INTRODUCTION: Evans syndrome is a rare condition in the adult population and is defined by the presence of autoimmune hemolytic anemia with thrombocytopenia and occasionally neutropenia We report the case of an individual with catastrophic hemolyses in a 24-year-old man with Evan's syndrome which was successfully treated with the anti-CD 20 monoclonal antibody rituximab, after failure of conventional therapies.
CASE PRESENTATION: A 24 year-old man with Evans Syndrome presented with a 3-day history of fever, diaphoresis, and cough. The past medical history was significant for long term anemia, thrombocytopenia and splenectomy. On physical examination he was a thin man in mild respiratory distress. Temperature was 38.9°C, blood pressure 117/58 mmHg, pulse rate of 132 /min, and respiratory rate of 27 /min. Oxygen saturation was 95% while receiving 4 liters/ min per nasal cannula. There was Eugophony and wheezing over the right lower lung field. The remainder of the physical examination was unremarkable. Laboratory workup revealed hemoglobin of 8.2 g/dl, white blood cell count of 21,100 /ml (59% neutrophils, 30% band forms, 6% lymphocytes, and 5% monocytes), and platelets of 32,000/mm 3. Peripheral smear showed ovalocytes, spherocytes, anysocitosis and poikilocytosis. Reticulocytes were elevated (9.9%), liver panel was normal except for an increase in total bilirubin and indirect bilirubin (2.6 mg/dl); coombs test, direct and indirect, were both positive. Hemoglobin electrophoresis, HIV, HEP C, and B serologies, complement and ANA were all normal. A chest radiograph showed patchy opacifications in the right middle lung zone with air bronchograms. The patient was admitted to the ICU with a community-acquired pneumonia and acute autoimmune hemolytic anemia. Antibiotic treatment was initiated with ceftriaxone and azitromycin. He received bronchodilators, supplemental oxygen, methylprenilosone 125 mg IV every 6 hours, IVIG (600 MG /KG) QD. Four hours later his hemoglobin decreased to 4 g/dl. He required transfusions over the following 24 hours. On Hospital day 2 dyspnea worsened and he required mechanical ventilation. Hemoglobin failed to raise over 5 g/dl despite transfusion of 18 units of PRBC's, indirect bilirubin increased to 9.0 mg/dl and the patient became jaundiced. Due to lack of response to IVIG and methylprednisolone, a trial of 375 mg of rituximab were given. There was significant hematological improvement after a single dose, (Hg of 10 mg/dl and platelets to 165,000 /mm3). Patient did not required furthered transfusions. We were able to extubate patient by day 3. The patient was discharged home in stable condition after 10 days from admission.
DISCUSSIONS: Evan syndrome is characterized by a chronic course with frequent exacerbations and remissions. Conventional treatment includes corticosteroid, splenectomy, IVIG or immunosuppressive agents such as cyclosporine, mycophenolate mofetil, vincristine, and danazole. More recently some cases have been treated with rituximab. Rituximab has gained widespread acceptance in the management of B-Cell malignancies. Rituximab is a monoclonal antibody against CD-20. Binding of CD-20 receptor by rituximab depletes B Cells in the circulation and in the lymphoid tissue. In this case rituximab was able to stop the production of auto antibodies allowing to stop the cycle of autoimmune hemolyses restoring immune tolerance.
CONCLUSION: In very severe cases of autoimmune hemolytic anemia refractory to conventional therapies rituximab may be an effective option.
DISCLOSURE: Shigeki Saito, No Financial Disclosure Information; No Product/Research Disclosure Information