An unusual case of autoimmune hemolytic anemia-thinking outside the box for management
Williams P, Lopez-Plaza I. An unusual case of autoimmune hemolytic anemia-thinking outside the box for management. Blood 2018; 132(Suppl 1):5075.
A 27 year old male with no significant past medical history presented to an outside hospital for chief complaints of nausea/vomiting, epigastric pain, and acute jaundice. Initial laboratory workup revealed a hemoglobin of 5.5 g/dl, normal WBC and platelet count, elevated direct bilirubin (10 mg/dl), elevated lactate dehydrogenase (969 IU/L), low haptoglobin (<8 mg/dl), a peripheral smear showing rare polychromasia and spherocytes, and positive direct antiglobulin test (DAT: BS +, IgG +, C3d-) with presence of warm autoantibodies. All infectious disease testing was negative. Initial CT abdomen showed a normal sized spleen. The patient was diagnosed with idiopathic warm autoimmune hemolytic anemia and was treated with pulsed steroids, one dose of Rituximab, and transfused with up to 13 units of packed red blood cells. The patient did not respond to medical treatment, and after one week, the patient was transferred to our institution for escalation of care. At our institution, the patient presented with similar symptoms. Due to lack of response to packed red blood cell transfusions, As the patient continued a downtrend in the hemoglobin despite continued transfusion support, Transfusion Medicine advised to only transfuse phenotypically matched, least incompatible, packed red blood cells for significantly symptomatic anemia or hemodynamic instability. High dose steroid were continued and IVIG, Cytoxan and Vincristine were added to his warm autoimmune hemolytic anemia (WAIHA) therapy. Despite these efforts, the patient's hemoglobin continued to decrease despite an additional 9 RBC units transfused at our institution, reaching a hemoglobin nadir of of 2.1 g/dL. In addition, the patient developed thrombocytopenia, NSTEMI, and left lower quadrant pain accompanied by bloody bowel movements. A repeat CT scan of the abdomen was performed, which revealed an enlarged spleen with a span of 16.5 cm in its maximum dimension. Due to concerns for splenic infarction and/or rupture, and since the patient was not a candidate for surgery, partial splenic artery embolization was performed. Thirty percent residual viable spleen remained. Within hours post embolization, a dramatic increase in hemoglobin was observed without any additional transfusion support. Shortly after splenic embolization the patient developed severe left upper quadrant abdominal pain with a perisplenic fluid measuring slightly greater than simple fluid attenuation on CT which was managed medically. After close observation the patient was discharged home 2 weeks after admission. At home the patient was doing well with the exception of persistent left upper quadrant pain. He had no clinical or laboratory signs of recurrent hemolysis. Three months post discharge, due to persistent left upper quadrant pain and presence of a splenic cyst, the patient underwent a laparoscopic splenectomy that was performed without complications. During this hospitalization, in preparation for surgery, compatibility testing showed no autoantibodies. However new clinically significant alloantibodies were identified. Hypersplenism and/or reticulocytopenia are infrequent complications of a warm autoimmune hemolytic anemia, constituting a life threatening complication that may not respond to standard therapy and may not qualify for surgical intervention. The above patient did not improve with any medical therapies and displayed a decreasing trend in hemoglobin that was accompanied by increasing lactate levels and NSTEMI. Splenic embolization achieved a dramatic response that was maintained through follow up. Due to recurrent pain from the embolization, the patient eventually received a splenectomy once he was stable. Four months after initial presentation, post infusion of 4 doses of Rituxan and steroid therapy, the autoantibodies were not detectable. However, the patient developed 3 alloantibodies post multiple transfusions.