The absence of hemoglobin Hb H band in the proven hemoglobin H disease patient due to the iron deficiency anemia

Abstract

Introduction: Iron deficiency anemia has not only been rarely seen among hemoglobin H disease patients but can mask the diagnosis of hemoglobin H disease also.Case Presentation: A 50-year-old Thai woman had marked and progressive anemia with just splenomegaly on the physical examination. Her blood tests showed: Hb 3.8 g%, MCV 50.4 fl, MCH 15.4 pg, ferritin 5.0 ng/ml, serum iron 33 ug/dl, TIBC 123 ug/dl, the Hb analysis using the high-performance liquid chromatography method found only A 2 A, Hb A 2 2.1 %. Iron deficiency anemia was diagnosed and continuously treated with the oral iron tablets. After two months of treatment, her blood showed: Hb 7.7 g%, MCV 69.3 fl, MCH 20.5 pg, reticulocyte 6.0 %, ferritin 6.2 ng/ml, Hb analysis showed: A 2 AH Bart, Hb A 2 1.7 %. The diagnosis of Hb H disease was added. Later Hb H was confirmed by the positive PCR for alpha thalassemia-1, Southeast Asian deletion and alpha thalassemia-2, 3.7 kb deletion, genes. After five months of the iron treatment, Hb 8.8 g%, MCV 59.3 fl, MCH 16.7 pg, ferritin 110.7 ng/ml, Hb analysis: A 2 ABartH, Hb A 2 1.4 %. The band of Hb H was found after Hb concentration was raised even though the ferritin level was still low. Conclusion:The absence or presence of Hb H band of the Hb H disease patient on Hb electrophoresis seemingly depended on the hemoglobin concentration more than the sufficiency of the iron storage.