Idiopathic Autoimmune Hemolytic Anemia Presenting as Massive Splenomegaly in an Elderly Male Which Responded to Steroid Therapy

Abstract

Autoimmune hemolytic anemia is uncommon. The estimated overall (not age-adjusted) annual incidence is about 1 case per 100,000 populations; after age 60 years, the annual incidence reaches 10 per 100,000. The disorder can occur at any age, but most patients are older than 40 years. About 65% of patients with primary autoimmune hemolytic anemia are women, and almost all cases that complicate systemic lupus erythematosus occur in women. A 65 years old male presented with generalized weakness, breathlessness on exertion, swelling of lower limbs and pain abdomen of 6 months duration. He was previously admitted elsewhere on several occasions (within past 3-4 months) with similar complaints, and had received multiple blood transfusions. On clinical examination, patient had pallor and bilateral pitting pedal edema. Abdominal examination revealed massive Splenomegaly (12 cm below left costal margin), moderate Hepatomegaly. Investigations revealed Hb% of 8.6 g/dl, Platelet count = 1 lakh/cmm, ESR = 120 mm, retic count -2.2 %. Peripheral smear showed evidence of hemolysis. Serum LDH was high, Serum bilirubin predominantly indirect hemoglobin= 2, S. Haptoglobin below 6.63. These findings suggested hemolysis as a cause of his anemia and splenomegaly. Further evaluation was done to find out the cause of hemolysis in this elderly male. Hb Quantification using HPLC was normal. Serum G6PD activity was normal. ANA was negative. Hams test was negative. Direct & Indirect Coomb's tests were positive. Bone marrow examination showed erythroid hyperplasia. CT Abdomen showed hepatosplenomegaly. Upper GI endoscopy and colonoscopy were normal. Based on these findings a diagnosis of warm antibody type auto immune haemolytic anaemia (AIHA), probably of idiopathic type, was made and patient was started on steroid therapy. After 2 weeks, repeat haemoglobin was 12.8 gm%, WBC count was 7020/cumm reflecting response to steroid therapy. Severe AIHA can be a medical emergency. Red cell transfusion poses a special problem as the transfused cells are rapidly destroyed, but can be lifesaving and in the meantime steroids can exert their effect. This unique situation requires good liaison and understanding between the clinical unit and the serology lab. Thus it is very important to diagnose this condition and treat accordingly. Untreated, chronic auto immune hemolysis progresses to severe anaemia and associated complications. It is important to consider the diagnosis of autoimmune hemolysis in elderly males also, although this condition is usually seen in females. It is important to rule out malignancies and connective tissue disease in elderly presenting with autoimmune hemolysis.