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...
Main Authors: | , , , , |
---|---|
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 |
id |
doaj-11071def7a7d46b5a985c1f339a8a08f |
---|---|
record_format |
Article |
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 |
work_keys_str_mv |
AT eliyakimhershkop nonimmunehemolysisingaucherdiseaseandreviewoftheliteratureeliyakimhershkopidanbergmanalinakurolapnajibdallyandhagitbarisfeld AT idanbergman nonimmunehemolysisingaucherdiseaseandreviewoftheliteratureeliyakimhershkopidanbergmanalinakurolapnajibdallyandhagitbarisfeld AT alinakurolap nonimmunehemolysisingaucherdiseaseandreviewoftheliteratureeliyakimhershkopidanbergmanalinakurolapnajibdallyandhagitbarisfeld AT najibdally nonimmunehemolysisingaucherdiseaseandreviewoftheliteratureeliyakimhershkopidanbergmanalinakurolapnajibdallyandhagitbarisfeld AT hagitbarisfeldman nonimmunehemolysisingaucherdiseaseandreviewoftheliteratureeliyakimhershkopidanbergmanalinakurolapnajibdallyandhagitbarisfeld |
_version_ |
1721293813184462848 |