[Hemoglobin C disease: report of 16 Tunisian cases].
OBJECTIVE
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%.


CONCLUSIONS
The Hb C disease must be considered as a benign hemoglobinopathy which is associated with a long survival without major complications.
Service d'Hématologie Biologique, Hôpital Aziza Othmana, Tunis.
Raouf
R
Hafsia
Olfa
O
Marrakchi
Naouel
N
Ben Salah
Emna
E
Gouider
Ryhane
R
Ben Lakhal
Ramzi
R
Jeddi
Lamia
L
Aissaoui
Zaher
Z
Belhadjali
Hela
H
Ben Abid
Balkis
B
Meddeb
Aïcha
A
Hafsia
D000293:Adolescent; D000328:Adult; D005260:Female; D006444:Hemoglobin C / Q000032:analysis; D006445:Hemoglobin C Disease / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D006971:Hypersplenism / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013163:Splenomegaly / Q000209:etiology; D014416:Tunisia
D006444:Hemoglobin C