title: Persistent splenomegaly in an adult female with homozygous sickle cell anemia.
abstract: Sickle cell anemia (SCA) is associated with repeated episodes of erythrostasis in the spleen, which lead to thrombosis and infarction of the spleen resulting in "autosplenectomy" which is usually complete by 8 years of age. We present a case of a 22-year-old female who presented with complaints of fever, bone pain and joint swelling. On examination she had pallor, icterus and moderate splenomegaly. Her hemoglobin was 7.5 g/dl. Peripheral smear showed many sickled red cells. Slide test for sickling was positive with 2% sodium metabisulphite. Hemoglobin electrophoresis revealed a single band in the hemoglobin S, D, and G region. No band was seen in the HbA & HbA(2) region. HbF level was 0%. USG showed an enlarged spleen with few defined hypoechoeic lesion. We present this case because of rarity of association of homozygous SCA with splenomegaly in this age group, the confusion that echogenic lesions in spleen can create and to emphasize the risk of sequestration crises, which remains in such cases.
authors.affiliation: Department of Pathology, Maulana Azad Medical College, New Delhi, India.
authors.forename: Sompal
authors.initials: S
authors.lastname: Singh
authors.forename: Deepak Kumar
authors.initials: DK
authors.lastname: Singh
authors.forename: Ruchika
authors.initials: R
authors.lastname: Gupta
authors.forename: Sonu
authors.initials: S
authors.lastname: Nigam
authors.forename: Tejinder
authors.initials: T
authors.lastname: Singh
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000235:genetics; D005260:Female; D006455:Hemoglobins, Abnormal / Q000032:analysis; D006720:Homozygote; D006801:Humans; D013163:Splenomegaly / Q000097:blood / Q000209:etiology* / Q000235:genetics
chemical_list: D006455:Hemoglobins, Abnormal