ACD 8-7-14


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ACD 8-7-14

  1. 1. History Elderly person could present with a hematuria and incidentally found to have lots of bruising. Then the following labs:
  2. 2. Labs: What’s the diagnosis? 0 Factor V, VII, VIII, I X, X, II <1% 0 VW factor - WNL 0 Reptilase WNL 0 Risotcetin assay WNL 0 PS: dimorphic microcytic RBC, no schistocytes 0 Mixing study abnormal
  3. 3. Acquired Hemophilia Diagnosis
  4. 4. Bleeding History Bleeding Disorder Bleeding Symptoms Platelet defects (primary hemostasis) Clotting Factor Deficiencies (secondary hemostasis) Overview of bleeding events Mucocutaneous (oral cavity, nasal cavity, GI, GU) Deep tissue (joints and muscles) Excessive bleeding after minor cuts Yes Not usually Petechiae Common Uncommon Ecchymoses Usually small and superficial Large subcutaneous and soft tissue hematomas Hemarthroses Uncommon Spontaneous in severe deficiencies or with trauma in moderate deficiencies Bleeding with invasive procedures including surgery Often immediate with degree of bleeding dependent on severity of defect Procedural or delayed bleeding with degree of bleeding depending on type and severity of defect
  5. 5. Physical Exam Petechiae (< 3mm) Purpura (> 3mm)
  6. 6. Physical Exam Ecchymosis Telangectasia
  7. 7. Physical Exam Cushing - striae Senile Purpura
  8. 8. Basic Screening Laboratory Tests CBC  Peripheral Smear  PT/INR- Extrinsic pathway; deficiency of vitamin K, warfarin therapy, liver disease  PTT - intrinsic pathway; to monitor anticoagulation (heparin, parenteral direct thrombin inhibitors) and screen for hemophilia  Thrombin time and fibrinogen level - defects in the common pathway (factor X, V, prothrombin, and fibrinogen.
  9. 9. PT PTT Inherited Acquired High Normal Factor VII deficiency Vitamin K deficiency (mild) Liver disease (early) Warfarin Normal High Hemophilia A or B vWD (if F VIII <40%) Contact factor deficiency (XII, HMWK, prekallikrein) Inhibitor -Non specific (lupus anticoagulant) -Specific (i.e., factor VIII inhibitor) High High Factor X, V, or Prothrombin deficiency Dysfibrinogenemia/Hy pofibrinogenemia DIC Vitamin K deficiency (severe) Liver disease (late) Warfarin (supratherapeutic) Inhibitor (Factor V, thrombin) Normal Normal Factor XIII deficiency Factor XIII inhibitor
  10. 10. DD of prlonged PTT 1. Deficiency 2. Inhibitors 3. APLS 4. Anticoagulant use
  11. 11. Acquired inhibitors of the coagulation system 0 Antibodies that decrease activity or increase clearance of clotting factors. 0 Antibodies to multiple clotting factors ( II, VIII, IX, XI, XII,XIII) – SLE 0 Most common clotting factor affected is Factor VIII.
  12. 12. Predisposing conditions 0 Post partum state 0 RA, SLE 0 Malignancies ( CLL, adenocarcinoma lung) 0 Drug induced ( penicillin, sulfonamides, phenytoin, interferons)
  13. 13. Clinical Features 0 Large hematomas 0 Extensive ecchymoses 0 Severe mucosal bleeding - epistaxis, gastrointestinal bleeding, and gross hematuria. (Spontaneous hemarthroses, common in hereditary factor VIII deficiency, are unusual in those with acquired disease. )
  14. 14. Diagnosis 0 Exclude the use of heparin – Redraw, correct with Protamine, thrombin time and a reptilase time. 0 Inhibitor screen (mixing test) — initial diagnostic test for a factor VIII. Correction of the prolonged aPTT suggests a factor deficiency or VWD, while persistent prolongation of the aPTT indicates the presence of an inhibitor. 0 Addition of phospholipid to serum 0 For inhibitor specificity – Bethesda assay - establishes the diagnosis of a factor VIII inhibitor and quantifies the antibody titer.
  15. 15. Treatment 0 Non life-threatening bleeding and low inhibitor titers - DDAVP at a dose of 0.3 mcg/kg SQ per day given for three to five days. 0 Low titer inhibitors (ie, <5 Bethesda units) - human factor VIII concentrates at high doses 0 Higher titer factor VIII inhibitors or severe bleeding - activated prothrombin complex (eg, factor VIII inhibitor bypassing activity [FEIBA]) or human recombinant human factor VIIa (rfVIIa). (eg, typical FEIBA dose 75 units/kg; rfVIIa median starting dose 90.4 mcg/kg, range 45 to 181 mcg/kg). 0 Immunosuppressive therapy - Prednisone, Cyclophosphamide, Rituximab