J Med Assoc Thai 2017; 100 (6):702

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The Appearance of the Band of Hemoglobin H in a Patient of Hemoglobin H Disease and the Activity of Rheumatoid Arthritis: A Case Report
Sripavatakul K Mail, Insiripong S

Hemoglobin H disease is a genetic disorder characterized by the moderate degree of microcytic hemolytic anemia and diagnosed by the presence of the Hb H band on the hemoglobin electrophoresis. In this paper, we report a case of Hb H disease whose diagnosis is delayed because of active rheumatoid arthritis. She was a 64-year-old Thai patient who had been definitely diagnosed as having rheumatoid arthritis for 16 years. She was referred to a hematologist because of the long-term microcytic anemia. On the physical examination, she only had marked pallor without hepatosplenomegaly. The first blood test showed Hb 4.4 g%, Hct 13.2%, MCV 53.5 fL, MCH 17.8 pg, WBC 5,600/mm3, platelet 121,000/mm3, and reticulocyte 4.4%, while the Hb electrophoresis was Hb A2A, Hb A2 2.4%, Hb F 0.2%, and ferritin 1,470.8 ng/ml. When the activity of rheumatoid arthritis was well controlled with methotrexate, prednisolone, and sulfasalazine, the second blood test showed Hb 7.0 g%, Hct 21.3%, MCV 65.1 fL, and MCH 20.3 pg, while Hb electrophoresis was A2A(H?), Hb A2 6.1%, and Hb F 0.7%. Later the third blood test showed Hb 7.9 g%, Hct 23.0%, MCV 64.3 fL, and MCH 22.0 pg, while Hb electrophoresis was Hb A2ABartH, Hb A2 2.1%, and Hb F 1.6%. Finally, she was hematologically diagnosed as Hb H disease. Our patient showed that Hb H band in Hb H disease was absent when the Hb concentration was markedly low during active rheumatoid arthritis but the band became apparent when the Hb concentration was increased during the remission of rheumatoid arthritis.

Keywords: Band of hemoglobin H, Hemoglobin H disease, Rheumatoid arthritis


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