Bleeding Dissorder

 

HOW TO APPROACH APPROACH TO A PATIENT WITH WITH BLEEDING DISORDERS 

EVALUATION OF THE BLEEDING PATIENT 

DEFINITION •

Hemostasis The arrest of bleeding from an injured blood vessel, involving the combined regulatory activity of vascular, platelet, and plasma factors

Primary Hemostasis    

Vessel wall function Platelet functions of Adhesion, Aggregation

Secondary hemostasis  

Plasma coagulation factors leading to fibrin deposition ,clot formation Tertiary hemostasis    

•

Clot retraction Cross link of fibrin clot Fibrinolysis

Clotting The Coagulation of blood is a complex process by which fluid form of blood changes to semi solid or solid form.

•

Bleeding Eta!asation of blood from an ruptured blood !essel to

 

the internal or eternal tissue"

HEMOSTASIS AND THROMBOSIS 

•

#ependent on $ factors%



&ascular endothelium Platelets Coagulation system

 

1. CLINICAL ASPECTS OF BLEEDING

 

'" C()*)C+( +SPECTS F B(EE# B(EE#)*)*E!aluation of patients .ith bleeding is a multi/step process% • Complete history •

#etailed physical eam

•

(aboratory e!aluation

HISTORY

 



)s there a personal or famil !is"or of !is"or  of bleeding after surgical procedures, dental procedures, childbirth, childbirth, or trauma0



#!en the #!en  the bleeding episode started0



Has the patient recei!ed me$i%a"ions that can cause or make .orse a bleeding problem0

 

 

1any drugs can contribute to bleeding2 semisy semisynthe nthetic tic penicillins penicillins cephalosp cephalosporins orins calcium channel blocker dipyridamole thia3ides alcohol chlorproma3ine, &'inine( &'ini$ine  &'ini$ine  sulfonamides )*H, rifampin methyldopa phenytoin, barbiturates, .arfarin, heparin, thrombolytic agents *S+)#s, +S+ allopurinol T)P*S)+

PH4S)C+( E5+1 '" +ssess !olume status status 6correct shock if present7

8" (ook for hepatosplenomegaly $" #o a rectal eam for e!idence of -) bleeding 9" Eamine oropharyn for e!idence of petechiae

 

HEREDIATARY TELANGECTASIAS •    

1outh% -um Bleed -um Hypertrophy

  

Telangectasias +ngular stomatitis

  S,BCON-,NCTIAL HAE)ORRHAGE 

PH4S)C+( E5+1 





(ook for physical signs and symptoms of diseases related to %apillar fra/ili"0  fra/ili"0  Cushing:s syndrome, 1arfan syndrome or eogenous steroids ;senile purpura<

  



Petechiae secondary to coughing, snee3ing, &alsal!a maneu!er, blood pressure measurement !asculitis 6;palpable purpura;7

 



Telangiectasias 6sler/=eber/>endu syndrome7 6HHT7

PETECHIAE

&+SC?()T)S 6P+(P+B(E >+SH7

8" HE1+T(-)C #)S> #)S>#E>S #E>S C+?S)*- B(EE#)*-

 –

Platelet disorders

 –

Coagulation factor dis disorders orders

C()*)C+( #)FFE>E*T)+T)*

 

P(+TE(ET #EFECT &S  C+-?(+T)* #EFECT  P(+TE(ETS #EFECTS •

-ene -enera rally lly ha ha!e !e immed immedia iate te on onse sett of of b ble leed edin ing ga aft fter er trau trauma ma

•

Blee Bleedi ding ng is pred predom omin inan antl tly y in in sk skin in,, muc mucou ous sm mem embr bran anes es,, nose, -) tract, and urinary tract

•

Blee Bleedi ding ng ma may yb be e obs obser er!e !ed d as as p pet etec echi hiae ae 6@ 6@$ $ mm7 mm7 or or ecchymoses 6A$ mm

C1P+>)S* F P(+TE(ET +*# C+-?(+4)* #)S>#E>S  

Pl Plat atel elet et Diso Disord rder erss

Site of bleeding

Physical Fin Finding

Deep in soft tissues (joints Skin, mucous membrane and soft and muscles) tissue Petechiae, ecc ecchymoses Hematoma, Hem Hemarthrosis

Family History

Autosomal dominant

Autosomal or !linked

#leeding after cuts and scratches

$es

recessi"e %o

#leeding after surgery and trauma

&mmediate, usually mild

Delayed ('! days), often se"ere

Coagu oagula lati tion on Fac acto torr Disorder

 

C()*)C+( +SPECTS F B(EE#)*-

C+-?(+T)* #EFECTS •

;#eep; bleeding 6in the oint spaces, muscles, and retroperitoneal spaces7 is common" bser!ed on eam as hematomas and hemarthroses"

Hematoma

 

LABORATORY EAL,ATION OF BLEEDING CBC and smear Platelet count Thrombocytopenia RBC a an nd pl platelet mo morphology TTP, D DIIC, e ettc.

Coagulation pathways

PT

extrinsic/common

PTT

Intrinsic/ Intrinsic/common common pathways

Coag. factor assays

Specific factor deficiencie deficiencies s

(+B>+T>4 E&+(?+T)* F B(EE#)*50:50 mix Inhibitors (e.g., antibodies) Fibrinogen assayvon WilDecreased fibrinogen Platelet function lebrand fac tor vWD Bleeding time In vivo  test  test (non-specific) Thrombin time Qualitative Qualitative/quantitativ /quantitative e fibrinogen Plat Pl atel elet et fu func ncti tion on anal analyz yzer er (PFA (PFA)) Qual Qualit itat ativ ive e pla plate tele lett defects disorders D-dimer

Fibrinolys Fibrinolysis is (DIC)

BLEEDING TIME

• 5-10 -10% of pat atie ien nts hospi ospittal aliz ize ed pati tie ents nts ha hav ve a prolonged bleeding time • Mo Most st of tthe he pr prol olon onge ged d ble bleed edin ing g ti time mes s ar are ed due ue to as aspi piri rin no orr d dru rug g ingestion •

Pr Prol olon onge ged d bl blee eedi ding ng ti time me does does not not pre predi dict ct ex exce cess ss su surg rgic ical al bloo blood d loss loss

•

No Nott rrec ecom omm mende ended d for for ro rout utin ine e tes testi ting ng in pr preo eope pera rati tive ve patie atient nts s

 

THROMBIN TIME •

Measu sure res s rrat ate eo off ffib ibri rin nogen conve onverrsion ion tto o ffib ibrrin

•  –  –

Procthro edu re: with patient plasma Add thrombin mbin Measure ttime ime to clo clott

•

Variables:

 –

Source a and nd quan quantity tity of thr thrombin ombin

CAUSES OF PROLONGED THROMBIN TIME • • • • • •

Heparin Hypofibrinogenemia Dysfibrinogenemia Paraprotein Thrombin inhibitors (Hirudin) Thrombin antibodies

 

PLATELETS APPROACH TO THE THROMBOCYTOP THROMBOCYTOPENIC ENIC PATIENT •

History  – Is the patient bleeding? bleeding?

 

2. Are there symptoms of a secondary illness? (neoplasm, infection, autoimmune disease) 3. Is there a history of medications, medications, alcohol use, or recent transfusion? •

History 4. Are there risk factors for viral infection? 5.Is there a family history of thrombocytopenia? 6. Do the sites of bleeding suggest a platelet defect?

•

 –  –  –  –

Assess tth he n nu umber a an nd ffu unction o off p pllatelets

CBC with peripheral peripheral smear Bleeding time Platelet aggregation aggregation study PFA

CLASSIFICATION OF PLATELET DISORDER •

Quantitative d diisorders

 –

Abnormal distribu distribution tion

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Dilution e effect ffect

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Decreas Decreased ed produ production ction

 –

Increas Increased ed destru destruction ction

C(+SS)F)C+T)* F P(+TE(ET #)S>#E>S •

Qualitative disorders

 –

Inherite Inherited d disorde disorders rs (rare)

 –

Acquired disorders

• •

Immune Medications

 

• • •

Chronic renal failure Cardiopulmonary bypass Liver disease

)*HE>)TE# P(+TE(ET #)S>#E>S •

1ay/Hegglin%

Thrombocytopenia Large platelets Neutrophils – Dohle bodies

.-la3mann:s thrombasthenia% Congenital deficiency or abnormality of GP IIb-IIIa

•

Bernard-Solier syndrome :

Congenital deficiency or abnormality of GP Ib

ACQUIRED PLATELET DISORDERS •  

Decreased production: Ineffective thrombopoiesis - MDS

•

Increased destruction:

 

Immune Non-immune •

Poor aggregation INCREASED PLATELETS DESTRUCTION

ITP IS A DIAGNOSIS OF EXCLUSION !

COAG,LATION FACTOR DEFECTS )nherited Coagulation factor bleeding disorders  

!on=illebrand:s disease

 

Hemophilia 6+ and B7

HE1PH)()+ Clinical manifestations 6hemophilia + D B are indistinguishable7   Prolonged bleeding after surgery or dental etractions   Hemarthrosis 6most common7   Soft tissue hematomas   ther sites of bleeding ?rinary tract C*S, neck 6may be life/threatening7

 

 +C?)>E# B(EE#)*B(EE#)*- #)S>#E>S #)S>#E>S%%

         

&itamin  deficiency (i!er disease =arfarin o!erdose #)C )nhibitors to CF

VITAMIN K DEFICIENCY •

Source of vitamin K : Green vegetables Synthesized by intestinal flora

•

Required for synthesis Factors II, VII, IX ,X Protein C and S

•

Causes of deficiency : Malnutrition Billiary obstruction Malabsorption Antibiotic therapy

DIC DISSEMINATED DISSEMINA TED INTRAVASCULAR INTRAVASCULAR COAGULATION •

Sepsis

•

Trauma

 –  –

Head injury Fat embolism

•

Malignancy

 

PATHOGENESIS OF DIC Cons'mp"ion of  %oa/'la"ion fa%"ors presen%e of FDPs   aPTT   PT   TT   Fi2rino/en Presen%e of plasmin   D3$imer  In"ra4as%'lar %lo"   Pla"ele"s S%!is"o%"es

HEMOSTASIS IN LIVER DISEASE LIER DISEASE AND HE)OSTASIS •

Decreased synthesis of II, VII, IX, X, XI, and fibrinogen

•

Dietary Vitamin K deficiency (Inadequate intake or malabsortion)

•

Dysfibrinogenemia Enhanced fibrinolysis (Decreased alpha-2-antiplasmin)

•

DIC

•

Thrombocytopenia due to hypersplenism

•

)ANAGE)ENT OF HE)OSTATIC DEFECTS IN LIER DISEASE 

Treatme Tre atment nt for prolonged prol onged PT/PTT PT /PTT 

Vitamin K 10 mg SQ x 3 days - usually ineffective

 



 

Fresh-frozen plasma infusion: 25-30% of plasma volume (1200-1500 ml) (immediate but temporary effect)

Treatment for low fibrinogen 

Cryoprecipitate (1 unit/10kg body

APPROACH TO BLEEDING DISORDERS •

SUMMARY  Ide Identif ntify ya an nd c co orr rre ect an any y sp specif cific d de efe fec ct o off he hemosta stasi sis s

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Laboratory testing is always needed to establish establish the cause of bleeding bleeding

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Screening tests (PT,PTT, (PT,PTT, platelet count) count) will often allow placement iinto nto one of the broad categories

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Specialized testing is is usually necessary to establish establish a specific diagnosis diagnosis

•

Use no non n-tr -tran ansf sfu usio siona nall dru rug gs whene neve verr poss ssib ible le

•

RB RBC C ttra rans nsfu fusi sion ons s ffor or su surg rgic ical al pr proc oced edur ures es or la larg rge eb blo lood od lo loss ss

 

TH+* 4?G