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.

Delayed Hemolytic Transfusion Reaction With Hyperhemolysis Syndrome Due to Anti-M Alloantibody in Myelofibrosis: A Case Report

“…HHS commonly occurs in sickle cell disease (SCD) and beta-thalassemia [ 5 ], but rarely occurs in other hematological disorders such as chronic lymphocytic leukemia, myelofibrosis, anemia of chronic disease, marginal zone lymphoma, and anemia of chronic disease [ 11 ].…”

“…A delayed hematological transfusion reaction (DHTR) is a hematological reaction that occurs a few days to weeks post-RBC transfusion due to alloimmunization [ 4 ]. Hyperhemolysis syndrome (HHS) is a life-threatening condition occurring post-RBC transfusion, commonly seen in sickle cell anemia, b-thalassemia, and rarely in myelofibrosis [ 5 ].…”

“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

“…A main benefit of rituximab for pregnant women is that transplacental transfer of IgG is limited, so that B-cell depletion in the fetus is mainly observed when this drug is used during the last trimesters [44,57]. A number of measures to promote (maternal) erythropoiesis, using adjuvant therapy with vitamin B12, folic acid, and eventually erythropoietin can be beneficial [56].…”

Hyperhemolytic Crisis Following Transfusion in Sickle Cell Disease With Acute Hepatic Crisis: A Case Report

“…Subsequent transfusions may cause hyperhemolysis syndrome, whereby both native and donor red blood cells undergo further hemolysis, leading to lower posttransfusion hemoglobin levels. It can present acutely within seven days of the transfusion incident or may be delayed whereby alloantibodies are more likely to have formed [2]. We report a case of HHS in a patient who was initially admitted for a sickle cell painful crisis, developed signs of hemolysis acutely after he got blood transfusion to highlight the importance of early recognition of such a rare phenomenon besides being hypervigilant to a patient's blood transfusion record.…”