RT Book, Section
A1 Lichtman, Marshall A.
A1 Kaushansky, Kenneth
A1 Prchal, Josef T.
A1 Levi, Marcel M.
A1 Burns, Linda J.
A1 Armitage, James O.
SR Print(0)
ID 1133366048
T1 Hereditary Disorders of Fibrinogen
T2 Williams Manual of Hematology, 9e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9781259642470
LK hemonc.mhmedical.com/content.aspx?aid=1133366048
RD 2024/04/19
AB Quantitative  disorders  of  fibrinogen  may  be  afibrinogenemia  or  hypofibrinogenemia,  depending  on  the  severity.Normal  fibrinogen  levels  range  from  150  to  350  mg/dL.  In  afibrinogenemia,  the  fibrinogen  concentration  is  less  than  20  mg/dL.  In  hypofibrinogenemia,  the  level  is  less  than  normal.Approximately  100  distinct  mutations  have  been  identified  in  patients  with  afibrinogenemia  (in  homozygosity  or  in  compound  heterozygosity)  or  in  hypofibrinogenemia.  Causative  mutations  can  be  divided  into  two  main  classes:  null  mutations  with  no  protein  production  at  all  and  mutations  producing  abnormal  protein  chains  that  are  retained  inside  the  cell.