Therapeutic Plasma Exchange (TPE, plasmapheresis) for Treatment of Refractory Autoimmune Hemolytic Anemia (AIHA)

Abstract

Abstract Management strategies for AIHA include corticosteroids, intravenous immunoglobulin, immunosuppressive agents, cytotoxic agents, monoclonal antibodies (Rituximab) and in severe and or refractory cases splenectomy. Our patient is a 49 year-old Jamaican man with past medical history of HIV infection and recently diagnosed stage IV Hodgkin lymphoma with marrow and organ involvement who presented to the ER with severe fatigue, chills and high-grade fever. CBC testing revealed hemoglobin of 3.2gm/dl with a normal leukocyte and platelet count. Further testing revealed strongly positive direct coombs test, undetectable haptoglobin, elevated unconjugated bilirubin, elevated LDH and plasma free hemoglobin confirming the diagnosis of AIHA. Laboratory parameters for disseminated intravascular coagulation (DIC) were negative. Infection was ruled out with cultures and radiological studies. Severe ongoing hemolysis led to an acute change in mental status, hypotension and acute renal failure requiring the patient to be transferred to the ICU for hemolytic shock. Patient failed to respond to conventional treatments including high dose steroids and IVIG. A single dose of rituximab was also tried without any clinical improvement. Patient’s clinical status deteriorated rapidly and within 48 hours, he developed multi-organ failure requiring continuous venovenous hemodialysis (CVVHD), hemodynamic support with pressors, and aggressive hematological support with multiple blood transfusions. At this point a decision was made to initiate TPE. Within hours of the first TPE, the patient’s clinical status improved. His mental status improved dramatically from a stupor to a fully conscious state. Daily TPE was continued for four days, which resulted in stabilization of his hematocrit, discontinuation of pressors as well as CVVHD. Laboratory parameters of hemolysis normalized. He was eventually able to receive definitive chemotherapy for Hodgkin lymphoma. He is currently three months status post last TPE and undergoing the third cycle of chemotherapy with no recurrence of AIHA. Our experience indicates that TPE can be an effective and rapid treatment modality for patients with severe AIHA secondary to IgG antibodies refractory to conventional means of therapy.