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[Hemoglobin C disease: report of 16 Tunisian cases]
Raouf Hafsia1, Olfa Marrakchi, Naouel Ben Salah
1Service d'Hématologie Biologique, Hôpital Aziza Othmana, Tunis.
Aim:
was to provide the clinical and biological patterns hemoglobine disease in Tunisia.
Methods:
This retrospective study collected to 16 cases of hemoglobin C disease : 6 homozygotic Hb C and 10 heterozygotic Hb C/beta-thalassemia.
Results:
The clinical profile is characterized by mild hemolytic anemia (Hb = 11.7 g/dl) associated with splenomegaly and hypersplenism. Contrary to homozygous state, the Hb C/beta-thalassemia is associated with microcytosis and pseudopolycythemia. The diagnosis is based on target cells, specific intraerythrocytic Hb C crystals in blood smear and Hb C level at 100%.
Conclusion:
The Hb C disease must be considered as a benign hemoglobinopathy which is associated with a long survival without major complications.
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