Acquired haemoglobin H disease, complicating a myeloproliferative syndrome: a case report

A case of acquired haemoglobin H disease in association with a myeloproliferative disorder is described. Severe haemolysis with hypochromic microcytic anaemia was present. Haemoglobin H formed 18% of the circulating haemoglobin and 60% of the red cells showed multiple inclusions on incubation with b...

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Bibliographic Details
Published inPathology Vol. 10; no. 4; p. 329
Main Authors Lindsey, R J, Jackson, J M, Raven, J L
Format Journal Article
LanguageEnglish
Published England 01.01.1978
Subjects
Aged
Anemia, Hypochromic - etiology
Antigens
Hemoglobin H - biosynthesis
Hemoglobinopathies - complications
Hemolysis
Humans
Male
Myeloproliferative Disorders - complications
Syndrome
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Summary:A case of acquired haemoglobin H disease in association with a myeloproliferative disorder is described. Severe haemolysis with hypochromic microcytic anaemia was present. Haemoglobin H formed 18% of the circulating haemoglobin and 60% of the red cells showed multiple inclusions on incubation with brilliant cresyl blue. Blood film and absolute red cell values from a previous unrelated illness were normal, proving the acquired nature of the haemoglobin abnormality. Alpha/beta chain synthesis was measured in vitro and the degree of imbalance (alpha/beta ratio 0.39) was similar to that seen in the inborn thalassaemic disorder. A small proportion of red cells showed i-antigen reactivity but their haemoglobin H content was no different from the majority of cells which were l-antigen positive.
ISSN:0031-3025
DOI:10.3109/00313027809063521