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Q1 FBC, PT/aPTT
Q2 Primary hemostasis disorder. bleeding is profuse despite superficial cut, which is more suggestive in abnormalities in platelet plug formation
Q3 PT/aPTT is normal, thus unlikely secondary hemostasis disorder
Q4 bleeding is likely due to platelet abnormality  as vascular bleeding disorders are unlikely to cause profuse bleeding comparing platelet disorder
Q5 there is thrombocytopenia but doesnt correlate with bleeding in this case. in normal person, 30-50x10⁹ is sufficient for hemostasis
Q6 platelet function assay & aggregation tests, VWF antigen, multimer test and ristocetin induced platelet aggregation test.
Q7 large number of Giant plt result in lower platelet count as there is relative lower number of normal small platelets
Q8 abnormal ristocetin test not corrected with addition of VWF suggests defect in Gp1b/IX/V, thus defect in ability of platelt to bind to VWF. Gp1b/IX/V defect, giant plt with thrombocytopenia suggestive of Bernard Soulier Syndrome, which is hereditary disorder with autosomal recessive inheritance
Q9 platelet function test is done to identify possible defects that lead to failure in proper platelet plug formation. Aggregation response to ADP and collagen rules out defect in GPIIB/IIIA; collagen response rules out GP1a defect; adrenaline counteracts thromboxane A2 inhibition; ristocetin normally fascilitate VWF factor binding with GPIb/IX/V
Q10 Bernard soulier syndrome
Q11 molecular genetic test to confirm diagnosis

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What additional testing would be considered?

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10. What is the most likely condition?

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What is the significance of these platelet function tests?

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When asked if he had had bleeding problems in the past,Mr M recounted having petichiae, lots of bruises off and on since childhood, frequent nose bleeds and bleeding for a long time after cuts. Several testa are orders as below:

BT > 20 sec
Closure time on PFA increased
Plt aggregation studies shoed normal aggregattion with ADp, collagen, epineprine and thrombin.
Agglutination with ristocetin was abnormal and was not corrected when VWF was added.
Is this patient’s problem more likely acquired or inherited? Why?

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Mr M hematology report included a finding that at least 65% of the plt were large and giant forms with intense granulation.
Is there a possibility that the patient’s platelet count is spuriously increased or decreased? Why?

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What additional testing would be helpful in identifying the cause of the patient’s excess bleeding?

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Is this profuse bleeding with the presence of petechiae
consistent with the platelet count? Why?

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Is the bleeding more likely related to problems with the vascular system or with platelets? Why?

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Lab result:
WBC 10.5 x 10 9/L                        PT  11.2 sec
RBC 2.3 x 10 12/L                         APTT 25.6 sec
Hb 6.6 g /dL
Hct 19.3
PLT  133 x 10 9/L

Explain how this lab test comfirm that the patient's bleeding is related to a defect in primary hemostasis rather than secondary hemostasis?

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Does the patient most likely have a disorder of primary or of secondary hemostasis? Why

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What lab test, hematology and hemostasis, that would be more informative in interpreting the cause of the patient's bleeding would likely ordered immediately?

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Mr M, a saudi arabian was admitted in A n E after an automobile accident with several superficial cuts and bruises to the head and arms. He was bleeding profusely, having more severe bleeding than would be expected from the nature if his wound.