Haemophagocytic syndrome associated with Hodgkin lymphoma and Pneumocystis jiroveci pneumonitis

A 74-year-old, previously healthy, male presented with a 4-week history of general weakness, high fever, dyspnoea and sweats. Baseline laboratory investigations showed Hb 118 g/l, WBC 16.5 × 109/l, platelet count 381 × 109/l and C-reactive protein elevated to 132 mg/l. Computed tomography (CT) imaging showed considerable thoracic and abdominal lymphadenopathy. Lymphoma was suspected but histological examination of an axillary lymph node failed to confirm a specific diagnosis. The patient showed remarkable improvement on oral corticosteroids but symptoms recurred 6 weeks later. Chest X-ray and CT showed interstitial pneumonitis and bronchoscopy with bronchoalveolar lavage confirmed Pneumocystis jiroveci infection. Despite treatment, pulmonary function deteriorated and the patient was admitted to the intensive care unit. Peripheral lymphadenopathy progressed and an inguinal lymph node was removed for further histological examination. Within a few days, the patient developed pancytopenia with Hb 97 g/l, WBC 1.8 × 109/l and platelet count 32 × 109/l. A bone marrow aspirate was hypercellular with massive haemophagocytosis of all blood cells types (Figures). Hyperferritinaemia (ferritin 21821 μg/l, normal range: 20–300) and hypofibrinogenaemia (fibrinogen < 0.5 g/l, normal range: 2.0–4.3) also occurred. Treatment with etoposide, corticosteroids and ciclosporin was started. In the meantime, histological examination of the inguinal lymph node showed classical Hodgkin lymphoma, type nodular sclerosis. The patient initially improved on treatment but after several days worsened again and subsequently succumbed. Haemophagocytic syndrome has been previously associated with both Hodgkin lymphoma and P. jiroveci infection. In our patient, haemophagocytic syndrome associated with both these underlying conditions resulted in a severe haemophagocytosis with a fatal outcome.