heme/onc Flashcards
hemophilia A factor
hemophilia B factor
A = 8 B = 9
What coag factor will be altered in hemophilia?
PTT only
hemophilia: quantity of factor replacement in mild/moderate bleeding
- examples: soft tissue bleeding, muscular bleeding, hemarthrosis, epistaxis)
50%
how to replace factor in hemophilia A
1U/kg of factor 8 increases factor activity by 2%
- 25U/kg –> 50% correction
- 50U/kg –> 100% correction
how to replace factor in hemophilia B
1U/kg of factor 9 increases factor activity by 1%
- 50U/kg –> 50% correction
- 100U/kg –> 100% correction
hemophilia: what if you don’t have factor?
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
purpura fulminans is associated with
protein C deficiency
DIC tx
tx the underlying condition
supratherapeutic INR tx
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
aspirin: mechanism
inhibits synthesis of thromboxane A2, a stimulator of PLT aggregation
Distinguish HUS from TTP.
HUS = renal dysfunction, younger pt TTP = neuro dysfunction, older pt
HSP: definition
trunk/lower extremity purpura, arthritis, abdominal pain due to IgA-mediated vasculitis
HSP: 2 important complications
ileoileal intussusception
nephropathy
HSP: management
supportive
definition of neutropenia
ANC < 500
ANC = (% neutrophils + % bands) x WBC in thousands
neutropenic nadir
7d post-chemo
sickle cell: vasoocclusive crisis dx and tx
dx: clinical, CBC and retic count to eval for aplastic crisis
tx: supportive, hydroxyurea
sickle cell: acute chest syndrome tx
supportive, abx, exchange transfusion
sickle cell: splenic sequestration crisis sx, dx, tx
sx: abdominal pain, pallor, shock, splenomegaly
dx: severe anemia w/elevated retic count to compensate
tx: supportive, pRBC transfusion
sickle cell: CVA tx
supportive, exchange transfusion
sickle cell: where do leg ulcers happen?
malleoli
sickle cell: priapism tx
terbutaline, exchange transfusion, urology cs
leukapheresis is a tx for
hyperviscosity syndrome in pts w/heme malignancies
plasmapheresis is a tx for
TTP
transient aplastic crisis in a sickle cell pt
parvovirus B19
polycythemia vera: sx, exam, tx
sx: HA, dizziness, post-shower pruritus
exam: HTN, splenomegaly
tx: phlebotomy, hydroxyurea, aspirin
tumor lysis labs
hyperK, uric acid, phos
hypoCa
tumor lysis timing/context
1-5d post-chemo for heme malignancy
most common immunoglobulins in multiple myeloma
IgG > IgA
von Willebrand disease: pathophysiology, symptoms, treatment
pathophysiology: decreased production of vWF glycoprotein
sx: bleeding, especially mucosal
tx: DDAVP
