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A 3 1 -year-old African American female is admitted to the hospital with diffuse lower back in addition to right shoulder and knee pain. Patient reports several similar epi-sodes previously. Physical exami nation reveals decreased range of motion in affected joints secondary to pain with no obvious swelling. Laboratory evaluation reveals hemo-globin of 7. 7 g/dl, mean corpuscular volume (MCV) of 85 fl. and normal leukocyte and platelet coun ts. Peripheral smear is shown below.
What is the most likely underlying condition?
- Beta thalassemia major
- Hemoglobin H disease
- Sickle-beta thalassemia
- Sickle cell trait
- Sickle cell disease
Medicine
E. The case describes a young African American female with multifocal bone pain. Labora-tory evaluation reveals a moderately severe normocytic anemia. Normal MCV argues strongly against thalassemia. Peripheral blood smear reveals few sickled red blood cells (RBC) in addition to few baso-philic RBC inclusions representing Howell-Jolly bodies, which are nuclear remnants that are removed by the spleen. Their presence in the peripheral blood is suggestive of decreased splenic function. Func-tional asplenia is an early complication of sickle cell disease that is evident within the first year of life.
Nuclear medicine spleen and liver scan was obtained and revealed nearly absent splenic uptake consis-tent with functional asplenia (Blood. 201 1;117(9):26 14, Lancet. 20 1 1;378(9785):86).
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