OBSOLETE: Sickle cell disease associated with another hemoglobin anomaly

Sickle cell disease associated with another hemoglobin anomaly refers to a group of compound heterozygous hemoglobinopathies in which an individual inherits one sickle hemoglobin (HbS) gene along with a gene for another abnormal hemoglobin variant. This Orphanet entry (code 251355) is classified as obsolete, meaning it has been retired from active use in the Orphanet classification system and its constituent conditions have been reclassified under more specific disease entries. The most well-known examples of these compound heterozygous conditions include sickle cell–hemoglobin C disease (HbSC), sickle cell–hemoglobin D disease (HbSD), sickle cell–hemoglobin E disease (HbSE), and sickle cell–hemoglobin O-Arab disease, among others. Each of these conditions involves the red blood cells and can cause varying degrees of hemolytic anemia, vaso-occlusive crises, pain episodes, organ damage, and other complications characteristic of sickle cell spectrum disorders, though clinical severity varies depending on the specific co-inherited hemoglobin variant. Because this entry is obsolete, patients and clinicians should refer to the individual, more specific Orphanet entries for each compound heterozygous sickle cell condition for up-to-date clinical information, management guidelines, and treatment options, which may include hydroxyurea, blood transfusions, pain management, and in some cases curative therapies such as hematopoietic stem cell transplantation.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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