Asian Hematology Research Journal

A Case Report on Hyperhaemolysis Syndrome in a Patient with Sickle cell Anaemia

Okeke Chinedu N *

Department of Hematology and Blood Transfusion, Bingham University Teaching Hospital, Jos, Nigeria.

Ode Charity I

Department of Hematology and Blood Transfusion, Bingham University Teaching Hospital, Jos, Nigeria.

Nwankwo Chizoba G

Department of Hematology, Nile University of Nigeria, Abuja, Nigeria.

Jatau Ezra D

Department of Hematology and Blood Transfusion, Jos University Teaching Hospital, Jos, Nigeria.

*Author to whom correspondence should be addressed.

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Abstract

Aims: Increase awareness to the diagnosis of Hyperhaemolysis syndrome and prevent critical anaemia.

Presentation of Case: A 40 year old woman with sickle cell anaemia (SCA) was readmitted with symptoms suggestive of sepsis. In the previous admissions she was received 5 units of cross-matched compatible red blood cells (RBCs), and was discharged on last admission with a PCV of 24%. On readmission, she had a PCV of 13%, and was transfused 4 units of cross-matched compatible RBCs. Her PCV marginally improved to 16% but dropped to 12% the next day, with deteriorating clinical condition. 2 units of cross-matched compatible RBCs transfusions was requested with a haematologist review. A diagnosis of hyperhaemolytic syndrome was made, supported by laboratory findings of; haemoglobinuria and hyperbilirubinemia on urine analysis: negative direct antiglobulin test (DAT); negative Indirect antiglobulin test (IDAT); poor reticulocyte response with reticulocyte count of 8.3% (0.5%–2.5%), and reticulocyte production index at 1.66; Peripheral blood smear showed nucleated RBCs and spherocytes, Albumin- 32g/dl (35- 50g/dl), increased total bilirubin- 55.4mmol/L (17- 22.3 mmol/L). Further blood transfusions was suspended, and steroids commenced for 5 days. Patient’s clinical condition subsequently improved. She was discharged on the 5th day of steroid therapy with a PCV of 20%, followed by tapering doses of prednisolone for 4 weeks. One year of follow-up showed no new red blood cell antibody in her serum, no need further transfusions, and a steady state PCV of 25%.

Conclusion: Hyperhaemolysis syndrome is a potentially life threatening complication of blood transfusion. High index of suspicion and early recognition is important especially when managing patients with SCA who present with worsening anaemia after RBC transfusions.

Keywords: Blood transfusion, hyper haemolysis syndrome, severe haemolysis, steroid

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