CME: Bleeding Disorders - Case Scenarios


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CME: Bleeding Disorders - Case Scenarios

  1. 1. <ul><li>2 year old boy had a fall from chair. He developed swelling of right shoulder and upper arm. </li></ul><ul><li>On examination at a hospital the boy had a hematoma of the right shoulder. </li></ul><ul><li>There was no previous h/o surgeries, trauma or medication. </li></ul><ul><li>Following aspiration of hematoma the patient developed profuse bleeding. </li></ul><ul><li>His mother said that the boy’s cousin had a similar bleeding problem. </li></ul>
  2. 2. <ul><li>Investigations : </li></ul><ul><li>Hb- 8 gms </li></ul><ul><li>Hematocrit- 26% </li></ul><ul><li>Platelets- 2 lakhs. </li></ul><ul><li>Bleeding time- normal </li></ul><ul><li>Coagulation profile: </li></ul><ul><li>PT- 12 sec </li></ul><ul><li>aPTT- 60 sec </li></ul><ul><li>Thrombin time- normal </li></ul>
  3. 3. <ul><li>1.Does the lab data support a diagnosis of bleeding disorder? </li></ul><ul><li>2.The tests done shows what type of disorder? </li></ul><ul><li>3.What confirmatory test should be done ? </li></ul>
  4. 4. <ul><li>Answers: </li></ul><ul><li>1. it’s a coagulation defect. </li></ul><ul><li>2. normal platelets, bleeding time. </li></ul><ul><li>APTT prolonged, with normal PT, THROMBIN TIME. indicate a intrinsic pathway defect with normal common pathway. </li></ul><ul><li>There is a defect in factor VIII, IX, XI, XII. </li></ul><ul><li>3.Specific factor assay, factor substitution testing.If factor 8 activity < 1% </li></ul><ul><li>Diagnosis- hemophilia A </li></ul>
  5. 5. <ul><li>21 yr man was admitted for hernia surgery. No h/o of any medical illness, bleeding , or drug intake. Family history ? </li></ul><ul><li>Investigations : </li></ul><ul><li>Hb-15 gms </li></ul><ul><li>Hematocrit-44% </li></ul><ul><li>platelet count –normal </li></ul><ul><li>bleeding time-10 mins </li></ul><ul><li>PT- NORMAL </li></ul><ul><li>APTT- 55 seconds </li></ul><ul><li>Repeat APTT was also prolonged. </li></ul><ul><li>Surgery was postponed for him. </li></ul>
  6. 6. <ul><li>1.What coagulation disorder likely to be present? </li></ul><ul><li>2.what are the other investigations required? </li></ul><ul><li>3.what is the diagnosis if the patient has low normal factor 8 levels and defective ristosetin induced platelet aggregation? </li></ul>
  7. 7. <ul><li>1.Normal PT, prolonged APTT suggest a deficiency of VIII, IX, XI, XII deficiency. </li></ul><ul><li>Since deficiency of XI, XII are rare, this can be due to def of VIII, or XI. </li></ul><ul><li>2. factor substitution study. </li></ul><ul><li>Specific factor assay. </li></ul><ul><li>3. in this factor VIII assay showed 30% activity (n- 50-150%). This finding and lack of bleeding history show he may not have classic factor viii deficiency. </li></ul><ul><li>Further tests showed </li></ul>
  8. 8. <ul><li>Bleeding time prolonged. </li></ul><ul><li>Platelets aggregation decreased. </li></ul><ul><li>Factor VIII/ VWF decreased. </li></ul><ul><li>the lab findings support a diagnosis of von willebrand disease </li></ul>
  9. 9. <ul><li>62 year old with abnormal bleeding was admitted for dental surgery. His brother had died of traumatic bleeding following a car accident. </li></ul><ul><li>I- AT 7 YEARS following a lymph node resection. </li></ul><ul><li>II- at 30 years the pt had 3 weeks of bleeding following dental extraction. </li></ul><ul><li>At 31 years the pt received blood transfusion before appendicectomy. </li></ul><ul><li>At 59 years the pt developed gi bleeding following surgery for hiatus hernia. He received blood transfusions -4 units. </li></ul>
  10. 10. <ul><li>Lab data : </li></ul><ul><li>Hb-7 gms </li></ul><ul><li>Hematocrit-23% </li></ul><ul><li>Platelet count- 4.98 lakhs </li></ul><ul><li>Bleeding time-2mins </li></ul><ul><li>Clotting time-188 mins. </li></ul><ul><li>APTT- 53 sec( control- 35 sec) </li></ul><ul><li>PT- 13.8 SEC( control-13 sec). </li></ul><ul><li>Specific assay for factor VIII, IX Showed factor IX level less than 5% </li></ul>
  11. 11. <ul><li>1.what is the diagnosis? </li></ul><ul><li>2. can the patient safely undergo surgery? </li></ul>
  12. 12. <ul><li>1. hemophilia –B </li></ul><ul><li>2.yes. </li></ul><ul><li>He should receive factor IX concentrate rather than whole blood/. </li></ul>
  13. 13. <ul><li>22 year female was being evaluated for menorrhagia. Her menses lasted for 8-12 days. The patient also had several nose bleeds which needed cautery. She also reported that her mother and 2 sisters also had long menstrual periods, and her brother needed transfusion following an appendicectomy. </li></ul><ul><li>On examination the patient was pale and had large bruises in the extremities. </li></ul>
  14. 14. <ul><li>Lab data : </li></ul><ul><li>Hb-10 gms </li></ul><ul><li>Hematocrit-27% </li></ul><ul><li>platelet count – 2.5 lakh/cumm. </li></ul><ul><li>Bleeding time-7 mins( n- 1to 3 mins) </li></ul><ul><li>PT- 11 SEC (control-12 sec) </li></ul><ul><li>APTT- 29 sec( control-34 sec) </li></ul>
  15. 15. <ul><li>1.What is the likely abnormality? </li></ul><ul><li>2.What are the further tests required? </li></ul><ul><li>3. what is the most likely diagnosis if the patient has defective platelet aggregation? </li></ul>
  16. 16. <ul><li>1.Thrombasthenia </li></ul><ul><li>2. Platelet function studies </li></ul><ul><li>3. the patient had deficient platelet aggregation. A diagnosis of Glanzmann’s thrombasthenia was made . </li></ul><ul><li>Autosmal recessive disorder. </li></ul><ul><li>Platelet concentrate is needed during surgeries. </li></ul>
  17. 17. <ul><li>A woman was admitted in labour in an obstetric ward. Pt had no significant history and examination was normal. The patient had irregular contractions. </li></ul><ul><li>In the delivery room she developed profuse bleeding. </li></ul>
  18. 18. <ul><li>Hb-10 gms </li></ul><ul><li>Hematocrit-27% </li></ul><ul><li>Platelet count -75000 </li></ul><ul><li>Bleeding time-10 mins </li></ul><ul><li>PT-19 SECONDS- ( control-13 sec). </li></ul><ul><li>APTT- 65 sec( control-35 sec) </li></ul><ul><li>Thrombin time-30 sec( n- 18-22 sec) </li></ul><ul><li>Fibrinogen- 90 mg/dl (200-400 mg/dl) </li></ul><ul><li>Fibrin split product- positive </li></ul>
  19. 19. <ul><li>1. what is the probable diagnosis? </li></ul><ul><li>2. what is the probable etiology? </li></ul><ul><li>3. will whole blood transfusion repress the bleeding? </li></ul>
  20. 20. <ul><li>1. the diagnosis is DIC. </li></ul><ul><li>2. may be due to release of placental tissue in to maternal circulation triggering the coagulation mechanism. There is evidence of fibrinolysis. </li></ul><ul><li>3. transfusions may temporarily help. Heparin may be useful </li></ul>