Acute splenic sequestration crisis in an adult with sickle beta-thalassemia.
Acute splenic sequestration crisis (ASSC) is a major cause of morbidity and mortality in children with sickle cell disease. Reports of ASSC in adults with sickle beta-thalassemia (S-beta(thal)) are rare and consist of isolated case reports comprising a total of seven patients, three of whom died during the crisis. We report a 22-year-old man with S-beta(thal) who developed ASSC 1 day after suffering multiple blunt trauma. Systemic inflammatory response to severe blunt trauma may have precipitated ASSC in our patient. ASSC in adults with S-beta(thal) is a potentially life-threatening complication with a high risk of recurrence. Splenectomy is recommended after the first attack of ASSC in adults with S-beta(thal).
Koduri
Prasad Rao
PR
Kovarik
Paula
P
D000328:Adult; D006450:Hemoglobin SC Disease / Q000150:complications* / Q000601:surgery; D006801:Humans; D006971:Hypersplenism / Q000209:etiology* / Q000601:surgery; D008297:Male; D013156:Splenectomy / Q000379:methods; D013163:Splenomegaly / Q000209:etiology / Q000601:surgery; D018746:Systemic Inflammatory Response Syndrome / Q000209:etiology* / Q000601:surgery; D014947:Wounds and Injuries / Q000150:complications* / Q000601:surgery; D017086:beta-Thalassemia / Q000150:complications* / Q000601:surgery