This document discusses bleeding disorders, including disorders of platelets and clotting factors. It describes hemophilia, which is a rare bleeding disorder caused by a defect in clotting factors. There are various types of hemophilia depending on the specific clotting factor deficiency. The document outlines the classification, clinical manifestations, diagnosis, treatment, and complications of bleeding disorders.

1. Bleeding Disorders

2. • Disorders of platelets (Quantitative-
Thrombocytopenia, Qualitative-Defective
platelet function)
• Disorders of vessels
• Disorders of clotting factor

4. A rare group of blood disorders in
which there is defect in clotting factors
70% are X-linked recessive disorder.30%
spontaneous mutation.
The bleeding patterns of
haemophilia are similar.
Types:
 A:Deficiency in factor VIII (classic
haemophilia)
 B: Deficiency in factor IX (Christmas
disease)
 C: Deficiency in factor XI

5. Classification Clinical Manifestation
Severe
(<1% of normal)
•Manifest in infancy when child reaches
toddler stage
•Spontaneous bleeding – in muscles or
joints (haemarthroses)
•Excessive bleeding after minor trauma,
postoperatively, or after intramuscular
childhood vaccinations.
Moderate
(1-5% of
normal)
• Manifest after 2 years of life
• Moderate trauma causes bleeding episodes
• Occasionally spontaneous bleeding occurs
Mild
(>5% - <40% of
normal)
• Often diagnosed in teenagers and adults
• Significant trauma to induce bleeding
• No spontaneous bleeding

6. • Genetic inheritance
• Spontaneous mutation
• Acquired hemophilia
Signs and symptoms vary according to
the cause; There are various types of
hemophilia. They are:

7. Haemarthrosis (spontaneous bleeding
in muscle or joints - painful)
Illiapsoas bleeding
Joint Swelling
Easy bruising
Epistaxis
Haematuria
Intracranial hemorrhage

8. Complete blood count
Activated partial thromboplastin time
(aPTT)
– PROLONGE
Normal Prothrombin Time, Platelet
count Bleeding Time.
Specific factor assay : VIII or IX - LOW
Joint x-ray
Further Investigation
Hepatitis B, Hepatitis C, HIV serology
Diagnosis for carrier status for genetic
counseling

9. Factor concentrate infusion at
home as prophylaxis;
Repeated venopuctures
Need for venous access
Availability
Cost
Developed Inhibitors
Joint destruction; inflammation,
swelling, fibrosis.
Acquisition of virus (Hep B, Hep C, Hep
D &HIV)

10. First aid: (PRICE)
Pressure.
Rest
Ice
Elevation
Blood transfusion – severe blood lost
Factor concentrates; continuous infusion
(severe) or intermittent bolus (prophalaxis).
Factor VII given every 8-12 hours
Factor IX given every 12-24 hours
Desmorpressin acetate (DDAVP); mild &
moderate, not for severe.
Antifibrinolytics: Aminocaproic acid (Amicar)
Fresh Frozen plasma (high risk for virus)

11. Oblong diskshape
Size- 2-4 µm on the longaxis
Volume- 5-12 fL
Produced in bone marrow by
megakaryocte cell
Platelet count in blood- 150,000-350,000µL

12. Quantitative disorder
 Abnormal distribution
 Dilution effect
 Decreased production
(leukemia and some anemia)
 Increased destruction
Qualitative disorders
 Inherited disorders (rare)
 Acquired disorders (medication,
chronic renal failure,
cardiopulmonary bypass)

13. Defined as reduced in the platelet count< 150, 000µL that characterized
by spontaneous bleeding, a prolonged bleeding time, and a normal PT
and PTT.
The risk of bleeding depends on the level of the platelet count:
Mild
thrombocytopenia
(platelet <150 000
cells/µL)
Moderate
thrombocytopenia
(platelet 20 000 - 50
000 cells/µL)
Severe
thrombocytopenia
(platelet <20 000
cells/µL)

14. • DEFINITION
 Isolated thrombocytopenia with otherwise normal blood count in
a patient with no clinically apparent associated conditions that
can causethrombocytopenia (such asHIV infection, SLE,
lymphoproliferative disorders, alloimmune thrombocytopenia,
and congenital or hereditary thrombocytopenia).
 Caused by immune- mediated destrcuction of circulating
platelet d/t anti-platelet autiantibodies
 There are two clinical subtypes of ITP:
o Acute ITP.
o Chronic ITP (starts after the diseasehasbeen present
• for >6 months).

15. Feature Acute ITP Chronic ITP
Peak age Children (2-6 yrs) Adults (20-40 yrs)
Female:male 1:1 3:1
Antecedent Infection Common Rare
Onset of symptoms Abrupt Insidious
Platelet count at
presentation
<20 000 <50 000
Duration 2-6 weeks Long term
Spontaneous remission Common Uncommon

16. Inappropriate immune recovery follows an acute viral infection in
children.
Autoantibodies (IgG or IgM) directed against platelet membrane
antigens (especially glycoprotein complex IIb/IIIa).
Phagocytosis of antibody-coated platelets by the reticuloendothelial
system.
Increased destruction of platelets – Thrombocytopenia.

17. Onset isusually sudden for acute ITP
and inchronic ITP,it isinsidiousonset.
Petechiae orpurpura
 Feet, legs,arms, and buttocks.
Mucosalbleeding.
 Palatal petechiae, epistaxis, hematuria,
menorrhagia, GI bleeding.
Rarely, intracranial hemorrhage may
occur inlong standing severe
thrombocytopenia.

19. • History taking.
• Physical examination.
o Signsof bleeding (petechiae and
purpura).
o Mucosal bleeding.
• Investigations.
o Full blood count.
Low platelet count.
o Histological findings.
Platelets are normal in sizeor
may appear larger than normal.
Normal red blood cells
morphology.
Normal white blood cells
morphology.
o Coagulation tests.
 Prolong bleeding time, normal
PT and PTT.

20. Intracranial hemorrhage - 50% mortality rate.
Riskof ICH highestin:
 Platelet count <20 000/mm³.
 History of head trauma.
 Usesof aspirin (inhibitor of platelet
aggregation).
 Presence of cerebral arteriovenous
malformation.
50%of all ICH occursafter 1month of
presentation, 30%after 6 months.

21. Choice of treatment:
• Oral prednisolone - 4 mg/kg/day for
7days, taper and discontinue at 21
days.
• IV Methylprednisolone - 30
mg/kg/day for 3days.
• IV Immunoglobulin - 0.8 g/kg/dose
for
1day OR250 mg/kg for 2 days.
• IV Anti-Rh(D) immunoglobulin - (50-
75µ/kg) in
Rhesuspositive patients – may