Acquired Hemoglobin H Disease

Robert W. Hamilton, Elias Schwartz, Jean Atwater, Allan J. Erslev

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Although hemoglobin H disease is usually inherited, similar syndromes may be acquired. In a 73-year-old woman of British descent with a myeloproliferative syndrome characterized by striking megakaryocytic hyperplasia and thrombocytosis, we also found hemolytic anemia with hypochromia, microcytosis, marked poikilocytosis and 20 per cent hemoglobin H (beta4). Incubation with brilliant cresyl blue produced inclusions in 60 per cent of the red cells. Hematologic studies before the onset of this illness gave normal results. In vitro studies of globinchain synthesis revealed a marked decrease of alpha-chain production similar to that found in hereditary hemoglobin H disease. The alpha/beta ratio of radioactivities was 0.13, demonstrating a severe imbalance of globin synthesis. Studies of the patient's two children gave normal results. Hematologic and globin synthesis in this acquired disorder is similar to those of its hereditary counterpart.

Original languageEnglish (US)
Pages (from-to)1217-1221
Number of pages5
JournalNew England Journal of Medicine
Volume285
Issue number22
DOIs
StatePublished - Nov 25 1971

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alpha-Thalassemia
Globins
Hemoglobin H
Thrombocytosis
Hemolytic Anemia
Radioactivity
Hyperplasia
Alpha-Thalassemia Myelodysplasia Syndrome
In Vitro Techniques
Brilliant Cresyl Blue

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Hamilton, Robert W. ; Schwartz, Elias ; Atwater, Jean ; Erslev, Allan J. / Acquired Hemoglobin H Disease. In: New England Journal of Medicine. 1971 ; Vol. 285, No. 22. pp. 1217-1221.
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Hamilton, RW, Schwartz, E, Atwater, J & Erslev, AJ 1971, 'Acquired Hemoglobin H Disease', New England Journal of Medicine, vol. 285, no. 22, pp. 1217-1221. https://doi.org/10.1056/NEJM197111252852202

Acquired Hemoglobin H Disease. / Hamilton, Robert W.; Schwartz, Elias; Atwater, Jean; Erslev, Allan J.

In: New England Journal of Medicine, Vol. 285, No. 22, 25.11.1971, p. 1217-1221.

Research output: Contribution to journalArticle

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AU - Hamilton, Robert W.

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AU - Erslev, Allan J.

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AB - Although hemoglobin H disease is usually inherited, similar syndromes may be acquired. In a 73-year-old woman of British descent with a myeloproliferative syndrome characterized by striking megakaryocytic hyperplasia and thrombocytosis, we also found hemolytic anemia with hypochromia, microcytosis, marked poikilocytosis and 20 per cent hemoglobin H (beta4). Incubation with brilliant cresyl blue produced inclusions in 60 per cent of the red cells. Hematologic studies before the onset of this illness gave normal results. In vitro studies of globinchain synthesis revealed a marked decrease of alpha-chain production similar to that found in hereditary hemoglobin H disease. The alpha/beta ratio of radioactivities was 0.13, demonstrating a severe imbalance of globin synthesis. Studies of the patient's two children gave normal results. Hematologic and globin synthesis in this acquired disorder is similar to those of its hereditary counterpart.

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