Abstract

An 86-year-old woman was admitted for anorexia and weight loss. Her medical history was significant for psoriasis vulgaris and she was under no medication. On admission, clinical examination found fever at 38.4 8C with recurrent peaks, dyspnea, cough, diffuse ronchi, saturation at 90% with oxygen therapy (1 L/min), and confusion with neither neurological focus signs nor symptoms suggesting meningitis. Serum C reactive protein was at 174 mg/L. Haemogram showed thrombopenia at 86 G/L, haemoglobin level at 11.8 g/dl and leucopenia (2.6 G/L) with lymphopenia (0.36 G/L). Liver tests showed a rise in alkaline phosphates at 230 UI/L (N < 105 UI/L), gamma GT at 133 UI/L (N < 38 UI/L), ASAT at 126 UI/L (N < 35 UI/L), and ALAT at 73 UI/L (N < 35 UI/L). Repeated blood cultures were performed that remained sterile. Chest radiography (in bed) was not contributive. The hypothesis of haemophagocytic syndrome (HS) was suspected mainly due to the association of bicytopenia and fever. Complementary plasmatic dosages of ferritine was at 1760 mg/L (N = 12–165 mg/L) and triglycerides at 1.56 mmol/L (N = 0.5– 1.5 mmol/L). Treatment based on ceftriaxone with spiramycine was initiated but remained ineffective for clinical symptoms and biological abnormalities. Bone marrow biopsy with myelogram found megacaryocytes in normal quantity, macrophages in excess, and images of red cell and platelet phagocytosis suggesting HS. A thoracic and abdominal CT scan (Fig. 1) showed diffuse interstitial pulmonary syndrome compatible with lymphangitic or

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