1. Bleeding and coagulation disorders

2. Bleeding and coagulation disorders
any bleeding or coagulation defect may be :
Platelet defects;
Clotting factor defects which may be due to:
Decreased production .
Presence of antagonism e.g. heparin.
Consumption as in DIC or hemolytic uremic syndrome.
Vascular defects as occurs in Henoch-Schonlien purpura and in meninococcaemia.

3. Bleeding and coagulation disorders
history and examination:
Age:
Sex;
Race; Factor XI deficiency (Jews.)
F.H. of hereditary bleeding and coagulation disorders
History of any associated condition like; trauma, surgery ,dental extraction,
The site of bleeding;
The type and characteristic bleeding.
Evidence on examination like hepatosplenomegaly, lymphadenopathy or pallor.

4. Bleeding and coagulation disorders
Note:
Platelet and small vessel disorders are usually associated with:
Petechiae and echymosis.
Small hematomas.
Mucus membrane bleeding.
Short lived bleeding after trauma.
While coagulation factor defects are characterized by:
Large, deep hematomas.
Hemarthrosis, spontaneous or after minor trauma.
Persistent or recurrent oozing after trauma.

5. Bleeding and coagulation disorders
Investigations
F.B.C. including platelet count and blood film.
P.T
P.T.T.
T.T.(fibrinogen and heparin)
Reptilase Time
Fibrinogen or other single coagulation factor assay whenever indicated.
Platelet function tests.(Platelet Function Analyzer)
Bleeding and clotting times are rarely used especially in children.

6. Platelet disorders: 1: Thrombocytopenia (T.P.): (bellow 100.000/c.mm)
Decreased production:
Wiskott-Aldrich syndrome
TAR syndrome.(The causes of acquired aplastic anemia.
Increased destruction :
I: Immune mediated T.P.:
II: Drugs, DIC and moderate to huge splenomegaly.
III: Hemolytic –uremic syndrome.
IV: Large haemangiom
sequestration of the platelets within an enlarged spleen or other organ
2: Platelet function disorders

7. Platelet disorders: Idiopathic T.P. (ITP):
most common form of T.P. in children .
commonly idiopathic, but it may be viral or drug induced.
commonly between 2 and 6 years of age
few weeks after a viral or on bluesky
It begins with cutaneous bleeding or mucus membrane bleeding , and intracranil bl. Less than 1%.
there is neither pallor no organomegaly

8. Platelet disorders: Idiopathic T.P. (ITP) low platelet count
B.M. examination is not routinely indicated
10% of acute I.T.P. will become chronic (more than6 months)
Management of I.T.P. is :
mainly supportive,
moderate and severe forms may be treated with steroid, IVIG or anti-D
Platelet concentrate is rarely given
Splenectomy in over 5 years for chronic ITP

9. Platelet disorders: Neonatal immune mediated T.P.:
Passive –autoimmune T.P
Iso-immune T.P.
2: Platelet function disorders
Bernard-Soulier syndrome
autosomal recessive disorder
severe congenital platelet function disorde
decrease V WF receptor on the platelet membrane
thrombocytopenia, with giant platelets
Platelet aggregation tests show absent ristocetin-induced platelet
aggregation( normal to other agonists)

10. Platelet disorders: Causes of acquired thrombasthenia:
Glanzmann thrombasthenia
congenital disorder associated with severe platelet dysfunction
A ggregation studies show abnormal or absent aggregation with all agonists used except ristocetin
diagnosis is confirm ed by flow cytometric analysis of the patient' s platelet glycoproteins
Causes of acquired thrombasthenia:
drugs as aspirin and valproic acid,
uremia
severe liver disease.

11. Coagulation factor disorders

12. Coagulation factor disorders
Management principles include:
Preventing trauma.
Avoidance of aspirin and other NSAID.
Psychological support
Replacement therapy
Dose of factor VIII = % desired factor VIII to be raised x kg. Body wt.x 0.5
DDAVP may be helpful in mild cases.
Prophylactic factor VIII A
Hemophilia A (Classic Hemophilia) :
an XLR disorder
Various forms of severity exist
mild
Moderate
Severe
Hemarthrosis and deep soft tissue bleeding
Prolonged PTT with normal PT and normal BT
(study comparing prophylaxis with aggressive episodic treatment provides evidence for the superiority of prophylaxis in preventing debilitating joint disease)

13. Coagulation factor disorders
Hemophilia B :(Christmas disease):
5 times less than hemophilia
an XLR disorder
treated by replacement therapy
1 unit of factor IX /kg raises blood level by 1 unit/dl
Factor IX deficient patients are much less likely to develop antibodies against factor IX
U of required F IX= kg (body weight) X U% of desired rise.
Laboratory investigations reveal:
normal PT,
prolong PTT,
normal platelet count
decreased factor IX level
Hemophilia C (factor XI): this form of familial coagulopathy is only common among Jews. It causes mild to moderate bleeding tendency and FFP is given when required.

14. Coagulation factor disorders
Von-Willebrand disease (VWD)
is most common inherited coagulation disorder
Types:
type 1 mild , quantitative , (both VWF and factor VIII) this type is the most common form
type 2 , qualitative
type 3 , most severe, quantitative
mild-moderate bleeding from mucus surface and excessive bleeding after trauma.

15. Coagulation factor disorders
Von-Willebrand disease (VWD)
Laboratory findings reveal:
Prolong bleeding time.
Normal PT
Prolonged PTT (not always, but always and only in type 3)
Normal platelet count
Quantitative assay for VWF antigen activity (ristocetin factor assay) is diagnostic
Treatment:
DDAVP :useful in type 1 and occasionally type 2.
Cryoprecipitate which contains intact VWF is quite effective even in the severe (type 3) form,
factor VIII may occasionally be used.

16. Coagulation factor disorders
Vit. K deficiency:
acquired form of coagulopathy
vit. K dependent factors (factor II , VII ,IX ,X)
Etiology:
Dietary deficiency .
Defective fat absorption .
Drugs which interfere with Vit. K metabolism.
Hemorrhagic disease of the newborn; which occurs in 1 in live births

17. Coagulation factor disorders
Vit. K deficiency:
risk factors for Hemorrhagic disease of the newborn include:
Maternal.
Antibiotic given to the baby.
Preterm
Breast fed
Laboratory findings: prolonged PT and PTT
Treatment: Vitamin k injection ± FFP
I.M.or oral Vit. K is a reliable prophylaxis

18. DIC : acquired coagulopathy and bleeding
in vivo activation of coagulation
consumption of factor II, VIII and T.P
widespread deposition of fibrin ,bleeding from multiple sites and hemolytic anemia.
Blood film characteristically shows fragmented burr and helmet shaped red cells (Schizocytes):
↓ platelet count
↓ fibrinogen
↓ PT
↓ PTT
↓ T.T
↓ factor VIII
↑F.D.P

19. Recognition and treatment of the triggering factors.
Fresh blood, FFP, cryoprecipitate, replacement of other coagulation factors.
Exchange transfusion (double volume) helps by removing toxins and FDP, in addition to supplying the replacement factors.
Anticoagulants as heparin.
Steroid may rarely be used in cases like meningococcemia.