Use of purified fibrinogen concentrate for dysfibrinogenemia and importance of laboratory fibrinogen activity measurement

We report a patient with dysfibrinogenemia treated with purified fibrinogen concentrate who had discrepant post‐treatment laboratory values. The patient had mild bleeding symptoms and was diagnosed with dysfibrinogenemia based on fibrinogen activity of 51 mg/dl and antigen of 240 mg/dl. He was treat...

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Bibliographic Details
Published in:Pediatric blood & cancer 2013-03, Vol.60 (3), p.500-502
Main Authors: Franzblau, Emily B., Punzalan, Rowena C., Friedman, Kenneth D., Roy, Angshumoy, Bilen, Ozlem, Flood, Veronica H.
Format: Article
Language:English
Subjects:
Adolescent
Afibrinogenemia - drug therapy
dysfibrinogenemia
fibrinogen
Fibrinogen - analysis
Fibrinogen - metabolism
Fibrinogen - therapeutic use
genetics
Hematologic Tests - standards
Hematology
hemorrhagic disorders
Humans
Laboratories
Laboratories, Hospital
Male
Oncology
Pediatrics
thrombotic disorders
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Summary:We report a patient with dysfibrinogenemia treated with purified fibrinogen concentrate who had discrepant post‐treatment laboratory values. The patient had mild bleeding symptoms and was diagnosed with dysfibrinogenemia based on fibrinogen activity of 51 mg/dl and antigen of 240 mg/dl. He was treated for an adenoidectomy with purified fibrinogen concentrate (RiaSTAP®) at a dose of 70 mg/kg. A discrepancy in post‐treatment fibrinogen activity was observed between the hospital and reference laboratories. Investigation revealed differences in laboratory assay and calibration methods. Fibrinogen concentrate may be a treatment option for patients with dysfibrinogenemia, but accurate laboratory technique is critical for fibrinogen measurement. Pediatr Blood Cancer 2013; 60: 500–502. © 2012 Wiley Periodicals, Inc.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.24383