heme/onc Flashcards

1
Q

hemophilia A factor

hemophilia B factor

A
A = 8
B = 9
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2
Q

What coag factor will be altered in hemophilia?

A

PTT only

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3
Q

hemophilia: quantity of factor replacement in mild/moderate bleeding
- examples: soft tissue bleeding, muscular bleeding, hemarthrosis, epistaxis)

A

50%

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4
Q

how to replace factor in hemophilia A

A

1U/kg of factor 8 increases factor activity by 2%

  • 25U/kg –> 50% correction
  • 50U/kg –> 100% correction
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5
Q

how to replace factor in hemophilia B

A

1U/kg of factor 9 increases factor activity by 1%

  • 50U/kg –> 50% correction
  • 100U/kg –> 100% correction
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6
Q

hemophilia: what if you don’t have factor?

A

hemophilia A options:

  • cryo: 80-100U factor 8 per bag
  • DDAVP: 0.3 mcg/kg/dose IV increases factor 8 by 3-5x by promoting endogenous factor 8 release
  • FFP: 1U factor 8 per mL (need a large volume)

hemophilia B options:
- PCC: but consider thrombogenic risks

pt w/inhibitors to exogenous factor:

  • recombinant factor 7a: directly activates factor 10, bypassing need for factors 8/9
  • FEIBA (factor 8 inhibitor bypassing agent)
  • both of these options are potentially thrombogenic*

TXA: 10 mg/kg IV or 25 mg/kg PO for mucosal bleeds and stabilized clot formation

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7
Q

purpura fulminans is associated with

A

protein C deficiency

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8
Q

DIC tx

A

tx the underlying condition

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9
Q

supratherapeutic INR tx

A

INR < 4 = recheck in 1-2d
INR > 10 = PO Vit K, hold next dose, recheck in 1-2d
INR < 10 w/o bleeding = hold next dose, recheck in 1-2d
serious bleeding at any INR = KCentra

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10
Q

aspirin: mechanism

A

inhibits synthesis of thromboxane A2, a stimulator of PLT aggregation

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11
Q

Distinguish HUS from TTP.

A
HUS = renal dysfunction, younger pt
TTP = neuro dysfunction, older pt
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12
Q

HSP: definition

A

trunk/lower extremity purpura, arthritis, abdominal pain due to IgA-mediated vasculitis

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13
Q

HSP: 2 important complications

A

ileoileal intussusception

nephropathy

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14
Q

HSP: management

A

supportive

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15
Q

definition of neutropenia

A

ANC < 500

ANC = (% neutrophils + % bands) x WBC in thousands

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16
Q

neutropenic nadir

A

7d post-chemo

17
Q

sickle cell: vasoocclusive crisis dx and tx

A

dx: clinical, CBC and retic count to eval for aplastic crisis
tx: supportive, hydroxyurea

18
Q

sickle cell: acute chest syndrome tx

A

supportive, abx, exchange transfusion

19
Q

sickle cell: splenic sequestration crisis sx, dx, tx

A

sx: abdominal pain, pallor, shock, splenomegaly
dx: severe anemia w/elevated retic count to compensate
tx: supportive, pRBC transfusion

20
Q

sickle cell: CVA tx

A

supportive, exchange transfusion

21
Q

sickle cell: where do leg ulcers happen?

A

malleoli

22
Q

sickle cell: priapism tx

A

terbutaline, exchange transfusion, urology cs

23
Q

leukapheresis is a tx for

A

hyperviscosity syndrome in pts w/heme malignancies

24
Q

plasmapheresis is a tx for

A

TTP

25
Q

transient aplastic crisis in a sickle cell pt

A

parvovirus B19

26
Q

polycythemia vera: sx, exam, tx

A

sx: HA, dizziness, post-shower pruritus
exam: HTN, splenomegaly
tx: phlebotomy, hydroxyurea, aspirin

27
Q

tumor lysis labs

A

hyperK, uric acid, phos

hypoCa

28
Q

tumor lysis timing/context

A

1-5d post-chemo for heme malignancy

29
Q

most common immunoglobulins in multiple myeloma

A

IgG > IgA

30
Q

von Willebrand disease: pathophysiology, symptoms, treatment

A

pathophysiology: decreased production of vWF glycoprotein
sx: bleeding, especially mucosal
tx: DDAVP