An unusual case of autoimmune hemolytic anemia-thinking outside the box

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 (\u3c8 mg/dl), the peripheral smear revealed rare polychromasia and spherocytes, a positive direct antiglobulin test (DAT: BS +, IgG +, C3d -) with presence of warm autoantibodies. All infectious disease testing was negative. Anti nuclear antibodies were not identified. The 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 a total of 13 units 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, Transfusion Medicine was consulted and advised only transfusion of phenotypically matched pack red blood cells for significantly symptomatic anemia or hemodynamic instability, as the patient continued a downtrend in the hemoglobin despite continued transfusion support. Decadron was added to his therapy, as well as IVIG that needed to be discontinued due to infusion related shortness of breath and chest pain. Another dose of Rituxan along with vincristine and cyclophosphamide were also given. Despite these efforts, the patient’s hemoglobin continued to drop despite an additional 9 RBC units transfused at our institution, reaching a low of 2.1 g/dl. In addition, the patient develop 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 (PSAE) was performed as an alternative approach. The lower to mid portions of the spleen were embolized individually with 500-700 micrometer Embosphere microspheres followed with a gelfoam/contrast slurry mixed with gentamicin. 30% residual viable spleen was seen on the final DSA. 6 hours post procedure, a dramatic increase in hemoglobin was observed without any additional transfusion support. The patient suffered severe left upper quadrant abdominal pain that was controlled medically. After close observation the patient was discharged home 2 days later. At home the patient was doing well symptomatically with the exception of persistent left upper quadrant pain. He had no clinical or laboratory signs of recurrent hemolysis. Hemoglobin was 12.1 g/dl. On April 2018, due to persistent left upper quadrant pain, the patient was scheduled for a laparoscopic splenectomy that was performed without complications. During this hospitalization a type and screen was performed in preparation for surgery. No warm autoantibodies were detected; however, additional new 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, continued with a decreasing trend in hemoglobin that was accompanied by increasing lactate levels with no elevated troponin or signs of cardiac compromise. PSAE 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.https://scholarlycommons.henryford.com/merf2019caserpt/1084/thumbnail.jp