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V Génin, A Enfrein, M Lecouffe-Desprets, P Gallas, C Bossard, A Moreau, C Ansquer, M Hamidou, C Agard, A Néel, Hot lungs, bitter cherry: intravascular lymphoma, QJM: An International Journal of Medicine, Volume 111, Issue 1, January 2018, Pages 53–54, https://doi.org/10.1093/qjmed/hcx224
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Case presentation
A 63-year-old Caucasian woman had a 6-month history of exertional dyspnea. Her past medical history was significant for primary tuberculosis infection during childhood. She subsequently developed unintentional 10-kg weight loss, fever and night sweats and was referred to us.
Physical examination showed a mild hepatomegaly and splenomegaly. Oxygen saturation, cardio-respiratory, lymph nodes, neurologic and skin examinations were normal. Laboratory results revealed microcytic anemia (hemoglobin 7.6 g/dl, mean corpuscular volume 75 fl) with systemic inflammation (C-reactive protein 126 mg/l), thrombocytopenia (95 g/l) and features of macrophage activation syndrome (MAS) including relative fibrinopenia (3.3 g/l), hypertriglyceridemia (4.25 g/l), hyperferritinemia (978 ng/ml) and elevated lactate dehydrogenase (676 IU/l). Bone marrow biopsy showed hemophagocytosis, without malignant cells or granuloma. Blood cultures, blood and bone marrow polymerase chain reaction for cytomegalovirus, Epstein–Barr virus, human herpesvirus 8, toxoplasma and leishmania were negative. Direct examination of bone marrow aspirate and gastric lavage revealed no acid-fast bacilli. Computed tomography (CT) scan revealed hepatomegaly and splenomegaly, without lymph nodes or pulmonary abnormality. At this stage, the considered underlying causes of MAS were mycobacterial infection and lymphoma. Anti-tuberculosis treatment trial and diagnostic splenectomy were discussed.
