blood disorders and thrombophilias practice questions Flashcards
a patient presents with spontaneous bleeding into joints and soft tissue. You find that they have a disease that inhibits clotting factors 8 and 9
What are the possible diagnosis for this patient? What lab findings would you see in this patient? what long treatment would you give this patient? what about for pain control?
hemophilia A or B
you would see increased aPTT but normal PT/INR
plasma derived or recombinant factor concentrates.
most require infusions up to 3x weekly
for arthritic symptoms use celebrex
for other pain use opiod analgesics
a patient presents with a normal PT/INR and CBC. but they have an increased aPTT what is the possible diagnosis and what is acute treatment for these patients?
hemophilia
Hemophilia A mild = DDAVP in minor bleed and DDAVP or FVIII conc in major
Hemophilia B moderate/severe = FVIII conc in minor bleed and in major bleed
Hemophilia B mild/mod/severe = factor IX conc.
A patient is being treated with celebrex, as it is the only medication given to treat arthritic pain that does not interact with his bleeding disorder. what bleeding disorder does he have and what is the top cause of death in these patients? what is the top life-threatening condition?
hemophilia
transfusion-obtained HIV/AIDS and hepatitis/cirrhosis.
intracranial hemorrhage (2nd most common cause of death 1st most life threatening condition.)
A woman presents complaining of excessive menstrual bleeding. She reports she also suffers very frequent nosebleeds that are difficult to stop.
What is the disease this patient likley has?
what lab findings are we likely to see in this patient?
vWB disease.
prolonged bleeding time, aPTT is mildly prolonged in only half of patients. PT should be NORMAL
Which disease has three types? what are the inheritance pattern of each type? what is the defect of each type?
How do you treat these?
vWB disease
type 1 - autosomal dom - quantitative
type 2 - auto dom and rec - qualitative
type 3 - auto rec - profound quant (like hem A)
treatment:
DDAVP (used in mild)
Recombinant vWF
vWF/FVIII
This deficiency is sometimes referred to as hemophilia C. what is it? how do you treat?
Factor XI deficiency
treat with factor XI conc for bleeding
if not available use FFP
A child presents with a sudden onset petechial rash. as well as bruising. what is the tests that you would want to run on this patient to look further into their disease? what is the disease this patient may have?
CBC and peripheral blood smear
if CBC is clear and the only abnormality is a derceased platelet count and EVERYTHING else is normal!!! than she probably has immune thrombocytopenic purpura.
a patient presents with a disease that is treated using corticosteroids as first line therapy. You can also use IVIG, platelet transfusions and a splenectomy to treat this disease.
What disease does this patient have? What are the risk factors that prompt pharmacotherapy in this disease?
Immune thrombocytopenic purpura
risk factors are:
Severe thrombocytopenia (platelets <10,000/microL) with signs of substantial cutaneous bleeding
Moderate thrombocytopenia (platelets <20,000/microL) with mucosal bleeding
Past or anticipated factors that increase bleeding risk (such as recent head trauma)
A patient presents with severe thrombocytopenia and mucocutaneous bleeding 8 days post exposure to Ticlopidine. The mechanism underlying this disease is immune mediated in most cases.
What is the disease this patient is suffering from? How would you treat them?
Drug-related Platelet Destruction
Cessation of the drug will return platelets to normal levels.
In severe rare cases, immunosuppression with corticosteroids as first-line may be necessary (to reduce antibody titers)
two different patients are having surgery. One patient takes aspirin daily, the other takes NSAIDS daily.
they are both having the same surgery and have the same health concerns.
the one taking aspirin mus stop taking aspirin 5-10 days prior to the surgery, while the one taking NSAIDS must only avoid them one day in advance.
Why is there a difference in how long to stop these medications prior to surgery?
Aspirin and NSAIDS are both qualitative platelet inhibitors.
Aspirin is an IRREVERSIBLE platelet inhibitor therefore it malforms the platelets permanently and you must wait 5-10 days for the malformed platelets to die and the new platelets to be created.
NSAIDS are reversible platelet inhibitors therefore platelet function returns within 12-24 hours of taking this drug.
A patient presents with acute onset of altered mental status after having a seizure. the patient does not have hx of seizures. it is found that she is anemic and has a fever.
Labs show the patient has high fibrinogin and bilirubin. But normal PT, aPTT and LDH.
what is the mehcanism of action for this disease.
Thrombotic thrombocytopenic purpura
Patients with TTP have unusually large multimers of von Willebrand factor (vWF) in their plasma and lack a plasma protease that is responsible for the breakdown of these ultralarge vWF multimers (ADAMTS13) or have antibodies against the ADAMTS13 molecule
A patient presents with the following labs:
Platelets: decreased
Hgb: deceased
WBC: normal or elevated
PT: normal
PTT: normal
fibrinogin: increased
LDH: normal
Bilirubin: increased
The peripheral smear shows moderate to severe schistocytes
what disease does this patient likley have?
How would you treat this patient?
thrombotic thrombocytopenic pupura
plasma exchange/plasmapheresis with FFP.
mortality rate is >95% without treatment!
if inefffective could use rituximab, IVIG, splenectomy, vincristine, cyclophosphamide ect
