Abstract: |
A 63-year-old man presented to the hospital with generalised weakness, fatigue and a 22 kg weight loss 4?months after being diagnosed with sarcoidosis on a mediastinal lymph node biopsy, with minimal improvement in symptoms on prednisone and methotrexate therapy. On arrival, he was found to have a haemoglobin of 57?g/L and platelet count of 82×10/L. Further work-up revealed six of eight diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH): fever >38.9°C, splenomegaly, cytopaenia, hypertriglyceridaemia, haemophagocytosis and elevated ferritin >31?000 ng/mL. He was also found to have Epstein-Barr viraemia with greater than 17?000 copies. Bone marrow biopsy showed the presence of haemophagocytic histiocytes and evidence of classic Hodgkin's lymphoma. He was started on HLH-94 protocol. Later treatment was switched to lymphoma-directed therapy and he finished six cycles of A+AVD (brentuximab vedotin, doxorubicin, vinblastine and dacarbazine) with end-of-treatment positron emission tomography/CT and bone marrow negative for lymphoma. |