This document discusses bleeding disorders, their causes, and treatments. It covers the physiology of blood clotting including the coagulation cascade and identifies disorders in blood vessels, platelets, and coagulation factors that can cause bleeding. Specific disorders covered include hemophilia A/B, von Willebrand disease, vitamin K deficiency, and thrombocytopenia. Diagnostic tests and treatments using blood products like fresh frozen plasma, cryoprecipitate, and platelet transfusions are also summarized.

1. BLEEDING DISORDERS
ADENIYI O.A

2. OUTLINE
• Introduction
• Identifying clotting factors
• Physiology of Blood Clotting
• ◦ Hemostasis
• ◦ Bleeding disorders
• -Vascular disorders
• -Coagulation disorders
• -Platelet disorders

3. INTRODUCTION
• A group of disorders characterized by defective
haemostasis with abnormal bleeding.
• These causes are broadly divided into 4 groups :
• 1) Due to vascular abnormality.
• 2) Due to platelet abnormality.
• 3) Disorder of coagulation factor.
• 4) Combination of all these as in disseminated
intravascular coagulation.

5. HAEMOSTASIS
• Primary Haemostasis
◦ Blood vessel contraction
◦ Platelet Plug Formation
• Secondary Haemostasis
◦ Activation of Clotting Cascade
◦ Deposition & Stabilization of Fibrin
• Tertiary Haemostasis
◦ Dissolution of Fibrin Clot
◦ Dependent on Plasminogen Activation

6. CLOTTING FACTORS
• Factor I : Fibrinogen
• Factor II : Prothrombin
• Factor III : Thromboplastin
• Factor IV : Calcium
• Factor V : Labile factor
• Factor VII : Stable factor
• Factor VIII: Antihemophilic factor
• Factor IX: Christmas factor
• Factor X: Stuart Power factor
• Factor XI: Plasma thromboplastin antecedent
• Factor XII: Hegman factor
• Factor XIII : Fibrin stabilising factor

9. • BLEEDING TIME: Provides assessment of platelet count
and function
Normal value: 2-8 minutes.
• PROTHROMBIN TIME: Measures Effectiveness of the
Extrinsic Pathway
Mnemonic - PET
NORMAL VALUE: 10-15 SECS.
• PARTIAL THROMBOPLASTIN TIME: Measures
effectiveness of the Intrinsic Pathway
Mnemonic - PITT
NORMAL VALUE: 25-40 SECS

10. • THROMBIN TIME: Time for thrombin to
convert fibrinogen to fibrin
A measure of fibrinolytic pathway
• Normal value : 9-13 secs

12. DISORDERS OF HAEMOSTASIS
• Disorders of Blood vessels
Scurvy, Henoch-Schonlein syndrome.
• Disorders of Coagulation
Extrinsic, intrinsic, combined.
• Disorders of Platelets
Thrombocytopenia ITP, TTP, HUS, DIC.
Aspirin therapy, Thrombasthenia,
• Other disorders
DIC.

13. DISORDERS OF COAGULATION
Haemophilia A
SYMPTOMS:
• Bruising
• Spontaneous bleeding
• Bleeding into joints and associated
• pain and swelling
• Gastrointestinal tract and urinary tract hemorrhage
• Prolonged bleeding from cuts, tooth extraction, and
surgery
• People whose clotting activity is 5percent of normal
may have only mild hemophilia.

16. TREATMENT:
• Infusion of cryoprecipitate or desmopressin
Acetate (ddavp)
• Desmopressin injection or stimate nasal Spray.
• Cryoprecipitate
• Avoid certain drugs that can aggravate bleeding
problems:
• Aspirin, Heparin, Warfarin, Certain analgesics
such as nonsteroidal anti-inflammatory drugs

17. HAEMOPHILIA B
• Christmas disease- deficiency in clotting factor IX.
• Symptoms include:
• Nose bleeds , Bruising
• Spontaneous bleeding
• Bleeding into joints and associated pain and swelling
• Gastrointestinal tract and Urinary tract hemorrhage
• Prolonged bleeding from cuts, tooth extraction,
surgery,
• following circumcision
• People whose clotting activity is 5% of normal may
have only mild hemophilia.

19. COMPLICATIONS
• Chronic joint deformities,
• Intracerebral hemorrhage may also occur.

20. Von Willebrand Disease:
• hereditary deficiency or abnormality of the
von Willebrand factor in the blood, a protein
that affects platelet function
• .

21. Von Willebrand Disease
Classifications:
• Type I:
Most common and mildest form of von Willebrand
disease.
Levels of von Willebrand factor are lower than
normal, reduced levels of factor VIII.
• Type II:
• Von Willebrand factor itself has an abnormality.

22. V
• Type III:
• Severe von Willebrand disease. Total absence of
von Willebrand factor, and factor VIII levels are
often less than 10%.
• Inheritance Pattern
• occurs in men and women equally.
• Types I and II are usually inherited in what is
known as a "dominant“ pattern.
• Type III von Willebrand disease, however, is
usually inherited in a "recessive" pattern.

24. 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
Biliary obstruction
Malabsorption
Antibiotic therapy
• Treatment Vitamin K
Fresh frozen plasma

25. CLASSIFICATION OF PLATELET
DISORDERS
• Quantitative disorders
Decreased production
Increased destruction.
• Qualitative disorders
• ◦ Inherited disorders (rare)
• ◦ Acquired disorders
Medications
Chronic renal failure

26. THROMBOCYTOPENIA
• Definition:
• Thrombocytopenia is any disorder in which the platelet count is below
1OO,000/CC OF BLOOD.
• THROMBOCYTOPENIA
• Immune-mediated
a. Idioapthic
b. Drug-induced
c. Collagen vascular disease
d. Lympho proliferative disease
e. Sarcoidosis
• Non-immune mediated
a) DIC
b) Microangiopathic haemolytic anaemia

27. THROMBOCYTOPENIA
• Thrombocytopenia is often divided into three major causes of low
platelets:
• 1) Low production of platelets in the bone marrow
• 2) Increased breakdown of platelets in the bloodstream (called
• intravascular)
• 3) Increased breakdown of platelets in the spleen or liver (called
• extravascular
• 1)Disorders that involve low production in the bone marrow include:
• -Aplastic anemia
• -Cancer in the bone marrow
• -Infections in the bone marrow (rare)
• -Drugs (very rare)

28. THROMBOCYTOPENIA
• 2)Disorders that involve the breakdown of
platelets include:
• Immune thrombocytopenic purpura (ITP)
• Drug-induced immune thrombocytopenia
• Drug-induced non immune thrombocytopenia
• Thrombotic thrombocytopenic purpura
• Disseminated intravascular coagulation (DIC)
• Hypersplenism (an enlarged spleen)

29. THROMBOCYTOPENIA
Symptoms:
• Bruising
• -Nose bleeds or bleeding in the mouth
• ` -Rash (pinpoint red spots)
• -Other symptoms may be present as well,
depending on the
• cause of the condition.
• Mild thrombocytopenia can occur without
symptoms.

30. THROMBOCYTOPENIA
INVESTIGATIONS
• FBC shows low platelets
• -Bone marrow aspiration or Biopsy may be
normal or may show
• low megakaryocytes (platelet precursors) or an
infiltrating disease.
• -PTT clotting study is normal
• -PT clotting study is normal
• -Platelet associated antibodies may be present

31. THROMBOCYTOPENIA
• Treatment
• Depends on the cause of the condition. In
some cases, transfusion of platelets may be
required to stop or prevent bleeding.
• Complications:
• - Hemorrhage
• -Gastro intestinal bleeding
• -Intracranial haemorrhage

32. FRESH FROZEN PLASMA
• Content - plasma (decreased factor V and VIII)
• Indications
Multiple coagulation deficiencies (liver disease, trauma)
DIC
Warfarin reversal
Coagulation deficiency (factor XI or VII)
• Dose (225 ml/unit)
• 10-15 ml/kg
• Viral screened product
• ABO compatible

33. FRESH FROZEN PLASMA

34. CRYOPRECIPITATE
• Prepared from FFP
• Content - fibrinogen, von Willebrand factor,
factor VIII, factor XIII and fibronectin
Indications
• Fibrinogen deficiency
• Uraemia
• von Willebrand disease
Dose (1 unit = 1 bag)
• ◦ 1-2 units/10 kg body weight

35. CRYO PREPARATION

36. TREATMENT APPROACHES TO THE
BLEEDING PATIENT
• Red blood cells
• Platelet transfusions
• Fresh frozen plasma
• Cryoprecipitate
• DDAVP
• Recombinant Human factor VIIa

37. RBC TRANSFUSION: ADVERSE
REACTIONS
• Non-immunologic reactions
• Congestive heart failure
• Volume overload
• Fever
• Bacterial contamination
• Hypocalcemia
• Massive transfusion

38. PLATELET TRANSFUSION
• Source
Platelet concentrate (Random donor)
Pheresis platelets (Single donor)
• Target level
Bone marrow suppressed patient (>10-
20,000/μl)
Bleeding (>50,000/μl)

39. •THANK YOU FOR
LISTENING.