Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld

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. Ane...

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Main Authors: Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, Hagit Baris Feldman
Format: Article
Language:English
Published: Rambam Health Care Campus 2021-07-01
Series:Rambam Maimonides Medical Journal
Subjects:
Online Access:https://www.rmmj.org.il/issues/50/1229/manuscript
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spelling doaj-11071def7a7d46b5a985c1f339a8a08f2021-07-20T09:03:06ZengRambam Health Care CampusRambam Maimonides Medical Journal2076-91722021-07-01123e002510.5041/RMMJ.10446Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris FeldEliyakim Hershkop0Idan Bergman1Alina Kurolap2Najib Dally3Hagit Baris Feldman4The Ruth and Bruce Rappaport Faculty of Medicine, Technion–Israel Institute of Technology, Haifa, Israel; and Department of Internal Medicine, Maimonides Medical Center, Brooklyn, NY, USAThe Ruth and Bruce Rappaport Faculty of Medicine, Technion–Israel Institute of Technology, Haifa, IsraelThe Genetics Institute and Genomics Center, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, IsraelThe Hematology Unit, Ziv Medical Center, Safed, Israel; and The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, IsraelThe Genetics Institute and Genomics Center, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel; and Sackler School of Medicine, Tel Aviv University, Tel Aviv, IsraelGaucher 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.https://www.rmmj.org.il/issues/50/1229/manuscriptenzyme replacement therapygaucher diseasehemolytic anemianon-immune hemolytic anemia
collection DOAJ
language English
format Article
sources DOAJ
author Eliyakim Hershkop
Idan Bergman
Alina Kurolap
Najib Dally
Hagit Baris Feldman
spellingShingle Eliyakim Hershkop
Idan Bergman
Alina Kurolap
Najib Dally
Hagit Baris Feldman
Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld
Rambam Maimonides Medical Journal
enzyme replacement therapy
gaucher disease
hemolytic anemia
non-immune hemolytic anemia
author_facet Eliyakim Hershkop
Idan Bergman
Alina Kurolap
Najib Dally
Hagit Baris Feldman
author_sort Eliyakim Hershkop
title Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld
title_short Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld
title_full Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld
title_fullStr Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld
title_full_unstemmed Non-immune Hemolysis in Gaucher Disease and Review of the Literature Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, and Hagit Baris Feld
title_sort non-immune hemolysis in gaucher disease and review of the literature eliyakim hershkop, idan bergman, alina kurolap, najib dally, and hagit baris feld
publisher Rambam Health Care Campus
series Rambam Maimonides Medical Journal
issn 2076-9172
publishDate 2021-07-01
description 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.
topic enzyme replacement therapy
gaucher disease
hemolytic anemia
non-immune hemolytic anemia
url https://www.rmmj.org.il/issues/50/1229/manuscript
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