Abstract
Fibrinogen Longmont: A Clinically Heterogeneous Dysfibrinogenemia with Discrepant Fibrinogen Results Influenced by Clot Detection Method and Reagent.
Highlights
The initial provisional diagnosis was an evolving malignancy-associated disseminated intravascular coagulation
We report two adult siblings (►Table 1) diagnosed with the fibrinogen Longmont variant after an unmeasurable Clauss fibrinogen (FibC) was found
activated partial thromboplastin time (APTT) and prothrombin time (PT) were within reference range on the optical endpoint ACL TOP (Instrumentation Laboratory, Massachusetts)
Summary
The initial provisional diagnosis was an evolving malignancy-associated disseminated intravascular coagulation. APTT and PT were within reference range on the optical endpoint ACL TOP (Instrumentation Laboratory, Massachusetts). PT-derived fibrinogen (FibD) was mildly reduced; FibC was unmeasurable using bovine HemosIL QFA Thrombin on the ACL TOP. FibC is the most accurate clot-based assay to determine functional levels of fibrinogen, compared to FibD, which is an indirect measurement.[1] The thrombin clotting time (TCT) was mildly prolonged with a normal Reptilase time.
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