Bleeding Disorders

BLEEDING DISORDERS

HISTORYIS KEY—ASK ABOUT BRUISING, PROBLEMS WITH CIRCUMCISION, BLEEDING INTO JOINT SPACES, Fhx, ECT…

PROBABLY DO NOT NEED TO TEST IF HISTORY IS NL

GET PTS OFF OF THEIR ASA FOR AT LEAST 10 DAYS PREOP—THIS REALLY DOES MATTER—-ALSO NSAIDS–MIGHT AS WELL KEEP THEM OFF OF THESE AS WELL

PT

(EXTRINSIC PATHWAY)—-THINK OF WARFARIN/LIVER PROBLEMS ECT…

FOR REVERSAL VIT K CAN BE GIVEN (FAT SOLUBLE)—-FACTORS 2,7,9,10 ARE THE VIT K DEP FACTORS—FACTOR 7 IS THE LIMITING FACTOR—-T1/2 = 6 HOURS—-AFFFECTED BY ANYTHING THAT ALTERS HEPATIC METABOLISM

PTT

(INTRINSIC PATHWAY—-ACCELERATES THE ACTIVITY OF ANTITHROMBIN III (HEPARIN COFACTOR) IN BINDING TO THROMBIN

PRIMARY REASON IT IS ELEVATED IS PROBABLY A CIRCULATING “LUPUS” ANTICOAGULANT FROM A RECENT URTI—–NO CONSEQUENCE—CAN DO A “MIX”TEST TO CONFIRM—MIX PTS BLOOD WITH NL DONOR BLOOD–CORRECTS FACTOR DEFICIENCY

FOR REVERSAL PROTAMINE SULFATE CAN BE GIVEN—TOO MUCH PROTAMINE CAN BE COAGULOPATHIC

BEWARE OF HEPARIN ANTIBODIES—-THROMBOCYTOPENIA-HYPERCOAGULABLE STATES

BLEEDING TIME

—REALLY WHERE THE RUBBER MEETS THE ROAD?

NOT NECESSARILY WHAT YOU SEE IN THE OR

PROLONGED IN VWDz AND OTHER PLT DISORDERS (ASA, UREMIA, THROMBOCYTOPENIA, ANTIPLATELET AB’S, TICLOPIDINE)

THROMBIN TIME

(FACTOR 1 FIBRINOGEN LEVELS)

PLATELET COUNT

—NL IS 100-400 (<40 = THROMBOCYTOPENIA)—START LOOKING FOR CAUSE—ANTI-PLT ANTIBODIES, DRUGS, BM SUPPRESSION ECT…

ENTITIES:

VWDz =

MOST COMMON INHERETED BLEEDING DISORDER (PREVELENCE = 0.8%)

VWF CIRCULATES IN THE PLASMA AS A COMPLEX WITH FACTOR VIII—-GENERALLY FACALITATES THE EXISTENCE AND FUNCTION OF FACTOR VIII

THREE TYPES OF VWDz

TYPE I -75%—AUTO D —-PARTIAL QUANTITATIVE DEFECT—-RESPONDS TO DDAVP

TYPE II-AUTO D OR R—QUALITATIVE DEFECT—-PARTIAL RESPONSE TO DDAVP

TYPE III–AUTO R—ABSENCE OF THE VWF—NO RESPONSE TO DDAVP

WILL HAVE ABNL PTT IF LOW FACTOR VIII

BLEEDING TIME IS OFTEN ELEVATED

VWF ANTIGEN MEASUREMENT—-GIVES YOU A CLUE

RISTOCETIN COFACTOR ACTIVITY—THE MOST SENSITIVE TEST—EXPLORES THE ABILITY OF VWF TO BIND PLATELETS

Rx: DDAVP-

–ENHANCES THE RELEASE OF VWF FROM ENDOTHELIAL CELLS

CAN LEAD TO H2O RETENTION—HYPONATREMIA

FACTOR VIII/VWF CONCENTRATE–LASTS ABOUT A WEEK

 

FACTOR VIII DEFICIENCY = HEMOPHILIA A

X- LINKED RECESSIVE (1/10,000)

LONG PTT AND LOW FACTOR VIII WITH NL VWF

Rx: TRY DDAVP—-MOST HAVE TO USE CONCENTRATES

FACTOR IX DEFICIENCY (HEMOPHILIA B, CHRISTMAS Dz)

1/100,000

SAME DX AND RX TO HEMOPHILIA A

HIGH DOSES (>400 IU) VIT E AND FISH OILS CAN ALSO INCREASE YOUR BLEEDING TIME

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