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Hematology Reports
Volume 9
Issue 1
10.4081/hr.2017.6934
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Case Report

A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease

by
Matthew S. Karafin
1,2,*,
Arun Singavi
2,
Susan T. Johnson
1 and
Joshua J. Field
1,2
1
Medical Sciences Institute, BloodCenter of Wisconsin and Medical College of Wisconsin, 8733 Watertown Plank Road Milwaukee, WI 53226, USA
2
Medical College of Wisconsin, Milwaukee, WI 53226, USA
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2017, 9(1), 8-11; https://doi.org/10.4081/hr.2017.6934
Submission received: 13 October 2016 / Revised: 19 November 2016 / Accepted: 21 November 2016 / Published: 1 March 2017

Abstract

In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis. Despite treatment, the bone marrow failure did not improve and the patient died on hospital day 38. This case illustrates the potential risks of transfusion in a patient with sickle cell disease, especially one with previous hemolytic reactions. While uncommon, hyperhemolysis can cause death, in this case by extensive bone marrow necrosis. In patients with sickle cell disease, judicious use of red cell transfusions with phenotypically-matched units can diminish, but never completely abrogate, the risks associated with transfusion.
Keywords: bone marrow necrosis; sickle cell disease; hyperhemolysis syndrome bone marrow necrosis; sickle cell disease; hyperhemolysis syndrome

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MDPI and ACS Style

Karafin, M.S.; Singavi, A.; Johnson, S.T.; Field, J.J. A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease. Hematol. Rep. 2017, 9, 8-11. https://doi.org/10.4081/hr.2017.6934

AMA Style

Karafin MS, Singavi A, Johnson ST, Field JJ. A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease. Hematology Reports. 2017; 9(1):8-11. https://doi.org/10.4081/hr.2017.6934

Chicago/Turabian Style

Karafin, Matthew S., Arun Singavi, Susan T. Johnson, and Joshua J. Field. 2017. "A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease" Hematology Reports 9, no. 1: 8-11. https://doi.org/10.4081/hr.2017.6934

APA Style

Karafin, M. S., Singavi, A., Johnson, S. T., & Field, J. J. (2017). A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease. Hematology Reports, 9(1), 8-11. https://doi.org/10.4081/hr.2017.6934

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