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Association of Type II Congenital Dyserythropoietic Anaemia and von Willebrand's Disease
Author(s) -
Hernandez P.,
Almagro D.,
Corral J. F.,
Opolski A.,
Sanchez J. A.,
Rodríguez N.
Publication year - 1974
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.1365-2141.1974.tb06811.x
Subject(s) - jaundice , medicine , agglutination (biology) , haemolysis , bone marrow , coombs test , gastroenterology , immunology , antibody
S ummary . In a patient with a history of anaemia, jaundice and splenomegaly, the bone marrow showed erythropoietic hyperplasia with 20% of multinucleated erythroblasts. His red cells gave high agglutination scores with anti‐i, and haemolysis tests with acidified iso‐group sera were positive. A type II congenital dyserythropoietic anaemia was diagnosed in this patient and this was shown to be associated with von Willebrand's disease. A brother of the propositus also had a positive acidified serum test and showed agglutination by anti‐i, but polynucleated erythroblasts were not found.