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BLEEDING DISORDERS. Dr. Lai Yin Win Chai Pathologist. Definition A group of disorders having an abnormal tendency to bleed due to a defect in the haemostatic mechanism. Characteristics of Abnormal Bleeding Tendency Spontaneous bleeding into skin/ mucous membrane/ internal organs

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bleeding disorders

BLEEDING DISORDERS

Dr. Lai Yin Win Chai

Pathologist

slide2
Definition
  • A group of disorders having an abnormal tendency to bleed due to a defect in the haemostatic mechanism.

Characteristics of Abnormal Bleeding Tendency

  • Spontaneous bleeding into skin/ mucous membrane/ internal organs
  • Excessive or prolonged bleeding following trauma or surgery
  • Bleeding from more than one site
slide3
Classification of Haemorrhagic Disorders
  • Vascular defect
  • Platelet defect
  • Coagulation defect
thrombocytopenia
Thrombocytopenia

Definition

  • Reduction of platelets in the peripheral blood below the lower normal limit of 150x109/L
  • N value – 150 – 400 x 109 /L

Classification

Primary - Immune Thrombocytopenic Purpura

Secondary

  • Acute leukemia
  • Drugs and chemicals
  • Aplastic anaemia
  • Secondary BM infiltration
  • Hypersplenism
  • SLE
immunethrombocytopenicpurpura itp
ImmuneThrombocytopenicPurpura (ITP)

Definition

An acquired quantitative platelet defect due to autoimmune destruction of mature platelets in the peripheral blood.

clinical features
Clinical features
  • Two types- Acute ITP

- Chronic ITP

clinical features1
Clinical features
  • Features of anaemia corresponds to the severity of bleeding.
  • No jaundice, no fever, Mild splenomegaly
  • No hepatomegaly nor lymphadenopathy
  • No gum hypertrophy nor sternal tenderness
slide10
Course of bleeding
  • Spontaneous bleeding – skin (petechiae and ecchymosis) & mucosal (epistaxis & gum bleeding, haematuria, menorrhagia, melaena) and internal organ (brain &spinal cord) relatively uncommon but give serious consequence
  • Trauma/minor surgery/dental procedure with prolonged excessive bleeding
  • Severity of bleeding correlates with the degree of thrombocytopenia
  • Haemarthrosis and wound bleeding – not a feature of thrombocytopenia.
laboratory investigations
Laboratory Investigations

Screening Tests

  • Bleeding time is prolonged (Normal – 2-7 minutes)
  • Hess test positive
  • Normal clotting time

Blood for Complete Picture

  • Hb level – Decreased
  • Total WBC count – Increased (during bleeding)

Peripheral blood film

  • RBC – Normochromic normocytic Anaemia
  • WBC – Neutrophil leucocytosis
  • Platelets – reduced
slide14
Specific tests
  • Platelet count (Thrombocytopenia - < 150 x 109/L)
  • Exclude qualitative platelet defect
  • Does not differentiate ITP from secondary thrombocytopenia

Bone marrow aspiration

  • Excludes secondary Thrombocytopenia
  • In ITP : Megakaryopoiesis – Increased predominately immature

megakaryocytes characterised by less cytoplasmic

granularity poor platelet budding and vacuolation

  • Erythroid hyperplasia
  • Active granulopoiesis

Demonstration of IgG Antiplatelet Auto Antibody

  • Confirms ITP

Treatment .Corticosteroid therapy

. High dose intravenous Ig therapy

slide16

Normal Megakaryocyte

Abnormal Megakaryocyte in ITP

coagulation disorders
Coagulation Disorders

Classification

Congenital coagulation disorders

(1) Haemophilias

Haemophilia A or Classical Haemophilia (Factor VIII deficiency)

Haemophilia B or Christmas disease (Factor IX deficiency)

(2) Von Willebrand disease

(3) Others - Factor I deficiency

Factor V, VII, X, XI, XII & XIII deficiencies

Acquired coagulation disorders

  • DIC
  • Liver disease
  • Vitamin K deficiency
  • Acute primary fibrinolysis
  • Massive transfusion of stored blood
  • Anti-coagulant therapy
  • Circulating inhibitors of coagulation
haemophilia

XX

XY

XY

XX

XX

XY

XX

XX

XY

XY

XX

Haemophilia

Definition

  • A group of congenital coagulation disorders leading to an abnormal bleeding tendency due to genetically determined sex-linked recessive defect.
      • Haemophilia A ( Factor VIII deficiency )
      • Haemophilia B ( Factor IX deficiency )
  • Males are affected. Females are healthy carriers.

XY

slide21
History
  • A male child with history of abnormal bleeding in early infancy or adolescence.
  • Bleeding is trauma associated. Spontaneous bleeding less common.
  • Insidious onset, delayed bleeding, recurrent bleeding.
  • Family history of abnormal bleeding in male relatives from the maternal side of the family.
slide22
Clinical Features

Due to abnormal bleeding

  • Types & Sites of bleeding
      • Wound bleeding - Haematoma formation within deep tissues presenting as a painful swelling.
      • Joint bleeding – recurrent haemarthrosis is a characteristic feature (the joints usually involved are knee joints & ankle joints leading to pain and disability)
      • Post - traumatic bleeding & post - surgical bleeding following minor surgery & tooth extraction – prolonged & excessive bleeding.
      • Skin bleeding (petechial & ecchymosis) are rare and mucosa bleeding can be present.

Features of anemia depend on the severity of bleeding.

slide24

Lateral View

Front View

Haematoma

Haematoma

slide26
Laboratory Investigations

Screening Tests

  • Clotting time prolonged (Normal – 5 – 11minutes)
  • Bleeding time normal
  • Hess test normal

Blood for complete picture

  • Hb level - reduced

Peripheral blood film

  • Normochromic normocytic anemia
  • Neutrophil leucocytosis
  • Platelets – adequate
slide27
Special Tests

(1) Prothrombin Time (OSPT) - normal (excludes extrinsic pathway of coagulation - II, V, VII, X)

(2) APTT (Activated Partial Thromboplastin Time) – prolonged

This indicates intrinsic pathway of coaguation (XII, XI,

VIII, IX, X) are defective, most likely Factor VIII or IX

(3) Thrombin Time - normal

Confirmatory Tests

To demonstrate the specific deficient factor as well as to determine the degree of deficiency.

  • TGT (Thromboplastin Generation Test) to distinguish between Factor VIII & IX
  • Specific factor assay

Treatment – Factor replacement therapy

disseminated intravascular coagulation dic consumption coagulapathy defibrination syndrome
Disseminated Intravascular Coagulation, DIC(Consumption coagulapathy / Defibrination Syndrome)

Definition

  • Acquired thrombo-haemorrhagic disorder due to secondary complications with a wide variety of clinical settings.
  • Thrombotic element in DIC is widespread disseminated microvascular thrombosis
  • Initiated by the following clinical conditions

1. Obstretrical Complications - 50%

      • Abruptio Placentae (APH)
      • Amniotic fluid embolism
      • Septic Abortion
      • Toxaemia of Pregnancy
      • Retained Dead Foetus
slide30
2. Infections
  • Gram (-) Septicaemic Shock, Meningococcaemia, Malaria (Falciparum), Angioinvasive Fungal Infection

3. Neoplastic Conditions

  • Advanced Mucin producing Adenocarcinoma
  • Carcinoma Pancreas/Prostate/Lung/stomach
  • Acute promyelocytic leukaemia (M3)

4. Massive Tissue Injuries

  • Massive Trauma, Crush Injury, Extensive Burns & Heart & Lung Surgery

5. Miscellaneous

  • Haemolytic Transfusion Reaction, Acute Intravascular Haemolysis, Snake Bite (Viper Bite)
slide32
Clinical Features
  • Widespread microthrombosis leads to pain (microinfarction) & microangiopathic haemolytic anaemia
  • Haemorrhagic diathesis follows as localised bleeding from venepuncture sites, operative site & birth canal
  • Generalised bleeding from skin & mucous membrane
slide35
Investigations

Blood for complete picture

  • Hb level – Reduced

Peripheral Blood Film

  • Anaemia – normochromatic normocytic anaemia,

fragmented RBCs (MicroAngiopathic Haemolytic Anaemia)

  • Neutrophil Leucocytosis (Active Bleeding)
  • Platelets - reduced

Screening Tests

  • Bleeding time – prolonged
  • Hess test – positive
  • Clotting time – prolonged
  • Prothrombin time – prolonged (Lack of Factors II, V, X)
  • Activated Partial Thromboplastin Time – prolonged

(Lack of Factors V, VIII, X)

  • Thrombin time – prolonged (Lack of Factor I)
  • Fibrin Degradative Product – increased
slide36
Treatment
  • Treat the underlying cause is the important factor
  • Bleeding – give platelet concentrate and fibrinogen
  • Thrombosis – heparin & antiplatelet drugs
vitamin k deficiency
Vitamin K Deficiency
  • It is a fat soluble vitamin which is essential for the synthesis by the liver of factor II, VII, IX, X.
  • Vitamin K deficiency may present in the newborn or in later life.
  • In new born, vitamin K level is low due to liver cell immaturity & lack of gut bacterial synthesis of vitamin.
  • Bleeding manifestations in adults due to obstructive jaundice, pancreatic disease or small bowel disease
slide38
Investigations
  • Bleeding time & Hess test – normal
  • Clotting time – prolonged
  • Prothrombin time – prolonged
  • Activated Partial Thromboplastin Time – prolonged

Treatment

Intravenous Vitamin K adminstration