Hereditary spherocytosis

Hereditary spherocytosis is a congenital hemolytic anemia with a wide clinical spectrum (from symptom-free carriers to severe hemolysis) characterized by anemia, variable jaundice, splenomegaly and cholelithiasis.

Immunodeficiency

Failure of the immune system to protect the body adequately from infection, due to the absence or insufficiency of some component process or substance.


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(per page)
PMID (PMCID)
10602897
 MIXED_SAMPLE Adult
Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection.
Eber SW, Langendorfer CM, Ditzig M, Reinhardt D, Stohr G, Soldan W, Schroter W, Tchernia G.
Ann Hematol. 1999;78(11):524-8.
Very late sepsis in splenectomized patients with hereditary spherocytosis has been seen rarely up to now; the frequency and the immunodeficiency causing it are largely unknown.