Persistent splenomegaly in an adult female with homozygous sickle cell anemia

Hematology
Sompal SinghTejinder Singh

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.

References

Aug 1, 1980·Radiology·B A Carroll, H N Ta
Feb 1, 1993·Clinical Radiology·T M Walker, G R Serjeant
Dec 1, 1996·The American Journal of the Medical Sciences·S Moll, E P Orringer
Jul 14, 2001·Radiographics : a Review Publication of the Radiological Society of North America, Inc·G J LonerganS L Abbondanzo
Apr 18, 2002·East African Medical Journal·A A Olatunji, P O Olatunji
Apr 25, 2002·Klinische Pädiatrie·R Dickerhoff

❮ Previous
Next ❯

Citations

Feb 14, 2015·Transfusion·Berta SantosMonique Loureiro
Jan 10, 2001·The Journal of Pediatrics·J Knight-Madden, G R Serjeant

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Related Papers

Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis
Sinan AkbayramHüseyin Caksen
The Ceylon Medical Journal
G N Lucas, D R Jayawardena
© 2021 Meta ULC. All rights reserved