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Gaucher‐like Cells and Congenital Dyserythropoietic Anaemia, Type II (HEMPAS)
Author(s) -
Dorpe A. Van,
Orshoven A. BroeckaertVan,
Desmet V.,
Verwilghen R. L.
Publication year - 1973
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1973.tb01726.x
Subject(s) - erythropoiesis , ineffective erythropoiesis , bone marrow , microbiology and biotechnology , pathology , immunology , biology , chemistry , medicine , anemia
S ummary . .‘Gaucher‐like cells’ were observed in the bone marrow of 18 out of 20 HEMPAS (CDA II) patients. These cells contain birefringent, PAS positive material. Further cytochemical procedures identified the inclusions as a glycoprotein or a glycopeptide but the presence of cerebrosides could not be excluded. An intralysosomal localization was suggested by electronmicroscopy. HEMPAS is characterized by inefficient erythropoiesis and it is suggested that accumulation of a catabolite of the erythroblasts gives rise to these cells. A possible relation with the cell membrane anomaly of HEMPAS erythrocytes is discussed.