Hematological malignancy in hemoglobin D disease

✍ Scribed by Sumitra Dash; Sudarshan Kumar; Radharaman J. Dash

• Publisher: John Wiley and Sons
• Year: 1988
• Tongue: English
• Weight: 61 KB
• Volume: 27
• Category: Article
• ISSN: 0361-8609
• DOI: 10.1002/ajh.2830270416

✦ Synopsis

Hematological Malignancy in Hemoglobin D Disease

Russo and Schiliro in their letter [l] have recommended that cases of association between hereditary hematologic diseases and various malignancies should be reported in order to establish if the incidence is higher than expected. In this connection we would like to report two cases of hematologic malignancy in hemoglobin D disease, an association not yet reported in the English literature.

One was a case of acute myeloid leukemia (M3-FAB) in a 27-year-old male, heterozygous for HbD-Punjab. Another was a case of Hodgkin disease, lymphocytic predominance type in stage IVB, in a 14-year-old boy who was found homozygous for HbD-Punjab. These two cases were detected among 95 cases of hematologic malignancies analysed by us for abnormal hemoglobin. The frequency was slightly higher than the prevalence of 1 % HbD-Punjab in the local population [2]. It is difficult to accept the several postulates put forward on the basis of a hemolytic process [3,4] as hemoglobin D-Punjab by itself either in heterozygous or homozygous state does not produce hemolysis [5].