec le caractère peu spécifique des symptômes. L’originalité de notre cas tient à son diagnostic précoce réalisé sur biopsies duodénales dans un contexte de douleurs épigastriques sans anomalie endoscopique, révélant une atteinte viscérale disséminée confirmée par TEP scanner et myélogramme. L’atteinte ganglionnaire et l’infiltration médullaire permettent également de discuter le sous-type rarissime dit « asiatique »

Pneumopathie induite par l'hydroxyurée. [Hydroxyurea-induced pneumonia].

International audienceIntroduction: Hydroxyurea is an antimetabolite widely used in the treatment of myeloproliferative diseases. Usual side effects are mainly hematological, gastrointestinal, neurological disorders and induced-fevers. More rarely, hydroxyurea-induced pneumonitis are reported. Case report: We report a case of a patient treated for polycythemia vera. She was admitted 20 days after introduction of hydroxyurea for a high fever, productive cough and clear sputum associated with nausea. Chest CT-scan found diffuse bilateral ground-glass opacities. The microbiological investigations were negative. Symptoms disappeared few days after discontinuation of treatment. Its reintroduction led to recurrence of symptoms. Conclusion: This additional case completes the 15 cases of hydroxyurea-induced pneumonitis described in the literature. Two forms of this disease seem to exist: an acute form with fever occurring in the month following introduction of hydroxyurea; and a chronic form without fever. Even if it is uncommon, pulmonologists should be aware of this complication

Les leucémies LGL-NK (caractéristiques d'une cohorte internationale de 74 patients)

La classification WHO 2008 distingue parmi les leucémies à grands lymphocytes à grains (LGL) 3 entités: les leucémies LGL T, les leucémies LGL de sous-type Natural Killer (NK) agressives, et l'entité provisoire des lymphoprolifération chroniques NK. Peu de publications portent sur ces dernières. Nous avons donc colligé de manière rétrospective les données de 74 cas internationaux de leucémies LGL NK. L'âge médian était similaire (60 ans) à celui des leucémies LGL T, comme la fréquence des syndromes tumoraux (26%), des manifestations auto-immunes (24%) et des hémopathies associées (11%). En revanche, il existait significativement plus de patients asymptomatiques au diagnostic, et moins de polyarthrites rhumatoïdes associées. Il existait significativement moins de neutropénies, notamment sévères. Une expression tronquée des récepteurs de surface était retrouvée chez 85% des patients qui avaient bénéficié d'une étude immunophénotypique complète, en faveur du caractère clonal de la prolifération. Il existait une mutation STAT3 dans 12% des cas étudiés. La survie globale était prolongée, confirmant le caractère chronique de cette pathologie. Ces données mettent en évidence des similarités mais également des disparités cliniques et biologiques entre leucémies LGL T et lymphoproliférations chroniques NK, justifiant la place de cette entité provisoire.RENNES1-BU Santé (352382103) / SudocSudocFranceF

Large granular lymphocyte leukemia associated with hepatitis C virus infection and B cell lymphoma: improvement after antiviral therapy.

International audienc

Characteristics, management and outcome of acquired amegakaryocytic thrombocytopenia.

International audienc

Dexamethasone is associated with early deaths in light chain amyloidosis patients with severe cardiac involvement

International audienceBackgroundCardiac light chain amyloidosis (AL-CA) patients often die within three months of starting chemotherapy. Chemotherapy for non-immunoglobulin M gammopathy with AL-CA frequently includes bortezomib (Bor), cyclophosphamide (Cy), and dexamethasone (D). We previously reported that NT-ProBNP levels can double within 24h of dexamethasone administration, suggesting a deleterious impact on cardiac function. In this study, we evaluate the role of dexamethasone in early cardiovascular mortality during treatment.Methods and findingsWe retrospectively assessed 100 de novo cardiac AL patients (62% male, mean age 68 years) treated at our institute between 2009 and 2018 following three chemotherapy regimens: CyBorDComb (all initiated on day 1; 34 patients), DCyBorSeq (D, day 1; Cy, day 8; Bor, day 15; 17 patients), and CyBorDSeq (Cy, day 1; Bor, day 8; D, day 15; 49 patients). The primary endpoint was cardiovascular mortality and cardiac transplantation at days 22 and 455. At day 22, mortality was 20.6% with CyBorDComb, 23.5% with DCyBorSeq, and 0% with CyBorDSeq (p = 0.003). At day 455, mortality was not significantly different between regimens (p = 0.195). Acute toxicity of dexamethasone was evaluated on myocardial function using a rat model of isolated perfused heart. Administration of dexamethasone induced a decrease in left ventricular myocardium contractility and relaxation (p<0.05), supporting a potential negative inotropic effect of dexamethasone in AL-CA patients with severe cardiac involvement.ConclusionDelaying dexamethasone during the first chemotherapy cycle reduces the number of early deaths without extending survival. It is clear that dexamethasone is beneficial in the long-term treatment of patients with AL-CA. However, the initial introduction of dexamethasone during treatment is critical, but may be associated with early cardiac deaths in severe CA. Thus, it is important to consider the dosage and timing of dexamethasone introduction on a patient-severity basis. The impact of dexamethasone in the treatment of AL-CA needs further investigation

Rituximab plus gemcitabine and oxaliplatin (R-GemOx) in refractory/relapsed diffuse large B-cell lymphoma: a real-life study in patients ineligible for autologous stem-cell transplantation.

There is no established standard treatment for relapsed/refractory (R/R) diffuse large B-cell lymphoma (DLBCL) in patients who are not eligible to receive an intensive treatment. The combination of rituximab gemcitabine and oxaliplatin (R-GemOx) is widely used in this population but data are scarce. We retrospectively collected the data of 196 patients with R/R DLBCL treated with R-GemOx in two French centers over a period of 15 years. The median age of the population was 72 years (range, 24-89), 63% of the patients had an international prognostic index of 3 or higher and 57% were refractory to the last treatment. At the end of R-GemOx treatment, 33% of the patients obtained a complete response. The median progression-free survival (PFS) of the population was 5 months and the median overall survival (OS) was 10 months. Several factors were predictors of unfavorable survival: age over 75 years, international prognostic index of 2 or higher, refractory disease and de novo DLBCL. The median PFS and OS of the patients who obtained a complete response were 22 months and 40 months, respectively. The most significant toxicities were grade 3-4 hematological toxicities (31% of patients). Given its efficacy and tolerability, R-GemOx can be used in patients ineligible for intensive treatment and serve as a basis for new regimen combinations