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APPROACH TO A BLEEDING PATIENT

APPROACH TO A BLEEDING PATIENT. Dr. CENGİZ CANPOLAT. How do the patients present. 1- referral from a family physician or pediatrician 2- detection of a bleding problem in a family member and bringing the child over for investigation

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APPROACH TO A BLEEDING PATIENT

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  1. APPROACH TO A BLEEDING PATIENT Dr. CENGİZ CANPOLAT

  2. How do thepatientspresent 1-referralfrom a familyphysicianorpediatrician 2-detection of a bleding problem in a familymemberandbringingthechildoverforinvestigation 3-detection of an abnormalphysicalorlaboratoryfindingduringroutineclinicvisit

  3. How do thepatientspresent 4-detection of an abnormality in preoperativelabtests. 5-unexplained bleedingduringoraftersurgeryorfollowing a trauma 6-presentationto ER with an activebleeding

  4. Howtoapproach • Detailedhistory • Systemic PE • Labtests

  5. History • Helpstodeterminewhetherthecondition is hereditaryoracquired • Helpstopredictwheretheabnormalitylies in -vesselwall? -platelets? -clottingfactors?

  6. History • Specificquestionsshould be asked: -is thereexcessivebleedingduringdentalprocedures, circumcision, orminorcuts? -is there a history of spontaneousechymosis, intramuscularorintraarticularbleeding? -is there a history of bloodorbloodpruducttransfusion?

  7. Agewhenthefirstsymptomsbegin • Newborn: cephalhematomaincreasing in size maysuggest severe hemophiliaorhemorrhagicdisease of thenewborn • Umblicalbleedingmayalsooccur in both • Bleedingoccurs in thefirstweeks of life in onethird of thehemophiliapatientsand in lessthen 10% of theotherherediteryclottingdiseases

  8. Bleeding in thefirstfewmonths of life • Hemorrhagicdisease of thenewborn • Intrauterineinfections (TORCH) • Maternal ITP, SLE • Maternaldrugexposure • TAR syndrome • Congenitalamegakaryocyticthrombocytopenia

  9. Age • Hemophilicbleedingstarts at age 1-2 • ITP is usuallyseenbetween 2-4 years of age • Henoch-Schönleinpurpurapeaks at age 4-7

  10. Bleedingtypeanddissemination • Petechia, purpura, echymosis • the size, numberanddissemination as well as whethertheyoccuredspontaneouslyorafter a traumashould be takenintoaccount • occurrence in areasremotefromtraumasuch as trunkorbackshouldsuggestbleedingtendency

  11. Petechia • on legswherethevenouspressure is high • in theheadandneckarea in thecryingchild • Pressureareasfromthebeltorsocks • Areaswherethechildscratches • On themucousmembranes (wetpetechia)

  12. bleedingtypeclottingfactor def. Plateletandvascular Petechiararetypical Deephematomatypicalrare Ecchymosisfreq. generallybig ,singletypical, gen. smallmultiple Hemarthrosistypicalrare prolongedbleed. Freq. rare bleed. superf. cut Minimal Persistant, freq. heavy sex 80-90%malefreq. in females familyhistory + rareexceptvWDand HHT

  13. Nutritionandmedicationhistory • Especially aspirin and NSAID thatimpairplateletfx • Manyotherdrugs can alsocausethrombocytopenia • Prolongeduse of widespectrumantibioticsmayresult in vit K deficiency

  14. Nutritionandmedicationhistory • Ptswithmalnutritionormalabsorbtionmayhavealterations in thelevels of theclottingfactorsdependent on vit K • Bleeding on the skin may be secondarytovit C deficiency • Bleedingtendencymaytakeplace in the presence of liverorkidneydisease.

  15. Proceduresandsurgery • Absence of significantbleedingduringdentalextraction, circumcision, surgeryandmajorinjuriesmay! ruleouthemostaticproblems

  16. Clinicalpresentation • Echymosis on the skin as well as painfulswelling in thejoints in a toddlersuggesthemophilia • Echymosisandpetechiafollowing a viralinfectionwithoutactivebleeding in an otherwisewellchildsuggests ITP

  17. Clinicalpresentation • Repetitiveexcessivemenstrualbleedingandbilateralepistaxis in a 16 yo femalemay be duetovWD • A childpresentingtothe ER withfevernuchalrigidity, shockandmucocutaneouspurpurashouldalertthephysicianfor DIC secondarytomeningococcemiaor gram (-) sepsis.

  18. Clinicalpresentation • Femalept in her seconddecadewithinsidiousrecurrentpetechiaandechymosisand a history of autoimmunedisease in thefamilysuggestchronic ITP • Malignantdiseaseshouldalso be kept in mind in childrenpresentingwithbleeding. Bone orjointpain, weightloss, hepatosplenomegaly, lymphadenopathy, abdominaldistensionorpalpablemassesshould be carefullyevaluated

  19. Mensturalbleeding • Prolongedandexcessivebleeding is typicalforplateletdisordersorvWD • Use of oral contraseptives, beinganemic, usingironmedicationforirondeficiencyarecluestosuspectprolongedmenses

  20. Hemarthrosis • Typicalbleedingforhemophilia • Mostcommonlyaffectedjointsareknee, elbowandankle • Pain, swellingandrestriction in motionoccur. Skin is tense andwarm • Depending on theseverity of thedisease, it’susuallyseenaroundage 1-2 • Recurrentbleeding in a singlejointmayleadtotargetjointandchronicsynovitis

  21. Othertypes of bleeding • Epistaxisandgingivalbleedingarefrequentlyseen in plateletdisordersandvWD • Prolongedwoundhealing, formation of abnormalscartissue, latefalling of theumblicalcordmay be secondarytoafibrinogenemia, dysfibrinogenemiaor F XIII deficiency

  22. Rarebleedingtypes • melena • hematemesis • hemoptisia • hematuria, • retinalbleeding • retroperitonaloriliopsoasmusclebleeding • CNS bleeding

  23. Familyhistory • Consanguinityshould be asked in terms of autosomalrecessivelyinheriteddiseases • The presence of hemophilia, vWD, otherfactordeficiencies, hereditaryplateletfunctionaldisorders, TAR syndrom, Wiskott-Aldrichsyndromeshould be questioned • Thepossibility of spontaneousmutations in 30-40% of hemophiliapatientsshould be kept in mind

  24. Laboratory • CBC, especiallyplateletnumber • Peripheralbloodsmear • Prothrombine time (PT) • Activatedpartialthromboplastine time (aPTT)

  25. Platelets • Size of theplatelets (MPV) is alsoimportant • Normally MPV 7-11 fL, it is >10 fL in ITP, <6 fL in WAS

  26. Platelets • Macroplateletsareseen in Bernard-Souliersyndrome, May-Hegglinanomalyand, Grayplateletsyndrome… • Microplateletsareseen in TAR syndrome, irondeficiencyanemiaandstoragepooldisease

  27. A patientwithpurpura but normal plateletcount • Vascularreasons (hereditaryhemorrhagictelengiectasia, Ehlers-Danlossyndrome, Henoch-Schönleinpurpura, purpurafulminans, SLE ) or • Plateletfunctionaldisorders

  28. Bleeding time • Measuresthevascularandplateletphase of hemostasis • Difficulttointerpretbecause of theinterperformervariability • Not consideredreliablewhenevaluating a patientwithbleedingtendency. Normal resultdoes not ruleout an abnormalityand it can not be used as a screening test

  29. A patientwith normal plateletnumber but prolongedbleeding time • vWFag, Ristocetincofactoractivity, aPTT, FVIII activityshould be measured • Anyabnormality in thesetestswillsuggestthe presence of themostcommoncongenitalbleedingdisorder, vWD

  30. Not vWD but stillprolongedbleeding time • Plateletaggregationtests (with ADP, epinephrin, collagen, ristocetin) • This test willtellifthereareanyadhesion, aggregationorreleasedefects in theplatelets

  31. Plateletfunctionalabnormalities • InGlanzmannthrombastheniaabsence of aggregationwith ADP, collagenandepinephrin is characteristic • Abnormalresponsetoristocetin is seen in vWDandBernard-Souliersyndrome • Recently, plateletfunctionsaremeasuredwith a device called PFA-100 (PlateletFunctionAnalyzer) andmoreaccurateresultsareobtained

  32. Plasmaphase of coagulation

  33. Teststhatmeasuretheplasmaphase of hemostasis • aPTT , PT, TT (thrombin time) andfibrinogen • aPTTmeasuresintrinsicandcommonpathway, PT measuresexstrinsicandcommonpathway • normal value of aPTT is 20-35 sec. Itmay be as long as 50-85 sec in newbornsandprematurebabies

  34. aPTTprolonged PT normal • Inordertodifferentiatewhethertheprolongation is duetofactordeficiencyorthe presence of an inhibitor, patient’splasma is mixed 1:1 with normal plasmaandaPTT is repeated (mixing test) • aPTTreturnsto normal in clottingfactordeficiency • it remainsprolonged in the presence of heparin, lupusanticoagulant (LA) orantibodiesdirectedagainsttheclottingfactors (anti phospholipidantibodies)

  35. aPTTprolonged PT normal • Deficiencies of Factors VIII, IX, XI, XII, Precallicrein (Fletchertrait), HMWK (Fitzgerald trait), presence of LA orheparineffect • Onlydeficiencies of FVIII, IX and XI can causeclinicalbleeding • aPTTstays normal untilthe F leveldropsbelow 30% andevenuntil 15-18%

  36. aPTTprolonged PT normal • The diagnosis of hemophilia A and B are established by measuring the FVIII and FIX levels • It is classifiedaccordingtothefactorlevel as severe (<1%), moderate (1-5%), andmild (5-25%) hemophilia • Most of thecasesarehemophilia A (80-85%) and severe type (50-70%).

  37. PT prolongedaPTT normal • Typicalfor F VII deficiency • PT is prolongedifthelevels of oneormorethanone of theFactors II, V, VII and X are <40% • Iffibrinogenlevel is <100mg/dl, PT is prolonged • Normal valuefor PT is 11-12,5 sec in childrenandadults, 12,8-14,4 sec in terminfantsand 14,6-18,4 sec. in prematurebabies

  38. PT • Prolongedvalues in infantsnormalizewithin 2-6 months • INR (InternationalNormalizedRatio) is calculatedtopreventthedifferencesbetweenlaboratoriesduetotheuse of differentthromboplastineforthemeasurement of PT

  39. BothaPTTand PT areprolonged • Inthiscase TT is measured. If it is normal; liverdisease, vit K deficiency, coumadineffector presence of anticoagulantsshould be considered • If TT is prolonged, afibrinogenemia, dysfibrinogenemiaand DIC arethepossiblediagnosis • Faktör X, V, prothrombin ve fibrinogen deficiencies or inhibitors against these factors should be investigated • Itmaysometimes be seen in patientswithhighHtc (congenitalsyanoticheartdisease)

  40. Conditions in whichalltestsare normal • mildvWD, mildhemophilia, FXI ve FXIII deficiency, someforms of dysfibrinogenemia • Herediteryhemorrhagictelengiectasia, alergic ve vascularpurpuras • 2-plasmininhibitordeficiency, elevation of thelevels of plasminogenactivator

  41. Kanama diatezi Öykü-FM Trombosit sayısı Normal DüşükTrombositopeni nedenleri Normal PT,APTT,TT Akut hastalık Anormal Evet Hayır Kanama öyküsü Var Yok FVIII,vWag Ricof Çocuk istismarı Normal Anormal Meningokok Purpura fulminans Plt. Disfonk. vWH Varicella Variant vWH Hafif HA veya taşıyıcı Pnomokok Hafif hemofili Hafif DIC Hemofili taşıyıcısı Pro C.S, ATIII eks. FXIII ve hafif FXI eks.

  42. PT, APTT, TT Anormal Hasta ile normal plazma karıştırılır Düzelir Düzelmez Uzun APTT Uzun PT Uzun TT TT APTT Kanama öyküsü Sadece PT uzun 1:1 karışımla RT Yok Var düzelir FVII eks. FVIII,vWag FVIII, FIX, XI Oral antikoag Hipo-disfib vWRicof vWag,vWRicof rinojenemi PT+APTT uzun Normal karışımla Lupus AK Oral antikoag düzelmez Heparin FIX, XI, XII vWH Kc hast Reptilaz N HA veya HB Vit K eks Heparin FXI, XII eks Şiddetli FXI eks. Hafif DIC Rep T uzun Hafif HB veya taşıyıcı FII,V,X eks Fibrin YÜ Prekallikrein veya HMWK eks.

  43. Dikkatiniz için teşekkür ederim

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