Hemoglobin H disease, one of the more
recently recognized hemoglobinopathies, is
characterized by the presence of hepatosplenomegaly,
microcytic hypochromic anemia,
intraerythrocytic inclusion bodies, and
two hemoglobin components found on
electrophoresis—normal hemoglobin and an
abnormal component migrating faster than
normal hemoglobin. Hemoglobin H disease
has been observed in 3 members of a Chinese
family,1 3 other Chinese,2 28 Siamese,3 2 unrelated
Filipinos,4 a Greek family,5 a Cypriot,6
a Malayan,7 an Arab from Jordan,7
5 Chinese-Indonesians,8 and a Nepalese
Gurkha woman.2 In this report are presented
the clinical, hematological, and family studies
of another person of Chinese extraction with
hemoglobin H disease.
Report of Case
The propositus* (No. 1) is a 42-year-old
Chinese man, fire-control mechanic, with a history
of mild to moderate recurrent nonradiating epigastric
pain, postprandial bloating, nagging low
backache, small stature, and peculiar skin color
since childhood. In 1952 the epigastric pain was
especially severe, causing him to faint.
Jim RTS. Hemoglobin H Disease. AMA Arch Intern Med. 1958;102(3):400–404. doi:https://doi.org/10.1001/archinte.1958.00030010400008
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