malignancies

Question 1

Auer rods (multiple)

Answer 1

AML (APL)

Question 2

basophilic stippling

Answer 2

increased or defective RBC production

Question 3

nRBC

Answer 3

bleedin/hemolysis
primary myelofibrosis (messed up BM architecture)

Question 4

Howell-Jolly body

Answer 4

sign of asplenia (left over nuclear fragments not cleared)

Question 5

ring sideroblasts

Answer 5

MDS: refractory anemia with ring sideroblasts

Question 6

mononuclear Megs

Answer 6

MDS with del5q

Question 7

smudge cells

Answer 7

CLL

Question 8

what are forward scatter and side scatter proportional to on flow cytometry?

Answer 8

FS: size of cell
SS: granularity/segmentation of nuclei

Question 9

what three commonly mutated molecules play a role in the epigenetics of acute leukemias?

Answer 9

Tet1/2: normally demethylate cytosines, deactivating mutation=no differentiation
WT-1: localizes Tet to target genes, deactivating mutation=no differentiation
IDH: makes alpha-kg which blocks Tet, gain of function mutation blocks Tet too much=no differentiation

Question 10

what cytogenetics are associated with poor prognosis in AML? intermediate risk?

Answer 10

• del or monosomy of chromosomes 5 and 7 (q)

- c-KIT mutation

Question 11

APL: what mutation commonly drives proliferation?

Answer 11

FLT3, a receptor tyrosine kinase

Question 12

what is the most common chromosomal abnormality in B-ALL in children? 2nd most common?

Answer 12

hyperploidy

TEL-AML

Question 13

what is the most common chromosomal abnormality in adult B-ALL?

Answer 13

BCR-ABL

Question 14

BCR-ABL promotes proliferation. what usually inhibits differentiation in ALL with BCR-ABL translocation?

Answer 14

mutated IKZF1 transcription factor

Question 15

what is MLL and what does its translocation result in? what is commonly mutated with it?

Answer 15

MLL=histone methyl transferase, mutation=blocked differentiation
FLT3, tyrosine kinase–proliferation

Question 16

what’s the translocation theme in T-ALL

Answer 16

oncogene to TCR on chromosome 14

Question 17

what are 4common causes of leukocytosis beside infection or myeloproliferative disease?

Answer 17

smoking, obesity, corticosteroids, lithium

Question 18

what is erythromelalgia and what is it seen in?

Answer 18

red, painful digits due to clogging of small vessels

-P vera and ET

Question 19

what cells are increased in the peripheral blood of AML? CML?

Answer 19

AML: blasts
CML: mature PMNs, bands, (meta)myelocytes, eosinophils, basophils, platelets

Question 20

what cells are increased in the BM of P vera?

Answer 20

erythroblasts, Megs, and granulocyte precursors

Question 21

what causes splenomegaly in P vera?

Answer 21

stagnation and congestions

-late, can cause hepatosplenomegaly due to extramedullary hematopoiesis after BM fibrosis occurs

Question 22

how might you treat P vera?

Answer 22

• phlebotomy
• aspirin to prevent platelet aggregation
• hydroxyurea, IFN, or busulfan to prevent hematopoiesis
• Jak inhibitor

Question 23

what kind of anemia may ET cause?

Answer 23

microcytic, hypochromic due to iron deficiency

Question 24

what condition is a strong indicator of ET?

Answer 24

splanchnic or portal vein HTN or thrombosis

Question 25

how might you treat ET?

Answer 25

NOT phlebotomy - hydroxyurea, IFN - anagrelide-specific to Megs

Question 26

what do azacytidine and decitabine do and what are they used for?

Answer 26

hypo-methylating agents to reprogram cells in MDS

Question 27

what is lenalidomide used for?

Answer 27

MDS with 5q- | MM: immunomodular (inhibits immune cells)

Question 28

what is ibrutinib and what is it used for?

Answer 28

BTK inhibitor | -relapses of B cell lymphomas

Question 29

which lymphomas are indolent?

Answer 29

- follicular - CLL/SLL - mycoses fungoides - MALTomas

Question 30

which lymohomas are aggresive?

Answer 30

- DLBL - mantle cell - peripheral T cell NOS

Question 31

which lymphomas are highly aggressive?

Answer 31

Burkitt's and T cell lymphoblastic lymphoma

Question 32

CLL is a proliferation of which cells

Answer 32

memory B cells in marginal zone

Question 33

mantle cell lymphoma is a proliferation of which cells? which immunostains are used to diagnoseit?

Answer 33

B cells in mantle zone, not yet activated | -Ki-67 and cyclin D1

Question 34

what differentiates mantle cell lymphoma from follicular and CLL on a slide?

Answer 34

- smaller lymphocytes than the centroblasts of follicular lymphoma - no proliferation centers as seen in activated B cells of CLL

Question 35

what is Burkitt's lymphoma a proliferation of? what does it look like on slide?

Answer 35

memory B cells - cells very blue cytoplasm with cytoplasmic vacuoles and variation in size - starry-sky appearance from macrophages participating in high cell turnover

Question 36

what role does IL-6 play in which cancer?

Answer 36

MM - stimulates growth of plasma cells - high concentration = poor prognosis

Question 37

what two values define diagnosis of MM?

Answer 37

serum M protein > 3g/dL | BM plasma cells > 10%

Question 38

how does MM cause lytic bone disease?

Answer 38

produce DKK1 which stimulates RANKL and IL6 production by osteoblasts and stromal cells - osteoclast differentiation induced - osteoblasts inhibited

Question 39

what is lymphoplasmacytic lymphoma a proliferation of and what condition does it cause?

Answer 39

plasma cells Waldenstrom's macroglobulinemia: excess IgM production causes hyperviscosity of blood -blurred vision, changed mental status, headache

Question 40

what does lymphoplasmacytic lymphoma do that MM doesn't?

Answer 40

cause hepatosplenomegaly and lymphadenopathy | -overtakes BM and causes anemia

Question 41

what is follicular lymphoma a proliferation of?

Answer 41

centroblasts and centrocytes in germinal center | -larger with clumpier chromatin than small lymphocytes

Question 42

what chemo combo is commonly used in Hodgkin's

Answer 42

ABVD: doxorubicin, bleomycin, vinblastine and dacarbazine

Question 43

are any cell lineages increased in aplastic anemia? what type of anemia is it?

Answer 43

lymphocytosis: longer lived | normochromic and normocytic with low reticulocytes