Acute splenic sequestration in an adult with hemoglobin S-C disease.
Acute splenic sequestration crisis is a common, potentially life-threatening complication of sickle cell anemia in children that is uncommon in adults.We present the case of a 44-year-old gentleman with undiagnosed hemoglobin S-C disease who developed intense back pain, marked abdominal distension, systemic inflammatory response syndrome, and multisystem organ failure that first presented as acute splenic sequestration crisis. The hemoglobinopathy SC is a disease caused by heterozygous-globin chain mutations with over-lapping clinical features of sickle cell disease with changes in the frequency of these manifestations reflected by the combination of characteristics of hemoglobin C and hemoglobin S. In hemoglobin S-C disease, autosplenectomy is rare and splenomegaly usually persists until adulthood;vasoocclusive complications are seen less habitually and become evident at a later time compared with sickle cell disease. The diagnosis of hemoglobin S-C disease is essentially done by exclusion. Transfusion of red blood cells is the treatment of choice, but splenectomy is indicated if transfusion therapy fails. A review of the literature and keypoints for the emergency practitioner are included.
Rivera-Ruiz
Moises
M
Universidad Popular Autónoma del Estado de Puebla, Puebla 72160, Mexico.
Varon
Joseph
J
Sternbach
George L
GL
D000208:Acute Disease; D000328:Adult; D001416:Back Pain / Q000209:etiology; D006450:Hemoglobin SC Disease / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D008297:Male; D013156:Splenectomy; D013158:Splenic Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*