Presenting Author

Shreel H. Patel,MD

Presentation Type

Oral Presentation

Discipline Track

Patient Care

Internal Medicine

Abstract Type

Case Report

Abstract

Background: Autoimmune hemolytic anemia is caused by autoantibodies that react with self red blood cells and cause them to be destroyed. Warm AIHA, due to antibodies that are active at body temperature, is the most common type of the AIHA. It is mostly caused by the underlying disease like infections, autoimmune disorders, lymphoproliferative disorders, immunodeficiency or physiologic state like pregnancy. Rarely it happens that there is no underlying cause. Therefore, here we present a case of idiopathic autoimmune hemolytic anemia without any underlying cause.

Case Discussion: A 77 year old lady with known history of the diabetes mellitus type 2, essential hypertension and dyslipidemia presented to the emergency department complaining of generalized body weakness and recently started noticing yellowish-red urine and yellowish discoloration of the skin from last 1 week. On asking her further she denies any recent change in medications which were metformin and losartan, no recent sick contacts or any infections, any weight loss or chest pain or abdominal pain, near syncope or loss of consciousness, any change in bladder or bowel habits other than the change in the color of urine. Denies any positive family history or prior blood transfusions.

On admission her labs showed Hb of 5 g/dl, total bilirubin of 8.9 mg/dl, conjugated bilirubin was 0.43 mg/dl, LDH was 1572 u/L, MCV was 107.5 fl, haptoglobin less than 10 mg/dl, total reticulocyte count was 20.3 %. Urine only showed few RBC and 4+ blood. Her significant vitals were only tachycardia with the heart rate ranging from 110-130s. Further investigations were done including the CT abdomen pelvis with oral and IV contrast which was negative for inflammation or any tumor and CXR and EKG were not significant. Blood cultures and urine culture also showed no growth after 48 hours. Other infections like Hepatitis and HIV were ruled out.Direct antiglobulin test(Coombs test) came out positive. Her physical exam was only positive for sinus tachycardia and severe yellowish discoloration of skin and conjunctiva. Therefore, everything looked consistent with autoimmune hemolytic anemia on basis of the positive DAT test, high LDH, low haptoglobin, low HB, high indirect bilirubin and the peripheral smear showed polychromasia and nucleated RBC. The patient was started on the prednisone 1mg/kg/day and emergency release of O neg blood was done for transfusion to keep Hb above 7 g/dl. She was eventually started on the rituximab 375 mg per square meter once weekly for 4 weeks. She felt better after the prednisone and was discharged home with follow up with the Hematologist for rituximab.

Conclusion: The main learning point is that even without underlying factors the patient can have the autoimmune hemolytic anemia. The main treatment for this kind of scenario is oral or intravenous glucocorticoids and rapid blood transfusion. The second line therapy is Rituximab and other immunosuppressive agents and chemotherapy agents.

Peripheral Blood Smear (672 kB)
The peripheral blood smear showing microspherocytes and polychromasia suggestive of the hemolysis

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Too Yellow: An Idiopathic Case Of Autoimmune Hemolytic Anemia

Background: Autoimmune hemolytic anemia is caused by autoantibodies that react with self red blood cells and cause them to be destroyed. Warm AIHA, due to antibodies that are active at body temperature, is the most common type of the AIHA. It is mostly caused by the underlying disease like infections, autoimmune disorders, lymphoproliferative disorders, immunodeficiency or physiologic state like pregnancy. Rarely it happens that there is no underlying cause. Therefore, here we present a case of idiopathic autoimmune hemolytic anemia without any underlying cause.

Case Discussion: A 77 year old lady with known history of the diabetes mellitus type 2, essential hypertension and dyslipidemia presented to the emergency department complaining of generalized body weakness and recently started noticing yellowish-red urine and yellowish discoloration of the skin from last 1 week. On asking her further she denies any recent change in medications which were metformin and losartan, no recent sick contacts or any infections, any weight loss or chest pain or abdominal pain, near syncope or loss of consciousness, any change in bladder or bowel habits other than the change in the color of urine. Denies any positive family history or prior blood transfusions.

On admission her labs showed Hb of 5 g/dl, total bilirubin of 8.9 mg/dl, conjugated bilirubin was 0.43 mg/dl, LDH was 1572 u/L, MCV was 107.5 fl, haptoglobin less than 10 mg/dl, total reticulocyte count was 20.3 %. Urine only showed few RBC and 4+ blood. Her significant vitals were only tachycardia with the heart rate ranging from 110-130s. Further investigations were done including the CT abdomen pelvis with oral and IV contrast which was negative for inflammation or any tumor and CXR and EKG were not significant. Blood cultures and urine culture also showed no growth after 48 hours. Other infections like Hepatitis and HIV were ruled out.Direct antiglobulin test(Coombs test) came out positive. Her physical exam was only positive for sinus tachycardia and severe yellowish discoloration of skin and conjunctiva. Therefore, everything looked consistent with autoimmune hemolytic anemia on basis of the positive DAT test, high LDH, low haptoglobin, low HB, high indirect bilirubin and the peripheral smear showed polychromasia and nucleated RBC. The patient was started on the prednisone 1mg/kg/day and emergency release of O neg blood was done for transfusion to keep Hb above 7 g/dl. She was eventually started on the rituximab 375 mg per square meter once weekly for 4 weeks. She felt better after the prednisone and was discharged home with follow up with the Hematologist for rituximab.

Conclusion: The main learning point is that even without underlying factors the patient can have the autoimmune hemolytic anemia. The main treatment for this kind of scenario is oral or intravenous glucocorticoids and rapid blood transfusion. The second line therapy is Rituximab and other immunosuppressive agents and chemotherapy agents.

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