Hemorrhagic Diatheses Due to Abnormalities in Coagulation

8/9/2019 Hemorrhagic Diatheses Due to Abnormalities in Coagulation

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Hemorrhagic diatheses

due to abnormalities in

coagulation

SHIVAN KESAVAN





Clotting factor abnormalities

Inherited deficiencies Acquired deficiencies

Most common Rare : I,II,V,VII,X,XIII

Hemophilia A ( 1 n 5000)

Hemophilia B (1 n 30000)

Factor XII deficiency (1 n 1,000,000)

DIC

Liver disfunction

Vitamin K deficiency



Hemophilia A and B

X- linked inheritance

80% - A, 20% - B

Grading of severity

FVIII levels < 1% of normal ± severe

FVIII levels 1 to 5% -- moderate

FVIII levels 6 to 30% -- mild



Clinical presentation

Spontaneous ecchymoses ( Nopurpura / petechiae )

Epistaxis Menorrhagia

Post- partum / Post surgicalhemorrhage

GIT bleeds / Hematuria Recurrent hemarthrosis

Muscle hematomas



Bleeding can be

accelerated / unmasked by

Drugs and dietary supplements

² Aspirin and NSAIDs, Herbal

medicatons and foods rich in

Omega 3 fatty acids.

Systemic diseases ² Liver

failure, Severe renal

impairment, Hypothyroidism,

Paraprotenemias and

amyloidosis.



Life threatening sites of

bleeding

Intracranial

Retroperitoneal

Oropharyngeal



Management

Transfusional Non transfusional

1.Cryoprecpitate

2.Factor concentrates

3.Rcombinant factor

1.DDAVP2.Antifibrnolytics



Complications of

treatment

Inhibitor formation

Infection transmission



actor XI deficiency

High incidence in Ashkenazi and

Iraqi Jews.

Only mucocutaneous bleeding.

No joint bleeds.

Treatment is with Fresh Frozen

Plasma every alternate day.



Other rare disorders

Always mild ²

Can be severe ²

No hemarthroses. Mucocutaneous

bleeds only.

Treated with FFP / PCC

Factor VII deficiency

Dysfibrinogenemia

Factor X deficiency

Factor XIII deficiency



Familial Multiple

Coagulaton Deficiencies

FV and FVIII

Vitamin K dependent factors

! Mild bleeding tendency after trauma.

ERGIC-53 / MCFD 2 mutations.

GGCX / VKORC1 deficiency



DIC- Disseminated

Intravascular Coagulation

Thrombohemorrhagic disorder

Pathogenesis

Etologies: Malignancy

Obstetrc complications

Infections

Trauma and massive tissue damage

Envenomation

Vascular causes

Immunologic causes

Drugs

Diffuse liver disease



Clinical Manifestations

Hemorrhagic Thrombotic

Multi organ failure



Diagnosis Identify the cause

Coagulation tests

Platelet count

FDPs

Red cell count

Peripheral blood smear

Tests are repeated after 6 to 8hours



Differential Diagnosis for

DIC

Severe liver disease

TTP

Thrombocytopenia

Decreased plasma levels of coagulation factors

Increased FDP

× Values don¶t change rapidly

Thrombocytopenia

Fragmented red cells

Multi organ failure

×No consumption of coagulation factors

×No hyperfibirnolysis



Management

Treat the cause If bleeding is the predominant manifestation,

If thrombosis is dominant, low dosecontinuous infusion of heparin is done.

If thrombocytopenia is marked, give PRP

If PT is also markedly prolonged, give FFP

If fibrinogen level is markedly low or hyperfbrinolysis is

present, give Cryoprecipitate.

In pts with confirmed hyperfibrinolysis, anti fibrinolytics

can be given.

Clotting Factor concentrates not recommended.



Liver failure

Causes of bleeding in liver failure

1. Thrombocytopenia

2. Decreased synthesis of clotting

factors

3. Dysfibrnogenemia

4. DIC Treatment ² FFP, PRP,

Cryoprecpitate.



Vitamin K deficiency

Inherited- GGCX / VKOR1

Acquired

Treatment ² Vitamin K 10 mg

parenteral/oral



Acquired inhibitors of

clotting factors >60 yrs Most commonly against FVIII

Causes

Clinical features

Treatment- High dose FVIII , IVIG, Rituximab. In contrast to inhibitors in hemophilia, pts are

responsive to immunosupressive therapy.

Idiopathic

Autoimmune/ Neoplastic diseases

Dermatologic diseasesPrevious history of topical thrombin usage

× Hemarthroses

! But OP/ CNS/ RPH can occur

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Hemorrhagic Diatheses Due to Abnormalities in Coagulation