ABSTRACT
BACKGROUND: Hemophagocytic syndrome (HPS) is a rare but potentially fatal disease. The diagnosis is based on the combination of clinical and biological signs, requiring histological or cytological research hemophagocytosis and exhaustive etiological investigation. AIM: To report four cases of the HPS in an internal medicine department. CASES REPORT: We report four cases of HPS associated with Still's disease in two cases, with Sjogren syndrome in one case and a severe sepsis in one case. There are three women and one man. The mean age was 34.75 years, with extremes of 21 to 50 years. In all cases, patients were hospitalized for fever of unknown origin. The etiologic research of this fever remained negative. In all cases, patients validated criteria of HPS confirmed by cytological studies. The treatment was based on corticosteroids in all cases and immunosuppressant in one case. The evolution was favorable in two cases and fatal in two cases. CONCLUSION: HPS is a serious, often overlooked, may affect the prognosis and complicating various infectious, neoplastic or autoimmune diseases.
Subject(s)
Lymphohistiocytosis, Hemophagocytic/diagnosis , Adrenal Cortex Hormones/therapeutic use , Adult , Female , Fever of Unknown Origin/etiology , Humans , Immunosuppressive Agents/therapeutic use , Lymphohistiocytosis, Hemophagocytic/drug therapy , Male , Middle AgedABSTRACT
BACKGROUND: Arterial involvement is rarely described in Behcet disease BD and it is associated with poor prognosis. AIM: We report our experience with a rare and interesting subset of Behcet disease patients with arterial involvement: thrombosis and aneurysm formation types of angio-BD. METHODS: From 1994 to 2008, seven cases of arterial BD with 3 aneurysm formation and 4 thrombosis were found amongst BD patients in the department of internal medicine of Habib Thameur Hospital. RESULTS: Combination of venous and arterial manifestations occurred in 4 patients. All patients were male and the mean age when arterial involvement manifested was diagnosed was about 39.3. The artery most often affected is the aorta followed by the pulmonary arteries. Brain infraction occurred in about 4 cases. Pericarditis and neurological symptoms were more frequently noted in patients with arterial BD than others without vasculo-Behcet disease. All patients underwent corticosteroids and immunosuppressive therapy. Two patients were successfully operated for aneurysms. All patients were alive after a mean of 7.8 years follow-up. CONCLUSION: Arterial involvement is more frequently noted in vasculo-Behcet than venous thrombosis and it's associated with a high morbidity and mortality rate.
Subject(s)
Aneurysm/etiology , Behcet Syndrome/complications , Thrombosis/etiology , Adrenal Cortex Hormones/therapeutic use , Adult , Aneurysm/surgery , Aortic Aneurysm/etiology , Arteritis/etiology , Behcet Syndrome/drug therapy , Brain Infarction/etiology , Follow-Up Studies , Humans , Immunosuppressive Agents/therapeutic use , Male , Prognosis , Pulmonary Artery , Retrospective Studies , Time FactorsSubject(s)
Granulomatosis with Polyangiitis , Adult , Brain Diseases/etiology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/diagnostic imaging , Humans , Hypertension/etiology , Intracranial Hypertension/etiology , Magnetic Resonance Imaging , Male , Splenic Infarction/etiology , Splenomegaly/etiology , Thrombophlebitis/etiology , Time Factors , Tomography, X-Ray ComputedABSTRACT
BACKGROUND: Catastrophic antiphospholipid syndrome is a distinctly rare dramatic condition characterized by widespread thrombosis of small vessels. Early diagnosis and aggressive therapies are essential in this condition because of its extremely high mortality rate. Therapeutic management include heparine, high dose steroids, cyclophosphamide, plasma exchange, intravenous immunoglobuline, however a number of patients are refractory to treatment. AIM: We review and discuss alternative and emerging treatment options by rituximab for patients who fail or cannot tolerate conventional therapy. CASE-REPORT: A 36-year-old female with a two mounths history of dyspnea, palpitation and chest pain was admitted. Physical examination upon admission revealed a fever, ischemic digital necrosis, scleroderma of the hands and beaking of the nose. Laboratory tests showed normal level of liver enzymes, elevation of creatinine level, lymphopenia, haemolytic anaemia with negative Coombs tests, low platelet count, prolonged partial thromboplastin time. The D-Dimer value was 158 ng/ml. Urinalysis revealed a proteinuria. Antinuclear antibody tests and lupus anticoagulant were strongly positive. Echocardiography revealed severe pulmonary hypertension and pericarditis. There was no pulmonary embolism on thoracic angio tomodensitometry. The diagnosis of catastrophic antiphospholipid antibody syndrome associated with systemic lupus and scleroderma was established. She was treated with anticoagulants, corticotherapy, one pulse of intravenous cyclophosphamide, 2 doses of intravenous immunoglobuline and 5 sessions of plasmapheresis. Because of lack of response 2 doses of 375 mg weekly rituximab i.v. were added but she developed pulmonary embolism, alveolar haemorrhage and she died. CONCLUSION: Effectiveness of Rituximab for the CSAPL should be demonstrated by further studies.
