Disorders of Secondary Hemostasis Flashcards
von Willebrand Disease
Most common inherited bleeding disorder
Symptoms similar to platelet disorder, mild to severe
Weibel Palade bodies store vWF
vWF Disease Therapies
Cryoprecipitate: concetrated factors I, VIII, XIII, and vWF
Deamino-D-arginine vasopressin (desmopressin)
Concetrated immediate purity VIII
Factor I Deficiency: Quantitative
Type I
Afibrinogenemia: 23% of cases, no fibrinogen, other labs all increased, negative D-dimer test
Hypofibrinogenemia: 26% of cases, less severe, recurrent pregnancy loss, TT is prolonged
Factor I Deficiency: Qualitative
Type II
Dysfibrinogenemia: 51% of cases, fibrinogen levels are normal
PT, PTT, TT prolonged
Therapy: cryoprecipitate or fibrinogen concentrate as needed
Factor II Deficiency
PT and PTT prolonged
Treat with prothrombin complex concentrate (PCC)
Factor V Deficiency
AKA parahemophilia
PT and PTT prolonged
Diagnose with Factor V assay
Treat with FFP
Factor VII Deficiency
Prolonged PT with normal PTT
Treat with recombinant factor VIIa or plasma derived factor VII concentrate
Factor VIII Deficiency
Hemophilia A
Second most common inherited bleeding disorder (85% of cases)
X linked recessive
PTT prolonged
Factor IX Deficiency
Hemophilia B or Christmas disease
Clinical symptoms identical to Hemophilia A
Factor X Deficiency
PT, PTT, and RVVT prolonged
Some genetic variants only have prolonged PT (3) or PTT (1)
Treat with FFP or PCC
Acquired is associated with systemic amyloidosis
Factor XI Deficiency
Hemophilia C
More common in Ashkenazi Jews
PTT prolonged
Treat with FFP or low dose rVIIa
Factor XII Deficiency
No history of bleeding
Prolonged PTT and normal PT
Needs to be distinguished from VIII, IX, XI, HK, and PK deficiencies by measuring factor levels
No therapy needed
Factor XIII Deficiency
Umbilical cord bleeding or excess bleeding after circumcision
Screen tests are normal
Measure factor XIII levels and functionality
Treat with factor XIII concentrate
DIC
Acquired, uncontrolled, and inappropriate formation and lysis of fibrin
CLotting factors are consumed by circulating thrombin activation (clotting and bleeding at same time)
High levels of plasmin activation leads to excess formation of FDPs
PT, PTT, TT prolonged
PLT and fibrinogen decreased
D-dimer increased
Treat by removing underlying causes, LMWH, tranfusions
Primary Fibrinolysis
Similar to DIC, caused by excessive activation of plasminogen, thrombin activation is absent
PT, PTT, TT prolonged
D-dimer normal
Treat with epsilon aminocaproic acid or tranexamic acid
