English abstract
Objective: Published data indicate that Alpha thalassemia trait is prevalent in 45% of population of Sultanate of Oman. Recent unpublished data suggest that this prevalence is higher than 45%.
Yet clinical suspicion or investigations into α-thalassemias arelacking. Moreover, Hemoglobin H disease is considered rare in Oman. We decided, therefore to look for Hemoglobin H disease
and characterize the clinico–hematopathological features of the disease.
Methods: Patient demographics, clinical details and detailed
hematology parametry of Hemoglobin H disease cases, diagnosed
by Department of Laboratory over a period of 5 years between
February 2002 and January 2007 in patients presenting at AlNahdha Hospital and Genetic counseling unit in Muscat were
compiled from hospital and laboratory records and analyzed.
Results: Twenty cases of Hemoglobin H disease in Omanis
were diagnosed mainly during the second decade. 60% belonged
to Al-Balushi tribe. 40% of cases presented with body pains.
35% presented with nonspecific symptoms. 50% of cases were
erroneously labeled as Iron deficiency anemia. Microcytic
erythrocytosis, high Red Cell Distribution Width, numerous
misshapen Red Blood Cells, pseudothrombocytosis, low A2 and
normal Ferritin were important diagnostic clues. Hemoglobin H
inclusions in special reticulocyte smears and Hemoglobin H on
HPLC or Electrophoresis were diagnostic.
Conclusion: Hemoglobin H disease is common in Oman. The
need to do HPLC, G6PD activity and Ferritin studies in all cases
of anemia in Oman to avoid missing diagnosis of Hemoglobin
H disease is stressed. This study is intended to create awareness
about Hemoglobin H disease in order to diagnose early, treat
rightly, counsel correctly and pave the path for prevention of αthalassemia disease in Oman.
