[Hémophagocytic syndrome: a study of 11 patients in Tunisian population]

Hemophagocytic syndromes are mostly associated with underlying pathology, they can reveal: immunodeficiency, infections, hemopathies, cancers and auto-immune diseases. To investigate clinical, biological features, outcome characteristics and underlying pathology of Tunisian patients with hemophagocytic syndromes. A retrospective study of patients with hemophagocytic syndromes admitted in an internal medicine department in Tunis over the period 2009-2012. There were 11 patients included, 4 men and and 7 women. Mean age was 47,3years. Purpura was observed in 3 cases, hepatosplenomegaly in 6 cases and peripheral lymph nodes in 3 cases. Hemorrhagic complication was noted in 2 cases. There were an increase of inflammatory indices in all the cases. Eight patients had pancytopenia and 3 had bicytopenia. Six patients developed intravascular dissiminated coagulation. High levels of triglyceridemia was noted in 5 cases and an hyperferritinaemia in all cases. Cytological examination of bone marrow confirmed hemophagocytosis in ten cases. Hemophagocytosis was associated to infectious diseases in 6 cases, there were 2 cases of leishmaniasis, septicemia to E.Coli and staphylococcus aureus and lymph nodes tuberculosis with HIV. We also noted a case of lymphoma, myelodysplasic syndrome, rheumatoid polyarthritis, adult onset still's disease. In one patient, the origin of hemophagocytosis remained indefinite. Visceral leishmaniose were treated by Glucantime[registered sign], adult Still's disease by corticosteroids and méthrotrexate[registered sign], lymphoma by chemotherapy. In lymph nodes tuberculosis, there were good evolution. Six patients died. Hemophagocytic syndrome is a rare and life-threatening disease. Pejorative prognosis requires an early therapy with etiological treatment

[Chronic dissiminated intravascular coagulation caused by aortic dissection]

Disseminated intravascular coagulation [DIC] is a severe disease. It's can be caused by lost of pathology. We report the case of chronic aortic dissection discovered during the evaluation of disseminated intravascular coagulation [DIC]. This case is characterised by the severity of clinical presentation, challenging diagnosis and difficulty of therapeutic approach. Low dose of heparine may reduce the severity of this situation; but vital prognosis remains obscure. Aortic dissection is a rare but a severe cause of disseminated intravascular coagulation

[Renal amyloidosis during behcet's disease. study of one case]

Amyloidosis in Bethel's disease is rare and has a poor outcome. Report a new case. We report a case of a 38-year-old tunisian woman who presented with Behcet's disease and nephrotic syndrome. Renal biopsy showed amyloid deposition consistent with AA type. She had not any associated disease that might be an additional cause of secondary amyloidosis. She was treated by colchicine. After 3 years, proteinuria had disappeared and persisted negative 11 years after the diagnosis. A second renal biopsy showed the persistence of amyloidosis. As amyloidosis is one of the prognostic factors affecting survival in Behcet's disease, patients with this disease should be screened for amyloidosis. Administration of colchicine to these patients may be beneficial