Hodgkin’s lymphoma with HLH and complete remission with brentuximab-based therapy

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 82×109/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.

An Interesting Case of Disseminated Histoplasmosis in a Patient With Hemophagocytic Lymphohistiocytosis

“…Both infection and genetic predispositions may contribute to disease etiology, but its pathophysiology is poorly understood. Preexisting autoimmune and rheumatologic diseases in adults are reported in cases of HLH in the literature [23][24][25][26][27]. It can also be associated with malignancies like Hodgkin lymphoma [28].…”

An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma

“…Treatment of secondary HLH must be initiated early and aims to address the underlying precipitating disorder, with the most commonly used HLH protocol relying on etoposide, dexamethasone, and hematopoietic stem cell transplantation as mainstays of therapy [ 32 ]. For HLH secondary to Hodgkin lymphoma, brentuximab therapy has been reported to be successful [ 33 , 34 ].…”