Thrombotic Thrombocytopenic Purpura Associated With Hodgkin Lymphoma

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia with usually less than <5% ADAMTS13 (a disintegrin and metalloprotease with thrombospondin repeats) activity level. Hodgkin lymphoma (HL) comprises 10% of newly diagnosed lymphoma cases.

A 45 year old healthy African American male presented with diffuse abdominal pain with associated nausea and constipation. Labs showed decreased hemoglobin, platelets, and haptoglobin with elevated bilirubin, lactate dehydrogenase, and creatinine. ADAMTS13 level was <1%. Peripheral smear revealed schistocytes. CT imaging demonstrated multiple lesions of liver, pleura, and bone, as well as diffuse adenopathy, all later confirmed to be hypermetabolic by PET scan. Pleura biopsy suggested HL. TTP was diagnosed, prompting start of high dose prednisone and plasma exchange (PE). Labs normalized after 5 exchanges, allowing for outpatient steroid and PE tapers. A pleura biopsy was suspicious but not definitive for HL. Three weeks later, he was admitted for opioid-induced constipation. He was still on PE. Despite labs without evidence of TTP, his ADAMTS13 persisted at <1%. A definitive excisional cervical lymph node biopsy yielded classical nodular sclerosing HL. Patient started systemic chemotherapy with doxorubicin, bleomycin, vincblastine, and dacarbazine (ABVD). PE was discontinued 7 days later. At follow-up 3 weeks from cycle #1, ADAMTS13 was 55% without evidence of TTP.

A 2012 review notes 4 cases of microangiopathic hemolytic anemia associated with HL, proposing an underlying immune component to this uncommon entity. Though rare, management of TTP associated HL can be challenging due to underlying cytopenias delaying systemic chemotherapy and yet refractoriness of ADAMTS13 to correct, portending a high risk of TTP relapse, without chemotherapy. Supportive measures to stabilize TTP and prompt initiation of chemotherapy can be an effective approach.

No relevant conflicts of interest to declare.