[Inherited primitive and secondary polycythemia].

Myeloproliferative disorders and secondary polycythemia cover most of the polycythemia cases encountered in daily practice. Inherited polycythemias are rare entities that have to be suspected when the classical causes of acquired polycythemia have been ruled out. Recent advances were made in the understanding of these pathologies, which are still little known to the physicians.

Thymoma associated with autoimmune diseases: 85 cases and literature review.

Objectives: To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma.

Design: Pathological records from three university hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD.

[Auto-immune diseases and cancers. Second part: auto-immune diseases complicating cancers and their treatment].

Autoimmune diseases may reveal or occur during the course of a neoplasia or its treatment. Autoimmune cytopenia, especially haemolytic anaemia, is common in lymphoproliferative disorders such as chronic lymphoid leukemia. The link between cancer and myositis is well established.

[Combined variable immunodeficiency with unusal features. A case report].

Introduction: The association granulomatosis - combined variable immunodeficiency (CVID) - is well known from the clinicians. However, the association with a large granular lymphocyte (LGL) leukemia has not been yet reported.

Case Report: We report a 50-year-old woman, followed for CVID associated with a granulomatous disease.

Axitinib-induced acute pancreatitis: a case report.

Axitinib is an oral second-generation selective inhibitor of vascular endothelial growth factor receptors recently approved for the treatment of advanced renal cell carcinoma. Numerous cases of acute pancreatitis have been reported after treatment with nonselective tyrosine kinase inhibitors such as sorafenib and sunitinib. We present the first report of a patient under axitinib treatment presenting with acute pancreatitis for which no other etiology has been found.

[Complicated course of eosinophilic gastroenteritis: a case report and literature review].

Introduction: Eosinophilic gastroenteritis is an unusual disease characterized by an eosinophilic infiltration of the gastrointestinal tract. The esophageal location of this disorder is uncommon and is usually revealed by dysphagia. Diagnosis is obtained by histology during endoscopy after exclusion of differential diagnosis.

[Autoimmune diseases and cancers. Part I: cancers complicating autoimmune diseases and their treatment].

The link between systemic disease and cancer is not fortuitous. An autoimmune disease can represent the starter for developing a non-Hodgkin lymphoma. This is particularly true for Sjögren's syndrome that is associated with the highest risk of lymphoma (odds ratio up to 44).

[Understand the neurodevelopment of language: a necessity to prevent learning disabilities in children].

Clinical and radiological knowledge of language development in the former premature infant compared to the newborn allows us to argue for exploration of the sensorimotor co-factors required for proper language development. There are early representations of the maternal language in the infant's visual, auditory, and sensorimotor areas, activated or stabilized by orofacial and articulatory movements. The functional architecture of language is different for vulnerable children such as premature infants.

Gelatin-thrombin matrix: a new and simple way to manage recurrent epistaxis in hematology units.

Introduction. In case of thrombopenia and/or thrombopathy, epistaxes are very difficult to manage. Case Series.

[Renal vein infarction, a complication of paroxysmal nocturnal hemoglobinuria].

Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli disease) is a rare acquired clonal disorder of the hematopoietic cell, to a somatic mutation in the phosphatidylinositol glycan (PIG-A). The most frequent clinical manifestations are hemolytic crisis and venous thrombosis of the mesenteric, hepatic, portal or cerebral territories. We report a case of paroxysmal nocturnal hemoglobinuria with renal vein thrombosis, a rare complication of this disease.

[Plasmacytosis and dengue fever: an underestimated abnormality?].

Introduction: A significant peripheral blood plasmacytosis is a rare finding associated with viral infections. We reported five consecutive cases of dengue virus infection, with circulating plasma cells.

Case Reports: Three women and two men, aged 26 to 75 years, had returned from French West Indies less than one week before the onset of the symptoms (mean: 2.

Uveitis and common variable immunodeficiency: data from the DEF-I study and literature review.

Purpose: To examine the relation between uveitis and common variable immunodeficiency (CVID).

Methods: Retrospective analysis of patients included in the French DEFI cohort of adults with CVID and of patients identified by reviewing the literature.

Results: Four patients were identified in the DEFI study (frequency of uveitis: 1.

[Granulomatosis and primary immunodeficiency in adulthood].

Primary immunodeficiency with granulomatosis in the adulthood mainly concern common variable immunodeficiency (CVID). Hypogammaglobulinemia in the adulthood is usually related to a secondary immunodeficiency. When a patient presents with the association of a hypogammaglobulinemia and a granulomatosis, an opportunistic infection must first be ruled out.

Safety and efficacy of rituximab treatment for vasculitis in hepatitis B virus-associated type II cryoglobulinemia: a case report.

Introduction: Systemic B-cell depletion and clinical remission of the systemic effects of cryoglobulins have already been achieved using rituximab in hepatitis C virus-positive immunocompetent patients. Conversely, to the best of our knowledge there are no reports in the literature regarding the use of rituximab in hepatitis B virus-associated cryoglobulinemia.

Case Presentation: We report here the case of a 60-year-old Caucasian man who presented with hepatitis B virus-associated type II cryoglobulinemia with severe multisystem disease, including membranoproliferative glomerulonephritis with acute renal failure.

[Delayed haemolytic transfusion reaction due to anti-JK1 antibody].

Introduction: The sensitivity of the detection of irregular antibodies (DIA) is one of the fundamental basis of transfusion safety. The production of alloantibodies is the first cause of adverse events following transfusion.

Case Report: We report a 77-year-old woman who was transfused and presented with a delayed haemolytic anemia due to anti-JK1 alloimmunization.

[Ischemic stroke as the first manifestation of Wegener's granulomatosis].

The most characteristic clinical manifestations of Wegener's granulomatosis are upper and lower respiratory tract and renal involvement. The central nervous system manifestations are uncommon and occur usually late in the course of the disease. We report a 48-year-old man who presented with an ischemic stroke as the first manifestation of Wegener's granulomatosis.

[Thyrotoxic hypokaliemic periodic paralysis revealing Graves' disease in a male Caucasian].

Objectives: Hypokaliemic thyrotoxic periodic paralysis (TPP) is an uncommon complication of hyperthyroidism. Mostly described among Asian patients, it is rare in other ethnic groups, in particular in Caucasian people.

Case Report: We present the case of a Caucasian male admitted to our unit after several paretic episodes.

[Etiology and prognosis of highly elevated C-reactive protein levels (≥500 mg/L): a retrospective study about 168 measures in a series of 113 patients].

Purpose: The C-reactive protein (CRP) is a useful inflammatory marker with a rapid kinetics during the inflammatory process. The objective of this study was to determine the etiology and prognosis of extremely elevated CRP values greater or equal to 500 mg/L.

Methods: We performed an exhaustive retrospective study from January 2004 to July 2009, in a general hospital, of all patients with a CRP value above 500 mg/L, admitted in all clinical departments.

European School of Internal Medicine (ESIM) in Brighton: experiences and reflections.

The European School of Internal Medicine (ESIM) is a meeting aimed at young physicians training in internal medicine throughout Europe. Since 1998 the school has been held annually, and in this report we reflect on our experiences during the recent school in Brighton in July 2010. The school combined a broad mix of lectures, workshops and case presentations covering a variety of rare diseases, and both faculty and residents participated with noticeable enthusiasm, making this a special experience for all of us.