Clinico-hematologic profile of factor XIII-deficient patients.
A retrospective analysis of clinico-hematologic parameters of 18 factor XIII-deficient patients was carried out. The hematologic tests included activated partial thromboplastin time (APTT), prothrombin time (PT), and clot solubility. Laboratory diagnosis of FXIII deficiency was made where bleeding time, PT, APTT, and thrombin time were normal and the clot solubility test result with 5M urea was positive. Factor XIII level with family screening was performed using commercially available kits. History of prolonged bleeding from the umbilical stump was present in four (22.2%) patients. The most common site of bleeding was the skin (11 of 18 patients). Three patients were given prophylaxis (FFP in two, factor XIII in one). A high prevalence of recurrent abortion in female patients with FXIII deficiency (two of the three patients in this study) was observed.
Bhattacharya
M
M
Department of Haematology, All India Institute of Medical Sciences, New Delhi, India.
Biswas
A
A
Ahmed
R P H
RP
Kannan
M
M
Gupta
M
M
Mahapatra
M
M
Choudhry
V P
VP
Saxena
R
R
D000026:Abortion, Habitual; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004198:Disease Susceptibility; D005177:Factor XIII Deficiency / Q000097:blood* / Q000150:complications / Q000503:physiopathology*; D005260:Female; D006470:Hemorrhage; D006801:Humans; D007223:Infant; D008297:Male; D010314:Partial Thromboplastin Time; D010375:Pedigree; D011517:Prothrombin Time