Successful resolution of Hemophagocytic lymphohistiocytosis associated to brucellosis in the adult.

Hemophagocyticlymphohistocytosis (HLH) is a proliferation of histiocytes with importanthemophagocytosisoccurring in different organs such as the spleen and the bone marrow. HLH is now increasingly diagnosed in the context of infections, malignancies and connective tissue diseases. Although brucellosis is an endemic infection in Tunisia, its association with HLH is a very rare condition which should be considered in patients with splenomegaly and cytopenia. Here, we describe brucellosis associated HLH in a 31 year-old man. The patient was admitted to our hospital with fever, sweating, and fatigue. Physical and laboratory findings revealed splenomegaly, pancytopenia, elevated serum transaminases, triglycerides, lactate dehydrogenase, and ferritin, and bone marrow hemophagocytosis. The Brucella agglutination test was positive. The patient improved after treatment with Rifampin and doxycyclin.

[Shwachman-Diamond syndrome: a case report]. / Le syndrome de Shwachman-Diamond : à propos d'un cas.

Shwachman-Diamond syndrome is a constitutional disorder characterized by exocrine pancreatic failure and neutropenia with dysgranulopoiesis. It is a rare disease, with less than 100 cases reported in France. Here we report the case of a 23-year-old woman with this syndrome. The clinical feature and the diagnostic steps are described, as well as the evolution and management in medical and laboratory medicine practice.

[Chronic lymphocytic leukemia with t(14 ;18) and trisomy 12: a case report]. / Leucémie lymphoïde chronique avec t(14;18) et trisomie 12 : un cas clinique.

Chronic lymphocytic leukemia (CLL) is a B-cell neoplasm defined by the presence of at least 5×109 G/L monoclonal B lymphocytes in the peripheral blood. It is the most common type of leukemia in adult patients from Western countries. CLL is characterized by a gradual accumulation of small, longliving, immunologically dysfunctional, morphologically mature-appearing B-lymphocytes in blood, bone marrow and lymphoid tissues. It has also been reported that CLL cells have a proliferation rate higher than previously recognized, particularly in the lymphoid tissues. The flow cytometry analysis of typical CLL identifies a monotypic B-cell population expressing a low level of surface immunoglobulins, light chain being either kappa or lambda-, CD5+, CD19+, CD23+, CD79b (dim), negative for FMC7 and CD10. Clinical presentation, course and outcome are highly variable. Interphase fluorescent in situ hybridization (I-FISH) identifies chromosomal abnormalities in about 80% of cases, most commonly involving 13q14 (55%), 11q22-23 (18%), or 17p13 deletions (7%) and trisomy 12 (16%). Therefore, five prognostic categories have been defined with a statistical model, showing the shortest median survival and treatment-free intervals in patients harboring 17p and 11q deletions, followed by trisomy 12 and a normal karyotype, whereas 13q deletion as the sole abnormality is associated with the best prognosis. We report here a rare case of CLL in a 54 year-old-man.

E hepatitis seroprevalence in a population of patients with transaminases increased at Le Mans' hospital (France).

In Europe, infection through the hepatitis E virus presents itself under the form of autochthone, sporadic cases with zoonotic transmission. The diagnosis is more frequent in the case of male subjects above 50, with a generally severe clinical presentation. Moreover, a transition to chronicity is described in the case of immunocompromised patients. In France and in the world, seroprevalence has not clearly been established yet. In the hospital of Le Mans, Sarthe, France, the serologic status regarding the hepatitis E virus was established between October 1(st) 2012 and April 3(rd) 2015 on a population of 299 patients who presented hypertransaminasaemia and negative serology regarding hepatitis A, B and C viruses. According to the Wantai(®) Assays technique, seroprevalence for IgG among this cohort was 26.1%, while 3.7% of patients presented acute hepatitis E and a positive IgM serology. Hepatitis E screening tests are indispensable in the context of acute hepatitis no A, no B, no C.

[Blastic plasmacytoid dendritic cell neoplasm: two case reports]. / Leucémie dérivée des cellules dendritiques plasmocytoïdes : à propos de deux cas.

Blastic plasmacytoid dendritic cell neoplasm (LPDC) is a rare and aggressive leukemia entity with cutaneous and extracutaneous involvement, reaching most often lymph, blood and bone marrow. Two cases of LPDC diagnosed in Hospital Center of Le Mans are reported, a 78 year old woman (case 1) and a 82 year old man (case 2), and have been clinically, biologically and histologically documented. The clinical presentation, diagnostic difficulties are reminded, as well as the pathogenesis and therapeutic aspect.

[Vancomycin-induced DRESS syndrome: a case report]. / DRESS (Drug reaction with eosinophilia and systemic symptoms) suite à la prise de vancomycine : à propos d'un cas.

DRESS syndrome is a severe adverse drug-induced reaction, characterized by generalized skin rash associated with hypereosinophilia, lymphocytosis and internal organ involvement. Antiepileptics, sulfamides and allopurinol are the most frequently reported drugs; vancomycin is less common. We report a case of vancomycin-induced DRESS syndrome in a 69-year-old male patient. Clinical symptoms and diagnosis difficulties are reported through this observation as well as pathogenesis and treatment of this syndrom.