Bleeding and coagulation disorders

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.

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.

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.

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.

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

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

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

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)

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.

Coagulation factor disorders

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)

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.

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.

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.

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

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

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

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.