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Acquired aplastic anaemia: a PNH‐like disease?
Author(s) -
Nissen Catherine,
Gratwohl Alois,
Speck Bruno,
Würsch Andreas,
Moser Yolanda,
Weis Johanna
Publication year - 1986
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.1365-2141.1986.tb04129.x
Subject(s) - aplastic anemia , immunology , bone marrow , haematopoiesis , paroxysmal nocturnal hemoglobinuria , medicine , chemistry , stem cell , biology , genetics
S ummary . Bone marrow from 20 patients with aplastic anaemia at different stages of disease and from three patients with paroxysmal nocturnal haemoglo‐binuria (PNH) was incubated in isosmolar sucrose with 5% autologous serum prior to culture in methylcellulose. If fresh serum was used, colony formation by granulocyte‐macrophage colony forming cells (GM‐CFC) and immature eryth‐roid precursors (BFU‐E) was reduced to approximately 50% in all patients tested, at any stage of disease, including complete autologous bone marrow recovery. Heat inactivation and complement inactivation with EDTA completely abrogated this inhibitory serum effect. Selective inactivation of the classical, antibody dependent complement pathway with Mg 2 + EGTA reduced the inhibitory effect by 50%. Complement sensitivity of haemopoietic precursors is a known feature of PNH. Since the majority of our patients did not have PNH as judged by a negative sucrose‐test on mature erythrocytes, we conclude that, in aplastic anaemia, haemopoietic cells express a PNH‐like defect at a primitive level.