[Acquired hemophilia as the presenting manifestation of neoplasia: diagnostic workup and monitoring]. / Hémophilie A acquise précédant l'apparition d'un cancer: quelles explorations et quelle surveillance?

Acquired haemophilia is a rare disorder caused by the development of autoantibody to factor VIII. It is sometimes associated with malignancies, and usually appears during disease course. In rare instances, acquired haemophilia is the presenting manifestation of a malignant disease. We report a 76-year-old man, who presented with spontaneous haematomas of his four limbs. A factor VIII inhibitor was found and the patient diagnosed with acquired haemophilia. Initial etiologic diagnostic workup including a thoracic and abdominal computed tomographic scan was negative. Factor VIII inhibitor disappeared on corticosteroids and factor VIII level normalized. Seven months later, the patient died from a multimetastatic cancer. About 15% of acquired haemophilia are associated with malignant disease (malignant lymphoma or solid neoplasia). Although rare, the development of a factor VIII inhibitor few months before the diagnosis of the malignant disease raised the issue of the appropriate initial investigations and further monitoring to recommend these patients. We propose a regular clinical monitoring and a thoracic and abdominal computed tomographic scan at six-month follow-up to screen for malignant disease.

[Pneumococcal purulent pericarditis]. / Péricardite purulente à pneumocoque.

INTRODUCTION: Purulent pneumoccocal pericarditis are extremely rare since the introduction of antibiotics. EXEGESIS: A 59-year-old woman presented to the emergency room with a seven-day history of dyspnea and fever. No signs of heart failure or cardiac friction rub were evidenced. Laboratory tests disclosed elevated acute phase reactants and elevated white blood cells with a high neutrophil count. Chest radiograph showed cardiomegaly and a bilateral pleural effusion. Chest-computed tomography confirmed the pleural effusion and evidenced a large pericardial effusion. Streptoccocus pneumoniae grew up form pericardial fluid and blood cultures. In addition to the pericardial drainage, the patient received intravenous amoxicillin therapy. Outcome was favourable. There was no evidence of immunodeficiency. CONCLUSION: Although exceptional, diagnosis of purulent pneumococcal pericarditis should not be missed as it may compromise vital prognosis. Therapy should combine pericardial drainage and antibiotics.

[Nonbacterial thrombotic endocarditis and gastric carcinoma]. / Endocardite marastique et adénocarcinome gastrique.

We report a 74-year-old woman with acute heart failure and recurrent ischemic strokes as the presenting features of a nonbacterial thrombotic endocarditis complicating a gastric adenocarcinoma. The treatment only allowed a few months remission. Diagnosis of nonbacterial thrombotic endocarditis is rarely obtained while the patient is alive. Coagulation abnormalities due to the tumoral process are responsible of the valvular thrombotic process. Anticoagulation with heparin is recommended. Valvular surgery remains controversial.

[Acquired von Willebrand syndrome: a case series of nine patients and literature review]. / Syndrome de Willebrand acquis: étude d'une série de neuf patients et revue de la littérature.

PURPOSE: Whereas von Willebrand disease is the most common constitutional bleeding disorder, acquired von Willebrand syndrome is rare. METHODS: Retrospective, monocentric descriptive study of consecutive cases of acquired von Willebrand syndrome diagnosed between 2000 and 2012. Diagnostic criteria included: absence of a past history of mucocutaneous bleeding, with low plasma levels of factor VIII (FVIII) and von Willebrand factor (VWF), ristocetine cofactor activity (RCo) and antigen (Ag). RESULTS: Nine men were diagnosed with von Willebrand syndrome. Six of them presented with recent mucocutaneous bleeding. In eight cases, the biological phenotype was a type 2 von Willebrand disease, with decreased VWF:RCo/VWF:Ag ratio. A lymphoproliferative disease with circulating paraprotein was identified in all patients, including one chronic lymphoid leukemia, three Waldenström and one marginal zone lymphomas, four monoclonal gammapathies of unknown significance. Screening for an anti-VWF inhibitor was negative. Symptomatic treatment using infusion of VWF concentrates was administrated in the presence of severe mucocutaneaous bleeding. Five patients received intravenous immunoglobulins with a good response only in patients with G isotype paraprotein. A chemotherapy was initiated if indicated for the underlying disorder. Three of the four patients who achieved remission of the associated lymphoma had a subsequent improvement of plasma VWF levels, while all other patients remained deficient. CONCLUSION: Acquired von Willebrand syndrome is a rare but potentially serious disease. The diagnostic should be suspected in adults with unusual mucocutaneous bleeding, with or without prolonged partial thromboplastin time (PTT), and confirmed with a decreased plasma level of VWF (Ag and RCo). An associated haematological, neoplastic or cardiac valvular disease must be searched.

[Hyponatremia: from physiopathology to practice]. / Hyponatrémie : de la physiopathologie à la pratique.

Hypotonic hyponatremia is the most common electrolyte abnormality encountered in hospitalized patients. It is often asymptomatic but associated with increased mortality and morbidity. Prompt recognition of the underlying cause using a systematic physiology based approach and careful evaluation the chronicity of the hyponatremia is mandatory for an optimal management. One should first document hypotonicity, and then assess the renal response to hypotonicity to exclude water intoxication, and the extracellular volumes. The further step will identify hyponatremia due to volemic stimulation of vasopressin associated to extracellular dehydration (corrected by isotonic saline infusion) or to oedematous states. After exclusion of hypocorticism and hypothyroidism, one would conclude to inappropriate secretion of antidiuretic hormone whose etiology would have to be established. The use of hypertonic saline solutions should be restricted to the treatment of acute and severe hyponatremia with evidence of brain damage. Chronic hyponatremia should be correct slowly to avoid the risk of osmotic demyelination syndrome. Water restriction is commonly recommended in inappropriate secretion of antidiuretic hormone or in hypervolemia with a questionable effectiveness. The recent development of vasopressin receptor antagonists (vaptans) will modify our therapeutic approaches. Yet, further studies are needed to document their additional impact on morbidity and mortality.

Unusual acute crystal-induced hip arthritis: hydroxyapatite deposition of the round ligament.

Hydroxyapatite (HA) crystal deposition disease is a common cause of acute inflammatory articular or periarticular pain. Here we report a patient with an acute hip arthritis due to HA crystal deposition of the round ligament. Therefore, HA crystal deposition in periarticular tendons and ligaments of the hip should be added to the differential diagnosis of an acute hip arthritis. Computed tomographic scan may be useful when standard radiographs do not evidence the calcification.