A 24‐year‐old woman with sickle cell disease (SCD) presented with 1 day of progressive bilateral lower‐extremity pain. She was afebrile and had no chest, back, or joint pain. She had edema and tenderness to palpation of both lower extremities. Her sclerae were anicteric, JVP was not elevated, and lungs had clear breath sounds. Her heart was regular and without murmurs. Her labs revealed a hemoglobin (Hgb) of 9.4 g/dL. reticulocyte of 4.7%, and bilirubin of 3.6 mg/dL. Several hours later, she developed gross hematuria, but the urinalysis revealed 0 RBCs/hpf. The Hgb was 8.3, with elevated lactate dehydrogenase and low haptoglobin values. With further questioning, it was learned she had received a transfusion of 2 units of packed red blood cells (PRBCs) 1 week prior for suspected acute chest syndrome. Over the ensuing 24 hours, she developed a fever with a leukocytosis of 32.000/mm3, and her Hgb decreased to 4. A detailed antibody screen returned negative. She was transferred to the intensive care unit for mechanical ventilation and hemodynamic support. Because of concern for hyperhemolysis syndrome, blood transfusion was held off initially; however, her condition progressed to multiorgan failure with troponin elevation to 80 ng/mL. and the patient was started on solumedrol, IVIG, and PRBC transfusion. Despite these efforts, she went into cardiac arrest and died 3 days after admission.
Hyperhemolysis syndrome (HS) is a rare but life‐threatening delayed hemolytic transfusion reaction in patients with SCD. It is critical to recognize this condition early, as further PRBC transfusions can accelerate hemolysis. The clinical findings of HS usually occur within 1 week of PRBC transfusion with fever, hemoglobinuria, and back, leg, and abdominal pain that may mimic sickle cell pain crisis. Characteristic features of HS include rapid hemolysis, absence of an identifiable RBC alloantibody, negative antibody screen (DAT), reticulocytopenia, and a precipitous drop in hemoglobin that is lower than the pretransfusion Hgb value. In patients with SCD, macrophages are chronically activated and have an affinity for HgbS. It has been suggested that HS involves further activation of these macrophages that then bind both sickled RBCs and transfused RBCs and destroy them via contact lysis or by erythrophagocytosis. There may be a benefit in solumedrol and IVIG therapy in this condition, as they suppress macrophage activity and block the adhesion of sickle cells/reticulocytes to macrophages, respectively. The risks and benefits of further transfusion must be weighed carefully in these patients.
Acute pain syndrome in SCD is a common condition encountered by hospitalists HS must be included in the differential diagnosis of a patient with SCD and a vaso‐occlusive crisis who have recently received a transfusion. Physicians should be aware of the rare, but potentially fatal complications of PRBC transfusions in SCD patients.
A. Weber, none; S. Silver, none: A. Campbell, none; M. McNamara, none; C. Kim, Pfizer, Advisory Board.
To cite this abstract:Weber A, Silver S, Campbell A, McNamara M, Kim C. Acute Pain in a Sickle Cell Patient, Beyond Just a Vaso‐Ocelusive Pain Crisis. Abstract published at Hospital Medicine 2010, April 8-11, Washington, D.C. Abstract 378. Journal of Hospital Medicine. 2010; 5 (suppl 1). https://www.shmabstracts.com/abstract/acute-pain-in-a-sickle-cell-patient-beyond-just-a-vasoocelusive-pain-crisis/. Accessed August 24, 2019.