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:: Volume 13, Issue 4 (Winter 2016) ::
Sci J Iran Blood Transfus Organ 2016, 13(4): 348-364 Back to browse issues page
A practical approach in laboratory diagnosis of Factor XIII deficiency
A. Dorgalaleh , Sh. Tabibian , Y. Farshi , M.S. Hosseini , M. Shams , B. Tavasoli , M. Shakouri , F. Roshan zamir
Abstract:   (1081 Views)

Abstract

Background and Objectives

Factor XIII (FXIII) deficiency is an extremely Rare Bleeding Disorder (RBD) with estimated incidence of 1 per 2 million in the general population. All routine coagulation tests including Bleeding Time (BT), Prothrombin Time (PT), Activated Partial Thromboplastin Time (APTT) and platelet count are normal in FXIII deficiency (FXIIID) and this makes the diagnosis of disorder difficult. In this study, we presented a practical approach for laboratory diagnosis of FXIIID.

Materials and Methods

For this review article, all relevant articles were searched using appropriate keywords.

Results

Clot solubility test is the most common diagnostic test of FXIIID that has low specificity and sensitivity and also can be affected by several factors including clotting and solubilizing agents. Although this test is not further recommended by experts for diagnosis of FXIID but due to expanded use of this method, it cannot be abolished and we can use it to screen the disease with some modifications. FXIII activity assay should be considered as a screening test of FXIIID. FXIII activity is performed by several methods among them photometric assay is the most common. Without a serum blank, photometric assay overestimates FXIII activity that in patients with severe FXIIID can be accompanied by life threatening bleeding.

Conclusions

FXIIID is a rare disease with lethal outcomes. A best clinical examination, family history as well as a suitable laboratory approach led to timely diagnosis of FXIIID and prevented misdiagnosis of the disease and the arising consequences. Thus, this study is to give a proper laboratory approach for diagnosis of FXIIID.

Keywords: Key words: Factor XIII Deficiency, Bleeding, Laboratory Diagnosis
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Type of Study: Review Article | Subject: Hematology
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Dorgalaleh A, Tabibian S, Farshi Y, Hosseini M, Shams M, Tavasoli B, et al . A practical approach in laboratory diagnosis of Factor XIII deficiency. Sci J Iran Blood Transfus Organ. 2016; 13 (4) :348-364
URL: http://bloodjournal.ir/article-1-963-en.html
Volume 13, Issue 4 (Winter 2016) Back to browse issues page
فصلنامه پژوهشی خون Scientific Journal of Iran Blood Transfus Organ
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