Fever, splenomegaly and lymphopenia in sarcoidosis

To cite: Ravaglia C, Gurioli C, Casoni GL, et al. Thorax 2013;68:496–497. CLINICAL PRESENTATION A 42-year-old woman was referred to our department with a 5 months history of intermittent fever and fatigue. Her past medical history included a consolidated diagnosis of sarcoidosis (obtained 7 years before with a sub-carinal lymph node biopsy) and in the previous years she had been treated with steroids, hydroxychloroquine, methotrexate and azathioprine with persistent and progressive enlargement of mediastino-hilar adenopathies and bilateral nodular infiltrates. Her symptoms progressed despite a course of antibiotics prescribed for presumed community-acquired pneumonia and a course of corticosteroids. The patient lived in Italy and there was no history of travels or other additional risk factors for infections. At the time of admission, the patient had a temperature of 38.5°C and the physical examination was only notable for hepatomegaly and splenomegaly. A white blood cell count showed lymphopenia (0.39×10^9/l, CD4+ 0.22×10^9/l) and anaemia (Hb 10.4 g/dl, MCV 78.7 fl). CT images of the thorax revealed bilateral nodules of variable sizes predominantly in the upper lung fields and a positron emission tomography showed several areas of increased metabolism in the liver, spleen, lymph nodes (above and under the diaphragm), D3 vertebra and bilateral nodular infiltrates (figure 1). Fibre-optic bronchoscopy was normal and all investigations for infections (blood, bronchoalveolar lavage, urine and serological tests) were negative. ECG was normal. A bone marrow biopsy was performed: the perivascular region was characterised by a concrete number of polyclonal mature plasma cells (for κ and λ light chains of immunoglobulins) and a diffuse interstitial infiltration of foamy macrophages with intracellular inclusions was noted as showed in figure 2.