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Hereditary Erythroblastic Multinuclearity Associated with a Positive Acidified‐Serum Test: a Type of Congenital Dyserythropoietic Anaemia
Author(s) -
Crookston J. H.,
Crookston Marie C.,
Burnie Katherine L.,
Francombe W. H.,
Dacie J. V.,
Davis J. A.,
Lewis S. M.
Publication year - 1969
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.1365-2141.1969.tb05660.x
Subject(s) - ineffective erythropoiesis , erythropoiesis , agglutination (biology) , lysis , medicine , antibody , immunology , microbiology and biotechnology , biology , anemia
S ummary . In five patients (including two sisters) an unusual anaemia was characterized by erythroblastic multinuclearity, ineffective erythropoiesis and a positive acidified‐serum test. Unlike PNH, the sugar‐water test was always negative in these patients. The lysis of their cells by acidified normal sera indicated their abnormal sensitivity to an agglutinating and complement‐binding antibody present in some normal subjects. The patients cells gave high agglutination scores with anti‐i, and were unusually susceptible to lysis by anti‐i and anti‐I. The disorder is apparently inherited as an autosomal recessive character.