Factor XI deficiency in Iranians: Its clinical manifestations in comparison with those of classic hemophilia

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

Background and Objectives. In patients with factor XI (FXI) deficiency the bleeding tendency is poorly correlated with plasma factor levels. The purpose of this study was to evaluate whether or not this discrepancy is also present in a large series of patients from Iran, a previously unexplored ethnic group. Design and Methods. In 28 FXI - deficient patients bleeding symptoms and their relation to FXI levels were compared with those of 100 patients with factor VIII (FVIII) deficiency (classic hemophilia), matched for severity of factor deficiency. Results. Spontaneous bleeding was definitely less frequent in FXI deficiency than in hemophilia, whereas post-operative and post-traumatic bleeding occurred with comparable frequencies. Among FXI-deficient patients the severity of symptoms was poorly correlated with FXI levels, mildly deficient patients bleeding almost as frequently as those severely deficient. In contrast, in patients with classic hemophilia there was a close relation between the severity of bleeding and degree of FVIII deficiency. Interpretation and Conclusions. As in other ethnic groups, in Iranians factor XI deficiency is less severe than classic hemophilia and the bleeding tendency is poorly correlated to plasma factor levels.

Original languageEnglish
Pages (from-to)512-514
Number of pages3
JournalHaematologica
Volume87
Issue number5
Publication statusPublished - 2002

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Factor XI Deficiency
Hemophilia A
Factor XI
Hemorrhage
Ethnic Groups
Iran

Keywords

  • Factor XI deficiency
  • Hepatitis B
  • Hepatitis C

ASJC Scopus subject areas

  • Hematology

Cite this

Factor XI deficiency in Iranians : Its clinical manifestations in comparison with those of classic hemophilia. / Peyvandi, Flora; Lak, Manijeh; Mannucci, Pier Mannuccio.

In: Haematologica, Vol. 87, No. 5, 2002, p. 512-514.

Research output: Contribution to journalArticle

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N2 - Background and Objectives. In patients with factor XI (FXI) deficiency the bleeding tendency is poorly correlated with plasma factor levels. The purpose of this study was to evaluate whether or not this discrepancy is also present in a large series of patients from Iran, a previously unexplored ethnic group. Design and Methods. In 28 FXI - deficient patients bleeding symptoms and their relation to FXI levels were compared with those of 100 patients with factor VIII (FVIII) deficiency (classic hemophilia), matched for severity of factor deficiency. Results. Spontaneous bleeding was definitely less frequent in FXI deficiency than in hemophilia, whereas post-operative and post-traumatic bleeding occurred with comparable frequencies. Among FXI-deficient patients the severity of symptoms was poorly correlated with FXI levels, mildly deficient patients bleeding almost as frequently as those severely deficient. In contrast, in patients with classic hemophilia there was a close relation between the severity of bleeding and degree of FVIII deficiency. Interpretation and Conclusions. As in other ethnic groups, in Iranians factor XI deficiency is less severe than classic hemophilia and the bleeding tendency is poorly correlated to plasma factor levels.

AB - Background and Objectives. In patients with factor XI (FXI) deficiency the bleeding tendency is poorly correlated with plasma factor levels. The purpose of this study was to evaluate whether or not this discrepancy is also present in a large series of patients from Iran, a previously unexplored ethnic group. Design and Methods. In 28 FXI - deficient patients bleeding symptoms and their relation to FXI levels were compared with those of 100 patients with factor VIII (FVIII) deficiency (classic hemophilia), matched for severity of factor deficiency. Results. Spontaneous bleeding was definitely less frequent in FXI deficiency than in hemophilia, whereas post-operative and post-traumatic bleeding occurred with comparable frequencies. Among FXI-deficient patients the severity of symptoms was poorly correlated with FXI levels, mildly deficient patients bleeding almost as frequently as those severely deficient. In contrast, in patients with classic hemophilia there was a close relation between the severity of bleeding and degree of FVIII deficiency. Interpretation and Conclusions. As in other ethnic groups, in Iranians factor XI deficiency is less severe than classic hemophilia and the bleeding tendency is poorly correlated to plasma factor levels.

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