6._Pathology-Bleeding_disorders_DR_OROMO.ppt

Pathology

1. Bleeding DisordersBleeding Disorders
Clinical Hematology
Presented by
Dr. Oromo Francis
MD clinical pathology

2. Review of HemostasisReview of Hemostasis
Function ofFunction of :- :-
Coagulatory systemCoagulatory system
PlateletsPlatelets
EndotheliumEndothelium

Fibrinolytic systemFibrinolytic system
Bleeding
Thrombosis
Both (DIC)

3. Clinical forms of bleedingClinical forms of bleeding

Mucosal bleeding: epistaxis, bleeding gum, Mucosal bleeding: epistaxis, bleeding gum,
menorrhagia,..menorrhagia,..

Skin: petechiae, purpura, ecchymosis Skin: petechiae, purpura, ecchymosis

Deep hematoma: muscle, joints..Deep hematoma: muscle, joints..

Wound bleedingWound bleeding

Hematurea,...Hematurea,...

4. ecchymosisecchymosisPurpuraPurpura//
petechiaepetechiae
hematoma

5. Investigations in Bleeding ptInvestigations in Bleeding pt
Routine:Routine:
CBC, plt count, CBC, plt count,
Bleeding time (8 mins) Bleeding time (8 mins)
PT (11 sec), APTT (28 sec), TT(11 sec)PT (11 sec), APTT (28 sec), TT(11 sec)
SpecificSpecific
Factors assay, plt function tests, ...Factors assay, plt function tests, ...
AdvancedAdvanced
..........

6. Vascular causes of bleedingVascular causes of bleeding

Congenital malformationCongenital malformationss

Infections ( viral , bacterial,..)Infections ( viral , bacterial,..)

Immune vasculitis (Henoch- Schonlen purpura)Immune vasculitis (Henoch- Schonlen purpura)

Vit C deficiencyVit C deficiency

Senile purpura.Senile purpura.

........
Mucosal/
skin
Bleeding
time

7. Coagulatory disordersCoagulatory disorders
CongenitalCongenital
Hemophilia A ( factor VIII: C)Hemophilia A ( factor VIII: C)
Hemophilia B (factor IX)Hemophilia B (factor IX)
Von Willibrand diseases( factor VIII:vw)Von Willibrand diseases( factor VIII:vw)
AcquiredAcquired
Vit K def.Vit K def.
Liver diseases Liver diseases
InhibitorsInhibitors
DICDIC

8. Hemophilia AHemophilia A
X linked inherited Factor VIII:C deficiencyX linked inherited Factor VIII:C deficiency
ClinicallyClinically : bleeding after trauma+ : bleeding after trauma+
heamartherosis+ muscles hematomaheamartherosis+ muscles hematoma
Lab finding :Lab finding :
Normal plt, bleeding time, PT,TTNormal plt, bleeding time, PT,TT
Prolonged APTTProlonged APTT
Mixing studies and factor assayMixing studies and factor assay
Gene studies Gene studies

9. Hemophilia AHemophilia A
Complications
...of bleeding
Disability (joints)
Of treatment
HIV, HBV, HCV transmission
Inhibitors

10. Hemophilia AHemophilia A
ManagementManagement
General ttt.General ttt.
Factor 8 concentrateFactor 8 concentrate
FFP, cryoprecipitate,...FFP, cryoprecipitate,...
Induce factor production (Desmopressin)Induce factor production (Desmopressin)
Treat complications, (rehabilitation)...Treat complications, (rehabilitation)...
Gene therapyGene therapy

11. Von Willibrand diseaseVon Willibrand disease
AD, factor VIII:wv defAD, factor VIII:wv def
Three types (1.2.3).Three types (1.2.3).
Both plt and coagulatory disorderBoth plt and coagulatory disorder
Clinically: both mucosal and deep tissue bleedingClinically: both mucosal and deep tissue bleeding
Lab finding:Lab finding:
Normal plt count, abn plt function testNormal plt count, abn plt function test
Prolonged Bleeding timeProlonged Bleeding time
Normal PT. prolonged APTTNormal PT. prolonged APTT Tre
a
tm
e
n
t
?

12. Disseminated intravascular Disseminated intravascular
coagulation (DIC)coagulation (DIC)

Massive activation of coagulation with Massive activation of coagulation with
consumption of factors and plt resulting in consumption of factors and plt resulting in
sever Bleeding.sever Bleeding.
DIC= Consumptive CoagulopathyDIC= Consumptive Coagulopathy

13. PathogenesisPathogenesis
Activation
of factor
Plt
activation
Thrombus
factor
Plt
FDPs
Vaso
occlusion
Organ
failure
Bleeding
cause
Endothelial
damage
Lysis

14. CausesCauses

Massive release of tissue factorMassive release of tissue factor

Septic shockSeptic shock

Snake biteSnake bite

MalignancyMalignancy

Liver and renal diseasesLiver and renal diseases

........
Acute
Chronic

15. ......DICDIC

ClinicallyClinically: bleeding+ necrosis+ ARF+...: bleeding+ necrosis+ ARF+...

Lab findingsLab findings: All abnormal.: All abnormal.

Treatment:Treatment: (difficult) (difficult)

The cause ?The cause ?

Fresh bloodFresh blood

FFPFFP

PLtPLt

Heparin?Heparin?

16. Platelets DisordersPlatelets Disorders
Normal structureNormal structure
GlycoproteinsGlycoproteins
Gramules (alpha, dense)Gramules (alpha, dense)
Normal functions:Normal functions:
Adhesion: gp 1-4, VWF, FVIII:C.Adhesion: gp 1-4, VWF, FVIII:C.
Aggregation: by thrimbin, PAF,...Aggregation: by thrimbin, PAF,...
Activation: PG, TXA2,.... Activation: PG, TXA2,....

17. Plt disordersPlt disorders
Function
--athenia
Number
--penia
Number
--cytosis
BLEEDING
THROMBOSIS

18. Production
Thrombocytopenia/ causesThrombocytopenia/ causes

Destruction
Abn .
distribution
BM , congenital, drugs
Spleen , immune, drugs
Spleen, fat embolism

19. Immune ThrombocytopeniaImmune Thrombocytopenia
(ITP)(ITP)

Idiopathic / autoimmune (IgG)Idiopathic / autoimmune (IgG)

Females 20-30 yrsFemales 20-30 yrs

Sever plt, BM megakaryocytesSever plt, BM megakaryocytes

Recurrent bleeding ,no spleenRecurrent bleeding ,no spleen

Do coomb test .Do coomb test .

Give plts, and steroids.Give plts, and steroids.

20. Thrombotic thrombocytopenic Thrombotic thrombocytopenic
purpura (TTP)purpura (TTP)

Congenital or acquired 5 disordersCongenital or acquired 5 disorders

plt (purpura)plt (purpura)

thrombosisthrombosis

MAHAMAHA

Renal impairment( uremia and HT)Renal impairment( uremia and HT)

CNS symptoms CNS symptoms

In children more hemolytic and ARF so called hemolytic In children more hemolytic and ARF so called hemolytic
uremic syndrome (HUS)uremic syndrome (HUS)

Usually follow E.coli infection( bloody dirrhea)Usually follow E.coli infection( bloody dirrhea)

21. Plt functional disordersPlt functional disorders
AssignmentAssignment! !

Congenital/ acquiredCongenital/ acquired
C/FC/F
Plt function testsPlt function tests

22. DisorderDisorder
testtest
Plt Plt
countcount
BleediBleedi
ng ng
timetime
PTPTAPTTAPTTTTTT
thrombocytthrombocyt
openiaopenia
NNNNNN
HemophiliHemophili
aa
NNNNNNNN
WV diseaseWV diseaseNNNNNN
DICDIC