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Bleeding disorder
- 2. Approach to bleeding tendency BY DR : MOHAMED BASIONY
- 3. 1 – massive uncontrolled bleeding 2 – bleeding from 1 orifice ( no local cause ) 3 – bleeding from 2 non related orifices 4 – uncontrolled bleeding after minor trauma 5 – uncontrolled bleeding after minor surgery Bleeding tendency
- 4. Haemostasis
- 5. It means stopping of bleeding and prevention of blood loss when ablood vessel is injured with the formation of haemostatic plug. It occurs by the following sequences: (1) Vasoconstriction. (2) Platelet plug. (3) Formation of blood clot. (4) Repair of the damaged blood vessel. Definition
- 6. Vascular endothelium and blood vessel wall. Platelets ( count and function ) Coagulation factors. Proper Function of Fibrinolytic Pathway So haemostasis is similar to a building which needs the following: Base Stones Cement substance Blood vessel Platelets Fibrin Needs
- 7. Role of each Vessel wall Vasoconstriction ( immediate ) 1- slow blood flow decrease bleeding. 2- by slowing allow contact activation of platelets & coagulation factors.
- 8. Platelets
- 9. Platelets are normally made in the bone marrow from progenitor cells known as megakaryocytes. Normal platelet lifespan is 7-10 Every day, 1/10 of platelet pool is replenished. Normal platelet count between 150,000-400,000/mm3 Contain intracellular granules that contain coagulation factors and ADP Production stimulated by thrombopoietin from liver/kidney Normal Physiology of Platelets
- 11. Under normal conditions vascular endothelium secretes (nitric oxide- prostacyclin-heparan )prevent platelet and wbc adhesion to endothelium
- 12. Platelet can close minor injuries only.
- 15. only if major injury target formation of fibrin mish Support platelet and close the opening. Coagulation system Secondry Haemostasis
- 17. Normal 2-7 minutes It is the time (in minutes) that is taken for bleeding to cease from asmall superficial wound. It is mainly affected by platelet count, function ,vascular integrity prolonged in thrombocytopenia – thromboathenia – vascular abnormality General tests for Haemostasis Bleeding time
- 18. Normal 5-10 minutes Measures coagulation function Prolonged in all types of coagulation defects Why it represents intrinsic pathway? Insensitive. Not done Coagulation time
- 19. Normal 12-14 seconds Assess extrinsic and common pathways. It is measured by adding tissue thromboplastin and calcium to the patient's plasma (extrinsic system) Prolonged in - liver disease, vit K deficiency. Differentiate? -deficiency of factors I, II, V, VII or X. - oral anticoagulant therapy, DIc Prothrombin time
- 20. international normalized ratio PT ratio = patient Pt ⁄ control PT. The INR is an accurate method for monitoring oral anticoagulant therapy. normal 0.8-1.2 the recommended therapeutic target is an INR with range of 2-3 INR for all indications( AF ) except prosthetic heart valves, INR 2.5-3.5 is suggested. INR
- 21. Normal 30-40 seconds Assess intrinsic and common pathways… Adding kaolin (surfaceactivator)+calcium+phospholipids to patient plasma…. prolonged in deficiency of factors: I, II, V, VIII, IX, X, XI Or XII. Also it is prolonged in DIC, heparin therapy and liver disease. Activated partial thromboplastin time
- 22. normal 12-20 seconds. Adding thrombin to patient plasma. Prolonged in : - hypo or dys fibrinogenaemia - DIC . Thrombin time
- 25. Primary Hemostatic Disorders Vascular defect : Congenital : Hereditary hemorrhagic telangectasia– E.D.S Acquired : Infections : Meningiococcemia. Allergic : Henoch schonlein purpura Vitamin C deficiency (scurvy) Connective tissue disorders SLE - (steroids) senile pupura Platelet defect : platelets thrombocytopenia: 1-Decreased platelet production 2-Increased destruction 3-Abnormal distribution ( spleen sequestration > 1/3 ) 4-Hemodilution (massive transfusion)(collocrystblood wout plat. Platelet Dysfunction Thrombasthenia
- 26. : Primary Hemostatic Disorders Congenital : TAR Syndrome (thrombopoietin receptor defect) ? Fanconi anemia (defecient all stem cell receptors) Acquired : BM suppresion (drugs-toxins-irradiation )or infiltration(leukemia – lymphoma ) – VIT B12 & FOLIC ACID Immune mediated : ITP - EVAN $ - DRUGS - SLE – HIT –post transfusion ? - Infections EBV, HIV Non immune mediated : DIC ?- TTP ?- HUS – HELLP $ hypersplenism . Kaselbach merrit syndrome(big hemangima) thrombocytopenia
- 27. Primary Hemostatic Disorders platelets (thrombocytopenia) : Platelet Dysfunction Thrombasthenia( discuss) Glanzmann’s normal platelet morphology - defect of platelet aggregation (abscent GP2b3A) ( the worst) Bernard solier abnormal platlet morphology (giant platelet ) defect in plateletadhesion (abscent GP1b) Von willebrand disease ( quantitative or qualitative deficiency of vWF which -binds to exposed subendothelial collagen - -mediates initial platelet adhesion Aspirin ( ANTI PLATLET ) Renal failure Heparin in high doses
- 28. Secondary Hemostatic Disorders Coagulopathies : Hemophilia A (factor VIII defeciency , classic hemophilia ) Hemophilia B (factor IX defeciency , christmas disease ) Hemophilia C (factor XI defeciency ) Von willebrand disease Congenital afibrinogenemia Vit. K defeciency Liver disease DIC ( consumptive coagulopathy) Anticoagulants : Oral anticoagulants – Heparin Factor XIII defeciency.
- 33. Pathogenesis E coli bact GE verotoxin(only from this strain rapidly absorbed blood activate coagulation system thrombus fibrinolytic act disolve this thrombus repeated consumption of platelets Toxins hemolysis of Rbcs MAHA Form immune complex in kidneys acute GN---RF More in children -- pregnant HUS
- 34. Allergic vasculitis after viral infection or drug More in males 2:1 Any age but more common 2-8years اماكن اربع بتاخد2و اساسين2اختيارين 1- extremeties (purpura) special distribution LL – buttoks - extensor surface of forearm rest of body clear اىpurpura ومتتحسش تتشاف النها وتتحس تتشاف دى االallergic فيه يعنىedema palpable purpura Itchy 2- joint affection--- arthritis-arthralgia 3- kidney acute GN or any picture of kidney affection 4-git vasculitis ----abdominal colic –diarrhea Bloody diarhea ------intussception investigation Cbc normal - Bt prolonged - ct normal - plat normal - pft normal Complication intussception renal failure Ttt self limited steroides ------ if joint affection never aspirin HSP anaphylactoid purpura
- 35. Most common cause of TCP AB against platelete Previous viral infection 2-3 weeks earlier (change cell antigenicity) (m-cp-eb vacc) Cp 1- Hx of viral infection 2-3 w 2-skin bleeding (petichea-purpura-ecymosis-( non blanching) 3- MM bleeding we fear of ICH 4-important noooooos no pallor no organomegaly no LN investigations BT prolon cbc dec plat BM inc megakaryocyte Ttt No active bleeding count >40000 follow up cbc / week <40000 or serious HGE 1- steroides (prednisolone) 2mg/kg/day max 60mg 2- IVIG (Blocking AB) (suppress AB dependant cytotoxic cells) 3-Anti D ab 4- plasmapharesis Chronic itp Not responding to ttt >6m (SLE-HIV-HL-EVAN $) ITP
- 36. A DEF F ( 8 ) B DEF F ( 9 ) C DEF F ( 11 ) A & B x linked inheritence c AR A & B more in males Clinically since birth After delivery bleeeding from umbilical cord - Circumcision - after injection of vit k Then bleeding from anywhere - Echymosis without purpura or petiche - minor trauma hematoma - Hemoarthrosis fibrosis joint stifness - Subperiosteal hge (areas w no muscles fibrosis calcification as bone (hemophillic pseudo tumor) hemophilia
- 37. hemophilia Complication 1- Intracranial HGE 2- comp of bl transfusion 3- heamarthrosis 4- factor replacement 4 life 5- with time AB against factor Investigations BT norm PT norm PTT prolon factor assay Treatment 1-avoid precipitating factor 2-factor replacment A FFp 1 all f b cryo 5-10 VIII - fib c fv111 conc 20-25 VIII -vwf vasopressin 3- ttt of comp 4- antifibrinolytic 5 analgesic never aspirin
- 38. Most common inherited bleeding disorder. Cause Defeciency of vwf Liver synthetize factor viiic (int path) (LMW ptn ) carried by vwf (HMW) in order not to be lost if passed through kidney and produce hemophilia A Vwf also play role in platelet adhesion فاكره Investigations Pt normal -- ptt prolonged -- fviiic lost -- BT prolonged PFT Normal ADP with restocetin -Ve add VWF +Ve Treatment as hemophilia A Von willibrand disease
- 39. approach History
- 40. 1- Site of bleeding: * Muscle & joint bleeds indicate a coagulation defect * purpura, prolonged bleeding from superficial cuts, epistaxis, GI bleeding, menorrhagia, indicate PLT disorder. or vW disease History
- 41. 2.Time of onset: congenital or acquired 3.Precipitating cause: if spontaneous indicate severe defect (spont platlet defect. after trauma coagulation defect ) 4.surgery: dental extraction, tonsillectomy, circumcision. Bleeding immediately after surgery indicate defective PLT plug formation. Bleeding after some hours indicate failure of PLT plug stabilisation by fibrin due to coagulation defect. History
- 42. 5. Family history: * Hereditary or acquired * Negative history does not exclude a hereditary cause, as e.g: about 1/3 of cases of hemophilia have negative family history (mutations). 6. Systemic disease review of other systems Hepatic or renal failure CT disease History
- 43. 7. Drugs: almost any drug can potentially produce bleeding( antithrombotic anticoagulant cytotoxics. NSAIDs…etc) • .8 Associated symptoms • Fever - neuro manifest (Meningococcemia) Abdominal pain and arthritic pain ( HSP) 9- Viral prodrome HUS - ITP history
- 45. • Petechiae : minor bleeding in dermis non blanching flat <2mm • Purpura : more than 2 mm and less than 10mm • Ecchymosis ( more than 10 mm in deep layers of skin. • Joint haemarthrosis – arthritis. • Hepatosplenomegaly • Lymphadenopathy • ACTIVE AND PLAYFUL VS. ILL LOOKING • pet or purp w or wout echym platlet or vascular • echymosis only ------coagulation Clinical Examination Clinical approach in making a diagnosis
- 46. Platelet defect Coag.fact defect Site of bl. Skin and mm. Deep in soft –jo-mu Petechiae Yes No Echymosis Small , superficial Large , deep Hemarthrosis Rare Common Hematomas Rare Common Bl.after cuts Early Late Bl.post surgery Early Late :vascular causes
- 49. Primary Hemostatic defect Secondary Hemostatic defect
- 50. investigations
- 51. Clinical approach in making a diagnosis General tests • CBC • BT • PT ,PTT • TT Specific tests • Blood film • Platelet f. tests • Fibrinolysis tests • Cl. factor assay • Mixing test • vWF assay • PFA
- 52. BT prolonged normal vascular platelet cbc Platelet count normaldecreased TCP 1- Vascular 2- thromboathenia Platelet function test normalimpaired Sure vascularthromboathenia
- 53. BT normal Coagulation defect PT PTT PT only EXTRINSIC Factor VII Factor V-II-x-I PTT only intrinsic Factor VIII –IX-XI Factor v – II – x – I - XII (not bleeding) PT&PTT Common pathway VIT K Not liver disease Not DIC Factor VII assay Hemophilic F assay normalPT &PTT FXIII
- 54. thrombocytopenia Peripheral smear Diagnostic Platelet clumping Pseudo TCP Primary marrow disorder (blast) Collect with Heparin not EDETA BM ASPIRATION Primary marrow disorder Leukemia Lymphoma Myeloma MDS MPD Aplastic anaemia If pregnant Sgpt HB RETIC HELLP
- 55. Peripheral smear scistocytes spherocytes Retic - LDH-BIL-HB Confirm hemolysis Coomb’s Test Negative positive Evan syndrome D Dimer In TTP Positive DIC Negative ADAMTS13 Normal in HUS
- 56. Peripheral smear Large plateletes ITP B S D GP 1b Contract plat wall MCV hyper SEG neutrophil VIT B12 &folic acid
- 57. Peripheral smear Non Diagnostic ISOLATED TCP YES ITP DITCP NO BM ASPIRATION Primary marrow disorder Leukemia Lymphoma Myeloma MDS MPD Aplastic anaemia CELLULAR HYPOPLASTIC