Hemoglobin H-Constant Spring Disease Who Survives Longer Than Eight Decades: A Case Report

Abstract

          Hemoglobin H-Constant Spring disease is a genetically transmitted microcytic anemia. It is one of alpha thalassemias that is usually characterized by the moderate degree of anemia. Although it is highly prevalentin Thailand, it has never been found in the patient of more than 8 decades of age as in our patient. He was an 83-year-old Thaimarried man who was referred to a hematologist because of chronic anemia. The physical examination revealed only pallor, no jaundice, and no hepatosplenomegaly. The blood tests showed: Hb 6.5 g%, Hct 22.0 %, MCV 71.0 fL, MCH 21.0 pg, normal WBC and platelet counts, mild poikilocytosis, microcytosis, and hypochromia, and the presence of polychromasia. The Hb electrophoresis using capillary zone electrophoresis method (Sebia®): Hb CSA₂ AH, Hb H11.3 % and Hb Constant Spring 3.7 %, serum ferritin 2,446.64 ng/mL. The direct antiglobulin test was negative. The definite diagnosis was Hb H-Constant Spring with secondary hemosiderosis. He refused to be transfused and he had never been transfused for life because of lack of anemic symptom at all. Only genetic counseling, folic acidanddeferiprone were offered to him. Many factors were proposed to contribute the long lasting sick patient the longevity more than 8 decades that was more than the male life expectancy of 70.96 years in Thailand, including the short stature with quite low BMI, serum ferritin less than 2,500 ng/mL, living in the country, no smoking, and working hard in the paddy field for life.