| |
| |
| phenotype |
| The
combined use of the global coagulation tests prothrombin time
(PT) and activated partial thromboplastin time (APTT) is usually
used to identify RBDs of clinically significant severity, but
not FXIII deficiency (Table 2).
|
|
| Specific
assays for each different coagulation factor are necessary to
determine the level of deficiency (severe, moderate, mild). |
| |
| Immunoassays
to measure the conserved antigen levels are not strictly necessary
for diagnosis and treatment but are necessary to distinguish
type I from type II deficiencies1. |
| |
| Most
RBDs are expressed phenotypically by a parallel reduction of
plasma factors measured by functional and immuno-assays (so-called
type I deficiencies). |
| |
| Qualitative
defects, characterized by normal, slightly reduced, or increased
levels of antigen levels contrasting with much lower or undetectable
functional activity (type II), are less frequent -2,-
3- |
| |
|
| |
|
|
