Hyperhemolysis Syndrome in a Patient With Sickle Cell Disease and Acute Chest Syndrome

Abstract

Sickle cell anemia patients often present to the hospital with acute vaso-occlusive pain crisis. Symptoms can include, but are not limited to, chest pain, abdominal pain, and musculoskeletal pain. These symptoms are brought about due to the pathology of the disease. Abnormal hemoglobin S causes red blood cells to band together, otherwise known as "sickling." These patients also often present with very low hemoglobin levels on initial evaluation. In most cases, packed red blood cell transfusions are needed in order to replenish these patient's functional hemoglobin supply. Unfortunately, transfusing sickle cell patients can lead to an unwanted consequence, that of hyperhemolysis syndrome, in which blood transfusions prompt further hemolysis of the already sickled red blood cells. When this complication arises, caution must be exercised in deciding the next steps of treatment.

Keywords: acute chest syndrome; hemoglobin; hemoglobinopathies; hyperhemolysis syndrome; methylprednisolone; nucleated red blood cells; packed red blood cell transfusion; reticulocyte count; sickle cell anemia; sickling.

Figure 1. Patient’s chest X-ray on admission

Figure 2. Patient’s chest X-ray after transfer to the ICU post-intubation. New diffuse bilateral infiltrates are noted (blue arrows), new consolidative changes in the left lower lobe are noted as well (orange arrow).