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.


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


How to Cite

N, Okeke Chinedu, Ode Charity I, Nwankwo Chizoba G, and Jatau Ezra D. 2024. “A Case Report on Hyperhaemolysis Syndrome in a Patient With Sickle Cell Anaemia”. Asian Hematology Research Journal 7 (3):110-14. https://journalahrj.com/index.php/AHRJ/article/view/175.

Downloads

Download data is not yet available.

References

Win H. Hyperhemolysis syndrome in sickle cell disease. Expert Review of Hematology. 2009;2(2):111–115.

Friedman DF, Kim HC, Manno CS. Hyperhaemolysis associated with red cell transfusion in sickle cell disease.Transfusion. 1993;33:148.

Petz LD, Calhoun L, Shulman IA, Johnson C, Herron RM. The sickle cell hemolytic transfusion reaction syndrome. Transfusion. 1997;37:382-392.

Win N. Hyperhaemolysis Syndrome in Sickle Cell Disease. Annals of Hematology and Oncology. 2019;6(7):1258.

Madu AJ, Ugwu AO, Efobi C. Hyperhaemolytic Syndrome in Sickle Cell Disease: Clearing the Cobwebs. Medical Principle and Practice. 2021;30:236–243.

Shankar K, Shah D, Huffman D L, Peterson C, Bhagavatula R. Hyperhemolysis Syndrome in a Patient With Sickle Cell Disease and Acute Chest Syndrome. Cureus. 2021;13(1):e13017. DOI:10.7759/cureus.13017.

Santos B, Portugal R, Nogueira C, Loureiro M. Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases. Transfusion. 2015;55(6pt2): 1394-8.

Banks M, Shikle J: Hyperhemolysis syndrome in patients with sickle cell disease. Arch Pathol Lab Med. 2018;1 42:1425-1427. DOI:10.5858/arpa.

Rogers M, Smith G. Hyperhaemolysis in a patient with chronic lymphocytic leukaemia: letter to the editor. Transfus Med. 2014;24(2):123–4.

Gupta S, Fenves A, Nance ST, Sykes DB, Dzik WS. Hyperhemolysis syndrome in a patient without a hemoglobinopathy, unresponsive to treatment with eculizumab: Hyperhemolysis. Transfusion. 2015;55(3):623– 628.

Treleaven JG, Win N. Hyperhaemolysis syndrome in a patient with myelofibrosis. Hematology. 2004;9(2):147– 149.

Yan M, Callum J, Lin Y. Hyperhemolysis associated with marginal zone lymphoma. Leuk Lymphoma. 2015;56(3):829– 831.

Eberly LA, Osman D, Collins NP. Hyperhemolysis Syndrome without Underlying Hematologic Disease. Case Reports in Hematology. 2015; 2015:180526. DOI: 10.1155/2015/180526.

Rihsling A, Simeunovic H, Sanchez S, Henny C, Lejon Crottet S, Mansouri Teleghani B, Daskalakis M, Müller M, Raio L, Rovó A. “Don’t Add Fuel to the Fire”– Hyperhemolysis Syndrome in a Pregnant Woman with Compound Sickle Cell Disease/β0-Thalassemia: Case Report and Review of the Literature. Acta Haematologica. 2024;147:378–387.

King KE, Shirey RS, Lankiewicz MW, Young-Ramsaran J, Ness PM. Delayed hemolytic transfusion reactions in sickle cell disease: simultaneous destruction of recipients’ red cells. Transfusion. 1997;37:376-381.

Kalter JA, Ranju G, Greenberg MR, Miller AJ, Allen J. Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report.Clinical Practice and Cases in Emergency Medicine. 2021;5(1):101-104

Mwesigwa S, Moulds JM, Chen A, Flanagan J, Sheehan VA, George A, et al. Whole-exome sequencing of sickle cell disease patients with hyperhemolysis syndrome suggests a role for rare variation in disease predisposition. Transfusion. 2018;58:726-735.

Win N, Tullie Y, Needs M, Chen PE, Okpala I. Use of intravenous immunoglobulin and intravenous methylprednisolone in hyperhaemolysis syndrome in sickle cell disease. Haematology. 2004;9:433–436.

Patel I, Odak M, Douedi S, Alshami A, Upadhayaya VD, Hossain M, Anne M, Patel SV. Eculizumab as a treatment for hyper haemolytic and aplastic crisis in sickle cell disease. European Journal of Case Reports in Internal Medicine 2021;8.

DOI:10.12890/2021_002824.

Win N, New H, Lee E, de la Fuente J. Hyperhemolysis syndrome in sickle cell disease: case report (recurrent episode) and literature review. Transfusion. 2008; 48:1231–1238.

Bolton-Maggs P. Transfusion Complications in Patients with Hemoglobin Disorders. Serious Hazards of Transfusion (SHOT) Annual Report; 2010.