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The Haemoglobin Picture in Cooley's Disease
Author(s) -
Silvestroni E.,
Bianco I.,
Graziani B.
Publication year - 1968
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.1365-2141.1968.tb01501.x
Subject(s) - agar gel , medicine , biology , microbiology and biotechnology
S ummary The haemoglobin picture has been studied in 38 patients with Cooley's anaemia, all homozygous for β‐microcythaemia. In 10 of the patients Hb A 1 was completely absent, in the remaindcr it was present on agar gel electrophoresis in amounts varying from minute traces to a rather abundant quota. In 34 of the Cooley's patients studied Hb A 2 was present in amounts comparable to those found in normal non‐microcythaemic subjects. In the rest it was raised as in heterozygous carriers of β‐microcythaemia. There was no correlation between the haemoglobin picture and the severity of clinical findings. The genetic significance of the different types of haemoglobin picture found in Cooley's patients is discussed.