Abstract
Glanzmann thrombasthenia (GT) is a rare autosomal recessive disease characterized by prolonged bleeding time with normal platelet count and morphology. It is caused by the quantitative or qualitative deficiency of the platelet glycoprotein IIb-IIIa. In 382 Iranian patients with GT diagnosed at a single center during the period 1969-2001, consanguinity between parents was 86.6%, in accord with the high frequency of intrafamilial marriages in Iran. Almost all patients had had abnormal mucocutaneous bleeding (epistaxis and gum bleeding); at follow-up, 4/5 of the patients had been transfused at least once to control hemorrhagic episodes. As expected, almost all the patients had a normal platelet count while the leukocyte count was increased in 19.3%. Among women, an unexpected low rate of pregnancies was observed.
| Original language | English |
|---|---|
| Pages (from-to) | 198-199 |
| Number of pages | 2 |
| Journal | American Journal of Hematology |
| Volume | 77 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - Oct 2004 |
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Keywords
- Consanguineous marriage
- Genetic consultation
- Glanzmann thrombasthenia
ASJC Scopus subject areas
- Hematology
Cite this
Presentation and pattern of symptoms in 382 patients with glanzmann thrombasthenia in Iran. / Toogeh, G.; Sharifian, R.; Lak, M.; Safaee, R.; Artoni, A.; Peyvandi, Flora.
In: American Journal of Hematology, Vol. 77, No. 2, 10.2004, p. 198-199.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Presentation and pattern of symptoms in 382 patients with glanzmann thrombasthenia in Iran
AU - Toogeh, G.
AU - Sharifian, R.
AU - Lak, M.
AU - Safaee, R.
AU - Artoni, A.
AU - Peyvandi, Flora
PY - 2004/10
Y1 - 2004/10
N2 - Glanzmann thrombasthenia (GT) is a rare autosomal recessive disease characterized by prolonged bleeding time with normal platelet count and morphology. It is caused by the quantitative or qualitative deficiency of the platelet glycoprotein IIb-IIIa. In 382 Iranian patients with GT diagnosed at a single center during the period 1969-2001, consanguinity between parents was 86.6%, in accord with the high frequency of intrafamilial marriages in Iran. Almost all patients had had abnormal mucocutaneous bleeding (epistaxis and gum bleeding); at follow-up, 4/5 of the patients had been transfused at least once to control hemorrhagic episodes. As expected, almost all the patients had a normal platelet count while the leukocyte count was increased in 19.3%. Among women, an unexpected low rate of pregnancies was observed.
AB - Glanzmann thrombasthenia (GT) is a rare autosomal recessive disease characterized by prolonged bleeding time with normal platelet count and morphology. It is caused by the quantitative or qualitative deficiency of the platelet glycoprotein IIb-IIIa. In 382 Iranian patients with GT diagnosed at a single center during the period 1969-2001, consanguinity between parents was 86.6%, in accord with the high frequency of intrafamilial marriages in Iran. Almost all patients had had abnormal mucocutaneous bleeding (epistaxis and gum bleeding); at follow-up, 4/5 of the patients had been transfused at least once to control hemorrhagic episodes. As expected, almost all the patients had a normal platelet count while the leukocyte count was increased in 19.3%. Among women, an unexpected low rate of pregnancies was observed.
KW - Consanguineous marriage
KW - Genetic consultation
KW - Glanzmann thrombasthenia
UR - http://www.scopus.com/inward/record.url?scp=4644247875&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=4644247875&partnerID=8YFLogxK
U2 - 10.1002/ajh.20159
DO - 10.1002/ajh.20159
M3 - Article
C2 - 15389911
AN - SCOPUS:4644247875
VL - 77
SP - 198
EP - 199
JO - American Journal of Hematology
JF - American Journal of Hematology
SN - 0361-8609
IS - 2
ER -