La leucémie lymphoïde chronique

Chronic lymphocytic leukemia (CLL) is a low-grade B cell lymphoma with circulating cells, often revealed by hyperlymphocytosis. Its diagnosis and therapeutic indications (not systematic) have been defined in 2018. In fact, CLL can be separated in two entities differentiating themselves by their IGHV mutational status, but the search of other prognostic parameters like TP53 disruption is mandatory before treatment. Numerous genetic alterations and mutations exist in CLL. CLL cells are highly dependent from their b-cell receptor stimulation and from their microenvironment, which takes a central place in disease progression. Infections, dysimmune manifestations, cancers and Richter transformation are classic complications, and patients have poor vaccine response even without a treatment. Chemoimmunotherapy is being challenged by the new highly active drugs such as Bruton tyrosine-kinase inhibitors (ibrutinib, acalabrutinib) and by the association of venetoclax and anti-CD20. Future treatment strategies might integrate both new drugs and classical chemoimmunotherapy.     

Néphropathies au cours de la leucémie lymphoïde chronique : à propos d’une étude monocentrique de 10 cas

Introduction

Chronic lymphoid leukemia (CLL) is a hematological malignant disease, associated with a clonal B cell proliferation. The incidence is 4400 new cases per year in France. The prevalence increases with age with a median age at diagnostic of 65 years. Renal involvement is rare and estimated at 1.2% of patients with CLL. Renal pathological diagnoses associated with CLL are variable and are not always related to the hematological disease. We report here on cases of patients with CLL who underwent a renal biopsy over the past 16 years in Marseille.

Déficit non amyloïde acquis en facteur X : un premier cas satellite d’une leucémie lymphoïde chronique atypique et revue de la littérature

Introduction

Acquired factor X deficiency is in most cases associated with AL amyloidosis. Acquired non-amyloid related factor X deficiency (DNAA-FX) has been exceptionally reported in the literature.

Un cas de manifestation cutanée d’un syndrome hyperéosinophilique lymphoïde

IntroductionLymphoid hypereosinophilic syndrome (HES) is a reactive HES, related to the presence of an abnormal circulating T cell clone. Cutaneous manifestations are frequent and sometimes inaugural, however few studies describe them specifically.Case reportWe report the case of a 63-year old patient, in good general condition, with no previous history and taking no treatment, who was being followed for non-specific skin lesions. Blood and skin examinations showed hypereosinophilia, the presence of an aberrant CD3-CD4+ phenotype and a positive T-clonality test. There was no differential diagnosis or argument for a systemic lymphoma.ConclusionCutaneous manifestations of lymphoid HES are variable, non-specific, and may differ according to lymphocyte phenotype. The discovery of SHE requires an extension workup and the risk of evolution towards a systemic lymphoma justifies a close surveillance. Treatment is adapted to the severity of the symptoms.     

Un cas rare de leptospirose révélé par une pancréatite aiguë

Leptospirosis is the most spread anthropozoonosis in the world. Its clinical appearances are highly polymorphic ranging from a pseudo-flu-like syndrome to a potential lethal multivisceral failure. Acute pancreatitis is an exceptional complication of leptospirosis. We report a case of leptospirosis revealed by acute pancreatitis with a good clinical outcome after medical treatment.     

Maladie de Gaucher de type 1 simulant une thrombopénie immunologique : intérêt de l’hyperferritinémie et de l’hypergammaglobulinémie dans le bilan d’une thrombopénie isolée

IntroductionGaucher disease type 1 is a rare genetic disease. It can cause thrombocytopenia. Current guidelines do not support bone marrow examination in front of isolated thrombocytopenia if no evidence suggests malignant hemopathy. This strategy aiming at sparing unnecessary investigations makes such rare diseases more difficult to diagnose.Case reportA 31-year-old woman was diagnosed with immune thrombocytopenia according to current guidelines. She presented later with mild splenomegaly. Bone marrow aspirate smears showed Gaucher cells. Gaucher disease was then confirmed. Looking backward, initial biological clues (hyperferritinemia, hypergammaglobulinemia) should have enabled to consider the diagnosis.ConclusionGaucher disease type 1 can be responsible for apparently isolated thrombocytopenia. The disease must be looked for if the thrombocytopenia is associated with unexplained hypergammaglobulinemia or hyperferritinemia. Diagnosing immune thrombocytopenia without bone marrow sample requires to systematically pay attention to any clinical or biological abnormality, not to ignore rare differential diagnoses.     

Un cas de pyohémie,de concert avec une méningite aiguë et des arthrites,provoquée par le bacille de Pfeiffer.

La moitié de tous les cas de pleurésie se rencontrent de 16 à 25 ans. Dans notre statistique la morbidité et la mortalité par tuberculose après la pleurésie sont plus faibles que dans celle de Köster, mais plus élevées que dans celle des sociétés d'assurances. La morbidité par tuberculose est surtout élevée dans les trois premières années qui suivent la pleurésie (10.2 %, 4.4 % et 4.2 %); la mortalité l'est surtout pendant les quatre premières années (de 2.8 à 1.7 %). La mortalité de la tuberculose consécutive à la pleurésie est plus faible chez les enfants que chez les adultes. Les grands épanchements s'accompagnent d'une fièvre plus prolongée et sont suivis de tuberculose plus souvent que les exsudats moins abondants et les pleurésies sèches. L'infection d'origine familiale ou domiciliaire est d'une fréquence égale aussi bien chez les individus demeurés ultérieurement bien portants que chez ceux devenus tuberculeux; il en est de měme pour les différentes formes de pleurésie. La pleurésie débute plus souvent en mars—mai que dans les autres mois (carence de vitamines?).     

La pancréatite aiguë au cours de la rectocolite ulcérohémorragique : complication rare de la mésalazine

Acute pancreatitis secondary to mesalazine is rare. Thirty-nine cases were described. We report a new case of pancreatitis during treatment of distal ulcerative colitis (UC) with mesalazine in a 38-year-old woman, without alcohol consumption. Treatment with enema and oral mesalazine was started. One week later, the patient was admitted for pancreatic epigastric pain; lipasemia was raised to threefold the upper normal limit. Ultrasound and abdominal CT scan showed an enlarged heterogeneous pancreatic head without Wirsung or biliary ducts abnormalities; treatment was stopped with regression of symptomatology as well as normalization of lipasemia after 10 days. Acute pancreatitis secondary to mezalasine is a rare side effect in the treatment of chronic inflammatory bowel diseases. This diagnosis must be evoked in any patient receiving mesalazine and presents pancreatic epigastric pain after excluding other causes of pancreatitis, particularly autoimmune etiology.     

Hépatite aiguë grave au paracétamol chez un enfant de cinq ans au CHU de Brazzaville

We report a case of serious acute hepatitis with paracetamol in a 5-year-old child, hospitalized for icterus, appeared after the paracetamol administration amount of 100 mg/kg per day during one week. The diagnosis of acute hepatitis with paracetamol was retained on the basis of chronological criteria; repetition of the signs of hepatitis to the paracetamol readministration; absence of other causes and score of imputability to 10.