Dr Amna Malik, Dr Hira Khalil, Dr Zeshan Ahmad
Purpose: To examine the clinical and hematological features of hemoglobin H disease. Study design: A descriptive cross-sectional study. Place and duration: In the Medicine Unit II and hematology department of Nishter Hospital Multan for one year duration from March 2019 to March 2020. Methods: We evaluated the clinical features and hematological values of patients for hemoglobin studies using non probability consecutive sampling. Complete blood count was performed on Sysmex KX 21 automated hematology analyzer. Hemoglobin H inclusions were visualized with supra vital staining with New methylene blue. Hemoglobin electrophoresis of the sample was carried out on cellulose acetate membrane in Tris EDTA borate buffer at 8.9pH. Results: Compared with 557 patients diagnosed with high / indirect thalassemia, 10 patients were diagnosed with hemoglobin H during the study period. Of the ten patients, 4 (40%) were male and 6 (60%) were female. Patient’s ages ranged from 7 to 32 years old. The clinical picture of patients included anemia 100% and jaundice 10%. 90% of patients had a transfusion history. During the study, pallor was 100% present, the liver was palpable in 20% and the spleen in 80% of cases. The average hematological parameters were TRBC 4.5 (± 1.6), Hb 8.8 (± 2.2), MCV 71.9 (± 11.8), MCH 20.4 (± 2.9), and MCHC 25.7 (± 8.7). The mean reticulocyte count was 15.8% (± 9.4). Hemoglobin H inclusions were observed in 100% of cases. Conclusion: Hemoglobin H disease is an uncommon disorder and out of 557 Thalassemia major/Intermedia patients diagnosed during this period 10(1.7%) patients were diagnosed as having Hemoglobin H disease. Keywords: Hemoglobin H disease, α thalassemia, unstable hemoglobin.