TY - JOUR
T1 - Non-immune hemolysis in gaucher disease and review of the literature
AU - Hershkop, Eliyakim
AU - Bergman, Idan
AU - Kurolap, Alina
AU - Dally, Najib
AU - Feldman, Hagit Baris
N1 - Publisher Copyright:
© 2021 Hershkop et al.
PY - 2021/7/20
Y1 - 2021/7/20
N2 - Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of “Gaucher cells.” These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.
AB - Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of “Gaucher cells.” These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.
KW - Enzyme replacement therapy
KW - Gaucher disease
KW - Hemolytic anemia
KW - Non-immune hemolytic anemia
UR - http://www.scopus.com/inward/record.url?scp=85111704240&partnerID=8YFLogxK
U2 - 10.5041/RMMJ.10446
DO - 10.5041/RMMJ.10446
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C2 - 34270405
AN - SCOPUS:85111704240
SN - 2076-9172
VL - 12
JO - Rambam Maimonides Medical Journal
JF - Rambam Maimonides Medical Journal
IS - 3
M1 - e0025
ER -