1. BLEEDING DISORDERS CLINICAL FEATURES PROF.DR.G.SUNDARAMURTHY’S UNIT M7 BHARGAVI.K

2. Bleeding disorders Vascular abnormalities *** Platelet disorders Clotting factor abnormalities DIC

4. Petechiae PETECHIAE:minute 1-2 mm hges into skin,mucous membranes or serosal surfaces Do not blanch with pressure (typical of platelet disorders)

5. Petechiae

6. Purpura :slightly larger 3 mm haemorrhages

7. Bleeding disorders Platelet disorders ↓ production ↑ destruction Sequestration Hypersplenism Primary/Idiopathic ITP Acute/Chronic Secondary Drugs, HIV

15. ITP Same Normal or ↑Megakaryocytes Bone marrow Same Thrombocytopenia & Giant PLTS Peripheral smear IgG against Platelet GP - Pathogenesis >6mnoths <2 months Duration - Viral infection/ vaccine Predisposing Factors Gradual Abrupt Onset Adult/Female Children Age / Sex Chronic Acute Feature

20. Thrombotic Microangiopathies HUS TTP Feature Genetic (vWF metalloprotease- ADAMTS 13 ) deficiency Cause Infection ( E.coli O157 : H7) Adults Age Children Less prominent Acute Renal Failure Prominent Prominent Neurological symptoms Absent

23. Glanzmann Thromasthenia BSS Inheritance Autosomal Recessive Autosomal Recessive, rarely AD Platelet Count Normal Low Size Normal Giant Glycoprotein Deficiency IIb/IIIa aggregation Ib-IX-V adhesion

24. Platelet functional disorders

31. CLINICAL DISTINCTION SMALL, SUPERFICIAL LARGE,DEEP ECCHYMOSIS SKIN,MUCOUS MEMBRANES DEEP SITE OF BLEEDING RARE-EXCEPT IN VWB N HHTELENGIECTASIA COMMON POSITIVE FAMILY HISTORY MC IN FEMALES MALES SEX PERSISTENT N PROFUSE MINIMAL BLEEDING FROM SUPERFICIAL CUTS RARE COMMON DELAYED BLEEDING RARE CHARACTERISTIC HEMARTHROSIS CHARACTERISTIC-SMALL N MULTIPLE COMMON-LARGE N SOLITARY SUPERFICIAL ECCHYMOSES RARE CHARACTERISTIC DEEP DISSECTING HEMATOMAS CHARACTERISTIC RARE PETECHIAE DISORDERS OF PLATELET OR VESSELS DIORDERS OF COAGULATION

32. Platelet Coagulation Petechiae, Purpura Hematoma, Joint bl.

33. Bleeding disorders Vascular abnormalities Platelet disorders Clotting factor abnormalities DIC

35. 1/50,000 1/10,000 males Incidence X-linkedrecessive X-linkedrecessive Inheritance Factor IX Factor VIII factor deficiency Hemophilia B Hemophilia A

36. Hge secondary to trauma or surgery Rare spontaneous hge. 6-30% Mild Hge secondary to trauma or surgery Occasional spontaneous hemarthroses 1-5% Moderate Spontaneous he from early infancy Frequent spontaneous hemarthroses <1% Severe Clinical features Levels Classificat ion

39. Ecchymosis

40. Ecchymoses (typical of coagulation factor disorders)

42. Hemarthrosis (acute)

43. Hemarthrosis

45. CT scan showing large hematoma of right psoas muscle

51. Mutations in vWF precluding binding of FVIII.- autosomal haemophilia 2N Dysfn of vWF molecule 2M Inc.spontaneous binding of vWF to platelets 2B Inc.susceptibility to cleavage by ADAMTS 13-loss of high n intermediate multimers 2A Defect Type

54. Hemophilia A Hemophilia B Von Willebrand Disease Inheritance X linked X linked Autosomal dominant Factor deficiency VIII IX VWF Bleeding site(s) Muscle,joint Surgical Muscle ,joint Mucous Skin Prothrombin time Normal Normal Normal Activated PTT Prolonged Prolonged Prolonged Bleeding time Normal Normal Prolonged or normal Platelet aggregation Normal Normal Normal

55. Bleeding disorders Vascular abnormalities Platelet disorders Clotting factor abnormalities DIC

60. Henoch-Schonlein purpura

62. Henoch-Schonlein purpura

63. Bleeding disorders Vascular abnormalities Platelet disorders Clotting factor abnormalities DIC

64. Disseminated Intravascular Coagulation (DIC) Mechanism Systemic activation of coagulation Intravascular deposition of fibrin Depletion of platelets and coagulation factors Bleeding Thrombosis of small and midsize vessels with organ failure

65. Trigger Thrombin Generation Fibrinemia Consumption of platelet &coagulation factor Microvascular thrombosis Preservation of fibrin Proteolysis of fibrin Hemorrhagic diathesis Organ damage D.I.C.

70. THANK YOU