Bleeding and coagulation disorders

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-Schonlienpurpura 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-Souliersyndrome • 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: • Glanzmannthrombasthenia • 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 cytometricanalysis 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 50000 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.