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Dr. Prakash Saldanha. BLEEDING/COAGULATION DISORDERS. BLEEDING/COAGULATION DISORDERS. HEMOSTASIS a) Vascular phase - Vaso constriction b) Platelet phase - Plug c) Plasma phase - Thrombus APPROACH a) Bleeding / Coagulation Spontaneous / Trauma
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Dr. Prakash Saldanha BLEEDING/COAGULATION DISORDERS
BLEEDING/COAGULATION DISORDERS HEMOSTASIS a) Vascular phase - Vaso constriction b) Platelet phase - Plug c) Plasma phase - Thrombus APPROACH a) Bleeding / Coagulation Spontaneous / Trauma Superficial / deep Small / large Short / prolonged
BLEEDING/COAGULATION DISORDERS b) Congenital or Acquired Family history Trauma Umbilical bleeding Immunological- Post transfusion Newborn (Vit. K) Congenital anomalies Auto immune- ITP Eczema Drug – Aspirin Skin elasticity Systemic illness Joint HE PEM Absent radius Sepsis HemangiomaLeukemia Liver Failure Renal Failure Vascular – HUS/HSP
BLEEDING/COAGULATION DISORDERS C) Investigations • Platelet count - ( Quality / Quantity ) • CBC, PS (Pancytopenia, Clumps, abnormal cells) • B.T. • C.T • P.T. - ( II, V, VII,X ) – Hepatic • Activated P.T.T. ( Intrinsic) • Special Factor assay VII, XIII, IX Bone marrow Invitro platelet adhesive test Qualitative Clot retraction test
BLEEDING/COAGULATION DISORDERS C. I.T.P Pathogenesis – Auto immune Types - Acute Acute relapsing Chronic Management :- • Supportive Avoid - Physical activity, IM injections, Drugs, Monitor - Neuro observation, platelet count • Drugs - Oral steroids Methyl Prednisolone 30mg/kg/day x 5 IVIG 400 mg/kg/day x 5 Anti Rh – D c) Platelet transfusion
BLEEDING/COAGULATION DISORDERS CHRONIC ITP PULSE THERAPY – Methyl Prednisolone / IVIG (Biweekly) VINCRISTIN CYCLOPHOSPHAMIDE DANAZOL INTERFERON SPLENECTOMY
BLEEDING/COAGULATION DISORDERS D. HAEMOPHILIA A - DECREASE VIII C B - DECREASE IX ‘X’ LINKED RECESSIVE BOYS ARE MORE DEEP BLEEDING - JOINTS DIAGNOSIS - INCREASE PTT / FACTOR ASSAY TREATMENT :- • CRYO PRECIPITATE • FACTOR VIII • DDAVP • PROTHOMBIN COMPLEX CONCENTRATE (HEMOPHILIA B )
BLEEDING/COAGULATION DISORDERS E. VASCULAR HEMOPHILIA 1.UNDERPRODUCTION OF VON WILLIBRAND PROTEIN 2.COMPONENTS – PLATELET ADHESIVE COMPONENT (VIII R ) FACTOR VIII CARRIER PROTEIN (VIII C) TYPES :- I. CLASSICAL AUTOSOMAL DOMINANT BOTH COMPONENTS DECREASED BOTH PLATELET / CLOTTING – ABNORMAL • AUTOSOMAL RECESSIVE ONLY PLATELET COMPONENT DECREASED ONLY BLEEDING ABNORMALITIES NO CLOTTING ABNORMALITY
BLEEDING/COAGULATION DISORDERS TREATMENT:- • CRYO PRECIPITATE • FACTOR VIII REPLACEMENT • DESMOPRESSIN (DDAVP)