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Haemoglobinopathy and coagulopathy quiz

Haemoglobinopathy and coagulopathy quiz. A patient presents with generalized fatigue. What are the differentials of this complaint?. If the patient has no other symptoms, what investigations should be performed? FBC Electrolytes BUN Creatinine Glucose Calcium LFTs CXR.

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Haemoglobinopathy and coagulopathy quiz

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  1. Haemoglobinopathy and coagulopathy quiz

  2. A patient presents with generalized fatigue. What are the differentials of this complaint?

  3. If the patient has no other symptoms, what investigations should be performed? • FBC • Electrolytes • BUN • Creatinine • Glucose • Calcium • LFTs • CXR

  4. Is caused by a point mutation in the DNA coding for the a-globulin chain • Is more common in Europe than Africa • Is associated with gall stones • Is associated with hypersplenism • May confer some resistance to malaria • False – B-globulin chain • False – common in W & E africa, mediterranean and middle east • True – due to chronic haemolysis • False – hyposlenism • True – Due to creation of hostile environment for plasmodium in RBC SICKLE CELL DISEASE

  5. 62 year old women • PC: persistent ginigival bleeding after dental surgery 5 hours ago • Application of pressure and ice didn’t stop bleed • Similar episode 6/12 prior – bleeding only stopped after 6 hours of pressure application • What are the differentials of bleeding??

  6. Deficiency or inhibition of coagulation factors • Von willebrands disease • Unfractionated heparin • Direct thrombin inhibitors • Vit K deficiency • Liver disease • Warfarin • Prothrombin, fibrinogen, factor V or factor X deficiency or inhibition • Disseminated intravascular coagulation

  7. What questions would you ask about her history?

  8. Previous response to haemostatic challenges – surgery, childbirth, tooth extractions • Sites of bleeding • Timing of bleeding after trauma • Current and past medications • Thorough systems review – screening for diseases that may disrupt haemostasiseg cancer, liver, renal or thyroid disease • FH of bleeding disorders

  9. Generally in good health • No previous: • Bruising • Epistaxis • Rectal bleeding • Haematuria • Weakness • Fatigue • Light-headedness • Fevers • Athralgias • Dyspnea • Jaundice • Abdo pain • Back pain • Rashes • Confusion

  10. PMH: • 20 yrs ago – DVT whilst on oral contraceptives • 2 previous surgeries with no complications • Meds: • Thiazide • Aspirin/acetaminophen/caffeine pill 1/24 before dental procedure • FH • Lung and bowel cancer • No bleeding problems • SH • Non smokeror drinker

  11. Overweight black women • Vitals normal • Oral cavity – slow oozing blood from molar, no mucosal laceration • No oral petechiae, bullae or ulcers • Mild conjuctival pallor, no icterus • No lymphadenopathy • Lung, heart, abdo, DRE, neuro, skin - normal

  12. What do these clinical findings indicate? • What investigations should be performed?

  13. Hg – slightly lowered • MCV - normal • BUN – high-normal • Creatinine – slightly raised • Glucose – normal • Calcium – high-normal • Total protein – 86 (60-80g/L) • Albumin – normal • LFTs and electrolytes – normal • Urinalysis – normal • PT – normal • aPTT – 49.6s (23.8-36.6) What do these tests indicate?

  14. What are DDxfor increased aPTT and normal PT? • Inhibition/Deficiency of factor VIII, IX, XI • Von Willebrand’s disease • Unfractionated heparin

  15. What causes factor inhibition? • Pregnancy • Thoracic surgery • Certain medications • Viral infections • Lymphomas and other cancers • Autoimmune disorders

  16. Further tests showed: • Reduced factor VIII activity • Reduced plasma von willebrandfactor antigen • Reduced ristocetin cofactor activity What do these tests indicate?

  17. In this case is the von willebrand’s disease more likely to be congenital or acquired? • This patient had acquired von willebrand’s disease • Caused by multiple myeloma A Bloody Mystery. NEJM, Nov 5 2009http://www.nejm.org.ezproxy.library.uq.edu.au/doi/pdf/10.1056/NEJMcps066546

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