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.
Highlights
We present a unique case of transfusion- ine) on their outer surface, which some the- ble patients.[18]
The strengthening of the s autoantibody was associated with worsenu ing hemolysis and a clinical picture consisl tent with acute hyperhemolysis: a recent ia transfusion, a new red cell antibody, reticc ulocytopenia, and a severe anemia. e in this case, the severe anemia and resultant ischemia from red cell m hemolysis likely fatally exacerbated extenm sive, clinically-significant bone marrow necrosis; a process that likely began during o her first hospitalization as reflected by her c progressively strengthening DAT and worsening thrombocytopenia during that time n period. o Hyperhemolysis syndrome is a wellN reported and life-threatening complication body-independent phagocytosis by activated macrophages in patients with Sickle cell disease (SCD).[6]
A recent study suggests that mixed-type immune hemolytic anemias may be more severe than other forms of immune hemolysis, with 63% of mixed hemolysis patients presenting with a hemoglobin
Summary
She developed multisys- destroys both donor and recipient red cells, necrotic marrow, rarely extensive tem organ failure and continued to decline known as bystander hemolysis.[6,11] As in our enough to impact hematopoiesis, as was cognitively during the hospitalization, and patient, previous studies have shown a seen in our case.[18,19] Common symptoms of was transitioned to comfort care.
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