bleeding disorders

BLEEDING DISORDERS

Dr.Abdulhak A. Alnuemi FICMS,CABM

Injury : * vess.wall cont * plat adhesion&aggregation * fibrin clot

Bleeding tendency likely in

Bleeding from 2 sites simultaneously spontaneous Bleeding is prolonged..hours following tooth extraction, minor surgery, childbirth, menorrahgia BL. that needs transfusion Family hx of bleeding from multiple sites(absence does no exclude--)

Types of bleeding lesions

Petechiae --pinpoint bleeds into dermis due to cap.leak-----platelet disordersPurpura—collection of blood of few mm in the skin---palpable?Ecchymosis--subcutaneous collection due to leak from arteriols&veniols--few cms—(bruises)Hematomas--deeper &palpable collection--platelet or coagulation disordersHemarthrosis--coagulation factor disorder-almost always factor VIII or IX def

Primary vs secodary hemostatic defect

Skin &mucous mem.-----usually pr Immediately after trauma----pr Readily controlled by pressure &local measures----pr petechiae&ecchymosis Vs hematomas&hemarthrosis Family history Aut.Dom.in platelet dysfunction . Aut. or X-linked Rec. in case of coagulation disorders

Platelates

The platelet(150-450 th/mm) contains lysosomes, granules, and trilaminar plasma membrane, microtubules. Granules are key in primary hemostasis and contain ADP, Thromboxane, platelet factor 4, adhesive and aggregation glycoproteins, coagulation factors, and fibrinolytic inhibitors

The Coagulation Cascade

Common Pathway

Laboratory Evaluation of the Coagulation Pathways

Partial thromboplastin time (PTT)
Prothrombin time (PT)
Intrinsic pathway
Extrinsic pathway
Common pathway
Thrombin time
Fibrin clot

A word on clotting factors

Vitamin K Dependent Factors : 1972Intrinsic Pathway : IX, XCommon Pathway: IIExtrinsic Pathway: VIIAll clotting Factors are produced in liver except vWFSites of heparin activity :Xa…inhibition is the major siteWarfarin act by depleting vit K depending factors

Testing the hemostatic system

Platelet count Thrombocytopenia and bleeding: Less than 50,000 Spontaneous bleeding possible: Less than 20,000 Count does not have anything to do with functionality of platelet


Testing the hemostatic system
Bleeding time(BT) Tests vascular integrity and platelet function Normal < 8 minutes Affected by Aspirin (permanent) and NSAIDs When BT prolonged with platelet count over 100,000; this suggests platelet dysfunction

Testing the hemostatic system

Prothrombin Time Test of extrinsic and common pathways Average normal 12-14 sec Used for monitoring Warfarin therapy Elevated in patients with liver disease.

Testing the hemostatic system

Partial Thromboplastin Time (PTT) Tests intrinsic and common pathway Average normal 25-29 sec Used for monitoring heparin therapy

Bleeding tendency due to vess. Wall abnomality..examples

Hereditary Hemorrhagic Telangectasia Ehler-Danlos , Pseudoxanthom elasticum Senile purpura Infection: measles ,Meningococcemia Long Steroid treatment Scurvy

Thrombocytopenia

Three mechanisms of Thrombocytopenia Decreased production Due to suppression as by cytotoxic chemotherapy, or effects of alcohol or thiazides Splenic Sequesteration And increased Splenic destruction Increased Destruction as in ITP


ITP
Diagnosis of exclusion,mucocut.bleeding Associated with IgG anti-GP IIb-IIIa Platelet count usually falls to less that 20,000 Acute Form Most common in children 2 to 6 years Viral Prodrome(URTI) common, 3 weeks prior Self Limited and > 90% remission rate Teatment: Prednisolon 2mg/kg/d for sever purpura or epistaxis & count less 10,000



ITP
Chronic FormAdult disease primarily Women more often than menInsidious onset with no prodrome6 months or more of Symptoms ,include: easy bruising, prolonged menses, mucosal bleedingTeatment: Prednisolon 1mg/kg/d is the usual treatmentRelapses usually treated with increasing dose of prednisolone if 2 relapses…splenectomy(curative in70%)

von Willebrand’s Disease (vWD) autosomal dominant patients range from asymptomatic to spontaneous bleeding similar to a severe hemophiliac characterized by mucocutaneous bleeding, BT prolonged, aPTT usually prolonged Treatment options: desmopressin, Intermediate purity F VIII, Cryoprecipitate, Amino caproic acid & Tranexamic acid

Hemophilia A and B

Hemophilia A Hemophilia B factor deficiency Factor VIII Factor IX Inheritance X-linked X-linked recessive recessive Incidence 1/10,000 males 1/50,000 males Severity Related to factor level <1% - Severe - spontaneous bleeding 1-5% - Moderate - bleeding with mild injury 5-20(50) % - Mild - bleeding with surgery or trauma *In sever Hemophilia patient may sustain 1-2 bleeds/week *Normal FVIII level is 50-150% Complications shortening tendon, muscle wasting, j.destruction

Hemophilia

X-linked Rec Clinical manifestations (Hemophilia A & B indistinguishable) usually after 6 mo of life Hemarthrosis (most common),Soft tissue hematomas (e.g., muscle),Muscle atrophy&Shortened tendons Other sites of bleeding Urinary tract , CNS, neck (may be life-threatening-closed space..) & Prolonged bleeding after surgery or dental extractions Treatment options : Recombinant factor VIII (on need) and Prophylactic Home Treatment to keep FVIII level above 1%. Other therapies: Amino caproic acid ,Tranexamic acid (both are antifibrinolytic agents)Rest &splinting of affected joint will decrease continuing bleeding there after mobilization &physiotherapy

Summary Hemostatic Disorders

BTPltPTPTT Vascular Dis -  - - - PLT Disorder -  -  - -Factor 8/9 *Congenital - - -  Vit K / Liver*Acquired - -  - Combined (DIC)   - 