Step 2 Heme Onc 1 7-1-2016

ruhland1's version from 2016-07-01 17:22

Section 1

Question Answer
vit C defud PT/PTT/bleeding time/pl8, but dn collagen * up bleed
thrombocytopeniaud PT/PTT, dn pl8, up bleeding time
VWDud pl8, increased bleeding time, ud PT, up PTT (b/c dn VWF * dn F VIII)
Hemophilia Aud pl8/bleeding/PT, up PTT (dn F VIII)
DICdn pl8, up BT, up PT, up PTT
VWD inheritanceAuto Dominant
Hemophilia A inheritenceX-linked recessive
Hemophilia B inheritanceX-linkned recessive
F VIIIintrinsic so up PTT
heparinup PTT b/c antagonizes intrinsic
coumadinup PT b/c anatg extrinsic
VWD dz why up bleeding timeup pl8 dysf func (VWF effect), up PTT b/c up FVIII effect
Idiopathic Thrombocyptopenci Purupuraud PT PTT, up Bleeding, dn PL8
ITP drugsquinine, heparin, H2 blockers
ITP rule outAI dz (lupus), liver dz, HIV
ITP txsteroids and IVIG
when to give pl8 in ITPif <20,000
ITP curativesplenectomy

Section 2

Question Answer
PTthink if affects F VII
PTTthink if affects FVIII , IX
BTif dn pl8, pl8 dysf (dn VWF)
up sx if use ASAVWDz finding
VWDz dzRistovetin low assay (measures capacitiy of VWF to agglut pl8)
plasma mixing studyhelp dx hemophilia, if pt plasma is mixed with normal plasma and corrects PTT * factor def is likely, if not corrected * pt may have clotting factor inhibitor
hemophilia tximmediate transfusion of clotting factors or cyroprecipitate
cryoprecipVIII, XIII, vWF, fibrinogen and fibronectin (does not correct hemophilia B)
OCPtx monorrhagia in female w VWD
desmopressinincreases release of stored vWF and FVIII from pl8

Section 3

Question Answer
CLLasymptomatic leukocytosis (>50K)
males greater than 55 yoCLL
CLL signsS meg, H meg, recurrent infxn, LAD
Auer rodsAML
AML findingfatigue, bruising, HS meg, recurrent infxn, up prevalence with age, pancytopenia
large myeloblasts with notched nucleiAML finding
CMLphilly chr 9:22
middle aged adults with hx od RADsCML up B sx
ALLbone marrow failure, claasic is 2-5 yo with fatigue, bone pain, neuton penia
unilateral painless testi enlargement in kidthink ALL
Hodgkin MC typenodular sclerosisng (80% of cases), up B sx
Hodgkin StagingAnn Arbor
Hodgkin histoowl eyes, mixed cellularity with many eosinophills
Hodgkin nodular sclerosing findingdiffuse band like fibrosis
Ann Arbor Stage 1single LN or 0 LN
Ann Arbor Stage 2two or more LN on same side of diaphragm
Ann Arbor Stage 3both sides of diaphragm
Ann Arbor Stage 4extranodal involve (ie. bone marrow)

Section 4

Question Answer
AML20 40 yo
CML40-60 yo

Section 5

Question Answer
up AFPyolk sac tmx , AFP great for monitoring dz progress
choriocxup BHCG
Leydigadult male 40-50, up Femininzation in men, up precoscioius pubertry (source of androgens * up femininzation)
Sertoli findingintestinal polyposis and muscosal melanin spots (assoc w Peutz Jeghers and Carney's synd)
testic Teratomacystic wityh heterogeneous echoes
tumor lysis syndromeup K up PO4 dn Ca up Uric up ARF, occurs w/in 72 hrs of initating chemo
tumor lysis txppx allorpurinol (dn uric) and IVF to dn renal damage
furosimideonly indic is CHF and edema
why dn Ca in Tumor lysis syndromeup [PO4] * up CaPO4 precip
uric acidmain cuase of ARF in TLSynd (aka Acute Uric Acid Nephropathy)

Section 6

Question Answer
metaphysial tmc with elevation of periostieumosteosacroma (hx of px w/o systemic dz)
Ober testtight IT band (put pt in decubitus, flex knee and internally rotate thigh
diaphysis tmxEwings Sarcoma (onion skin, 11:22)
osteochondromabony outgwoth with cartilage cap, found young men and pxless
osteoid osteomabenign tmx young men, XR shows round lucency
contains the growth pl8metaphysis
osteo sarcomameta to lung (20%) * get chest CT
transferrin saturationratio of serum iron and TIBC ( up if full of FE... duh)
hemachromatosis screenAFP for HCCx (like hep B and C)
Ca-19-9has no role in screening panc cx

Section 7

Question Answer
homocysteineelevated in folate and b12 def
methylmalonic acidelevated in b12 only
see elevated homocyestine and normal methylmalonic acisthink folate def
up homocystein and up methyl malonic acidup b12 def
beri berithiamine B1 def (dry beriberi is down nerves, wet beriberi is CVASC problems)
pernicious anemiaAI destruction of gastric parietal cells, lab is low B12 up homocystein up methylmalonic acis
celiac dzup folate def (folate abs in proximal jejunum) also seen in chrons and tropical sprue
vit K malabsulcerative colitis
zollinger ellsiondn ileum pH * reduced B12 abs

Section 8

Question Answer
Gingko Bilobableeding
Camelia Sinesisjaundic (black/green tea)
St john3A4 induction
Echinacea Goldensea"enhancea" for colds, up rash up nausea, up heptox
PanaXXX Ginsengup adrenergic ag properties
camiliLIVER sinesissupposed chemoprotective and up anti-ox

Section 9

Question Answer
CML histoleukocytosis with excess granulocytes and basophils
CML tximatinib or BM tp in chronic, dasatinib or stem cell TP in blast
CLL histosmudge cell (aka basket cells)
multiple myeoloma txmephalan and prednisone
mephalannitrogen mustard alkylating
Hodking DXneed excisional LN bx (need tissue architecture)
pel-ebstein feveralcohol induced fever specific of hodgkin
hairy cellmononuclear cells with abundant cytoplasm and cytoplasmic projections
CLL survivialcorrelates with Rai Staging system
CLL bone marrow bxrare used but helps prognosis and response to therapy
11 14 bcl-1mantle cell lymphoma
14 18follicular lymphoma
8 14burkitt (c-myc oncogene is translated next to have chain Ig gene on chr 14)
9 22 BCR-ABLCML gene
imantinibCML tx
abiciximabGP IIb/IIIa binder on activated pl8 * dn pl8 agg, use in ACS of PCTA
rituximabtx for Burkitt Lymphoma (CD 20 killer, * kills B cells)
trastuzamabfor HER-2(erb-B2) breast cx
traztuzamb s/ecardiotox

Section 10

Question Answer
sore tonguepernicious anemia (antiparietal AB), also up constip, dn lb 10-15, abd full, dn prprio
celiacgliadin protein intolerance
dermatitis hepetiformisextensor urface rash of celc
ETOHbone marro tox so dn RBC
TRAbthyroid stimulating hormone recp ab of Graves
pernicious dxget intrinsic factore AB
free erythrocyte protopohyrinmeasures effects of Lead on Hgb synth, shows 3 mo period of exposure
mild spleno meg and low hgbthink sickle cell dz
gonococcal tenosynovitisfever, erythema, tender joint, leukocytosis
solid tmx breast age 15-35, up in pregwell defined solid mass=fibroadenoma, need a core needle bx or f/u in 3-6 mo with repeat u/s
cyroablationalternative to surgical excision or fibroademonma
fine needle aspirationperferes in cystic mass, if solid need core needle bx to look at tissue architecture

Section 11

Question Answer
Gauchers basicmost common lysosomal stoarge d/o (def Glucocerebrosidase)
Gaucher findingaccum in BM , liver, spleen * up pancytop up HS meg, recurrent lung infxn
Fabry basicX-link recess (alpha galactosidase def)
bilobed PMNaka Pelger-Huet anomaly
Pelger huet anomalyAuto Dom d/o with Lamina B rec gene mutation, also find post-axial polydacyl, short stature, up risk AML
schistocyteup in mechanical dmg, finding in TTP or HUS "hemlets", in G6PD def and sickle cell
codocyteaka taget cell 9found in liver dz, Fe def, thallesemia, spleen dysf
Fabry sxpx full body or on extremities, htn, angiokertoma, corneal verticillata
Krabbe dz(def galactocerebrosidase) degen of myelin sheath, up sx at 3-6 mo, death age 2
Niemann pick type A or Bauto recessive dn SMPDA1 gene
Niemann pick type Cauto recessive NPC1/NPC2 gene def
Nieman pick pathophysidef of sphigomyelinase, HS meg, abd dist, dystomia
Tay Sachsauto re (hexoaminodase)
Tay Sachs sxinfants, blind, deaf, dysphagia, death age 4
Fabry defalpha galactosiadase
cornea verticillataspecific for Fabry, whorl golden brown or grey in cornea
Gaucger txenzyme replacment enzyme (imiglucerase=cerezyme)

Section 12

Question Answer
ITPanti-pl8 AB * dn pl8 up bleeding time, up petechia
up WBC and dn pl8think leukemia
ITP kid vs adultkid follows URI and resolves, adults it is chronic due to anti pl8 AB
hamburger and dn pl8think Hemolytic Uremic Syndrome
HUSanemia and ARF
breast mass mxpxex then dx then bx
woman preg or less than 30 yo with focal breast sxget targeted U/S
breast mass PXEXif normal f/u in 2- 3 mo
mammographyif >30 yo with new breast complaint, uncommon earlier b/c young have up nreast density
find breast cystget FNA if symptomatic, if solid get core needle bx

Section 13

Question Answer
MC sx Sickle cell traithematureia
sickle cell CVASCR sided HF and pulm htn
bone pxmc sx of sickle cell crisis
chronic vasocculisive crisiup avasc nec of femoral head
sickle cell trait geneone def B-globulin gene
sickle cell trait complicshematuria, renal med cx, splenic rupt at high alt, up DVT
why sickle cell trait up hematuriarenal papillary necrosis
sickle cell hematuria mxobserve, drink fluid
hydroxyureafor sickle cell dz, up production of Hgb Fetal, dn mort
when to tranfuse rbc in sickle cellpersistent hypoxi depsite O2 supplement