cancers

Question 1

CML

Answer 1

myeloproliferative disorder

pluripotent stem cell

9:22, BCR-ABL - Ph22, P210

imatinibi

basophila

chronic - accelerated (10-20% blasts) - blast crisis (AML or ALL)

Question 2

PV

Answer 2

JAK2 (crhom 9)

ruxolitinib

hypervisc syndrome with decreased EPO

prolif phase - spent phase - post poly myelofibroisis - AML(2%)

Question 3

primary myelofibrosis

Answer 3

megakaryocyte cancer

JAK2, calreticulin, MPL

tear drop RBC

treat with ruxolitinib

Question 4

essential thormbocytosis

Answer 4

megakaryocyte cnacer

JAK2, calreticulin, MPL

erythromelagia = hands and feet = red

Question 5

mastocytosis

Answer 5

C-Kit mutation

uticaria pigmentosa

Question 6

AML

Answer 6

t(15:17) = promyolcytic leukemia = ATRA, risk of DIC

auer rods = myeloid blasts

IP: CD34+, 13+, 33+, 117+, TdT-

Question 7

ALL

Answer 7

childhood

bone pain

may or may not see blasts in blood

TdT+, SIg-

t(9:22)-Ph+, t(4:11), t(12;21)

hyperdiploidy (51-65) = good prog

Question 8

CLL/SLL

Answer 8

post germ center memory B cell

smudge cells

CD19+, 20+, 5+, 10-, SiG+

mutated IgVh gene = good prog

treat when symptomatic

transformations: richer (diffsue large B cell lymphoma), polymorphocytic (larger cells in circ)

Question 9

Hairy cell

Answer 9

post germ center B cells

fried egg, reticulin fibrosis

CD 19+, 20+, 5-, 10-, SIg+

CD11c+, 25+, 103+, annexin A+

BRAF mutation

spleen involvemnt always

Question 10

follicular lymphoma

Answer 10

germ center B cells

t(14:18) = overexpress BCL2

no tingible body macs

CD 19+, 20+, 5-, 10+, SIg+

Question 11

Diffuse large B cell lymphoma

Answer 11

centroblasts (in GC) or extrafolliuclalr B lymphoblasts (more aggressive)

extranodal presentation

aggressive

CD 19+, 20+, 5-, 10+/- (could be the same as follicular lymphoma but can tell by morphology)

Question 12

plasma cell myeloma

Answer 12

CRAB (inc Ca, renal probl (bence jones prot), anemia, bone lesions

rouleaux formation

paraprotien

autologous SC transplant

MGUS - 1% transform each year

Question 13

Burkit lymphoma

Answer 13

early GC b cell

endemic, sporadic (US), HIV assoicated

EBV association

tingible body macs = starry sky pattern

t(8;14), t(2:8), t(8:22)

C-MYC

CD 19+, 20+, 5-, 10+, SIg+, TdT- (tells you its not ALL or AML)

fastest growing

Question 14

mantle cell lymphma

Answer 14

pre GC B cell (naive B cell)

CD 19+, 20+, 5+, 10-, SIg+,

cyclin D1+

t(11:14)

Question 15

mycosis fungiodes

Answer 15

T cell neo

patch-plaque- nodule

pautrer microabscesses

sezary syndrome - circulating lymphoid cells in blood

cerebraform nuc on smear

Question 16

adult T cell lekumia/lymphoma

Answer 16

HTLV1 DNA test to diag

flower nuc on smear

Question 17

anaplastic large T cell lymphoma

Answer 17

CD30+

t(2:5)

multilobated nucs in horse shoe

Question 18

Hodgkin lymphoma

Answer 18

young adults

reed stern cells (owl eyes)

A symptoms (LAD, splenomegaly), B symptoms (fever, night sweats, weight loss)

CD 15+, 30+, 20-, Ig-

NF-kB acitavtion

nodular sclerosis (fibrous tissue), mixed cellularity (no fib), lymphocyte depletion, lymphocyte rich (rare)