coagulopathies Flashcards
name some hereditary primary bleeding disorders
- vWD
- qualitative platelet disorders (storage pool, Glanzmann, Bernard-Soulier)
- congenital thrombocytopenia
name some acquired primary bleeding disorders
Medications Uremia Thrombocytopenia Immune Non-immune Cardiac bypass Myeloproliferative d/o Acquired vWD
name some hereditary secondary bleeding disorders
Hemophilia
Rare coagulation factor deficiencies
Fibrinolytic defects
name some acquired secondary bleeding disorders
Liver disease DIC Vitamin K deficiency Acquired coagulation factor inhibitors Anticoagulation
acquired vessel wall bleeding disorders
- vitamin C deficiency
- vascular trauma
vWD inheritance pattern
autosomal dominant
two functions of vWF
- tethers platelets to collagen
- chaperones factor VIII
quantitative types of vWD
1 and 3
qualitative types of vWD
2
lab tests for vWD
coagulations screening
- PT and TCT normal
- aPTT possibly prolonged if factor VIII low
- VIII low
platelet screening
- prolonged PFA-100
- prolonged bleeding time
- abnormal aggregation with ristocetin
treatment for vWD
- DDAVP (for type 1 and some type 2)
- vWF replacement (cryoprecipitate, alphate)
- antifibrinolytic therapy
- birth control for menorrhagia
what is DDAVP and what does it do?
desmopressin, synthetic vasopressin
stimulates release of vWF and VIII from endothelial cells
when avoid DDAVP in treatment of vWD?
type 2B, exacerbates bleeding
deficiency in hemo A?
VIII
deficiency in hemo B?
IX
inheritance patterns of hemophilia
- A and B are x-linked recessive
- C, parahemophilia are autosomal recessive
deficiency of hemo C?
XI
deficiency of parahemophilia?
V
what is common in the female carrier state of hemophilia?
menorrhagia
what are considered mild, moderate and severe for hemophilia A and B?
mild = 5-40% of factor level
moderate = 1-5% of factor level
severe = <1% of factor level
normal factor level is 50-150%
expected labs in hemophilia A and B
- platelet function normal
- PT normal
- prolonged aPTT except possibly in mild
- aPTT will correct with mixing study
- low specific factor levels
bleeding manifestations in hemophilia?
- may be delayed because primary still happens
- bleeding into joints
- CNS
- bleeding after minor trauma
- hematuria
pathological issue with hemophilia
no thrombin burst with no IX or VIII
hemophilia treatment
- recombinant IX and VIII, replacing plasma derived
- DDAVP sometimes effective in mild hemophilia
- factors can be used prophylactically in severe hemophiliacs to keep factor level above 1%
complications of hemophilia
- chronic arthropathy
- infectious diseases
- development of antibodies against missing factor (might have to bypass using VIIa)
explain acquired hemophilia
- spontaneous autoantibody to factor VIII
- most are idiopathic
labs in acquired hemophilia
- platelets and PT normal
- aPTT prolonged
- aPTT will not correct with mixing study
- low factor VIII
treatment of acquired hemophilia
- low antibody titer, treat with factor VIII
- high antibody titer, treat with bypass agent (recombinant VII or FEIBA which includes II, VII, IX, X)
- eradication by immune suppression
characteristics of fibrinolytic bleeding
- delayed 12-24 hours
- mucocutaneous/oropharyngeal
- menorrhagia, GU
is hereditary or acquired fibrinolytic bleeding more common?
acquired
explain hereditary fibrinolytic bleeding
- autosomal recessive
- deficiency in alpha-2-antiplasmin or PAI-1, leaving more plasmin to break down fibrin
- over expression of plasminogen activator (Quebec platelet disorder)
labs in fibrinolytic bleeding
- PT and PTT may be prolonged because fibrin is being broken down
- hard to distinguish from DIC
- increased products of fibrin breakdown (d-dimers)
treatment for fibrinolytic bleeding
- plasma infusions
- anti-fibrinolytic drugs (if no DIC)
- Amicar
- tranexamic acid
what is the most common cause of bleeding due to vitamin K deficiency?
over anticoagulation with warfarin
causes of vitamin K deficiency
- dietary (malabsorption, alcoholism)
- medications
- coumarins (warfarin)
labs in vitamin K deficiency
- PT and aPTT prolonged (PT only in early deficiency)
- PT and aPTT will correct with mixing study
- platelets normal
- decreased II, VII, IX, X
- FACTOR V NORMAL!
treatment of vitamin K deficiency
- oral or IV vitamin K (corrects in hours)
- fresh frozen plasm (only in emergencies, corrects factors immediately, must be used with vitamin K)
- prothrombin complex concentrates (II, VII, IX, X)
what is preserved in liver failure?
factor VIII
what does liver disease look like for bleeding?
- both bleeders and clotters, but bleeding usually wins out
lab findings in coagulopathy of liver disease
- prolonged PT/INR and aPTT
- low factors in INCLUDING V, but VIII is still preserved
- PT/INR is unreliable
liver disease treatment
- transplant
- vitamin K and fresh frozen plasma can be temporary fixes
DIC - major relevant pathology for bleeding
- excessive thrombin, increases fibrin and activates platelets
- compensatory increase in APC, plasmin, and fibrinolysis
- increased activation of endothelium, more TF, more coagulation
labs in DIC
- prolonged PT/INR and aPTT
- elevated d-dimers
- low platelets
- low fibrinogen
- anemia
- RBC schistocytes
- low AT, APC, S
treatment for DIC
- treat underlying cause
- transfusion support
plasma (coagulation factors)
cryoprecipitate (fibrinogen)
platelets/RBC - heparin (only in active thrombosis)
