luekemia + lymphoma + multiple myeloma

Question 1

tumor cells in bone marrow +/- peripheral blood

Answer 1

leukemia

Question 2

tumor cells in lymph node

Answer 2

lymphoma

Question 3

types of lymphoma

Answer 3

Hodgkin (1 type)

Non-Hodgkin (39 types)

Question 4

Reed-sternberg cells: large cell, bi-lobed nucleus, prominent nucleoli with clearing “owl eyes”, clearing around whole cell (white)
MORE = WORSE prognosis

Answer 4

hodgkin lymphoma

will be on test!

Question 5

localized, single group of nodes
PAINLESS LAD (neck usually)
mediastinal LAD (CT or CXR)
firm, rubbery lymph nodes
B symptoms: low grade fever, night sweats, weight loss (also in TB)
pruritis
associated with EBV: 50%

Answer 5

hodgkin lymphoma

Question 6

bimodal age distribution
1st peak: 20 yo
2nd peak: 65 yo
more common in men
except nodular sclerosing type

Answer 6

hodgkin lymphoma

Question 7

most are B cell lymphomas

Answer 7

Non-Hodgkin lymphoma

Question 8

widespread lymph nodes + other tissues: GI, thyroid, CNS
fewer B symptoms
varying age
some associated with HIV and immunosupression

Answer 8

Non-Hodgkin lymphoma

Question 9

types of hodgkin lymphomas

Answer 9

lymphocyte-predominant type
nodular sclerosing type
mixed cellularity type
lymphocyte-depleted type

Question 10

hodgkin lymphoma
least common, BEST prognosis (mostly lymphocytes, not many Reed sternberg cells)
younger males

Answer 10

lymphocyte-predominant type

Question 11

hodgkin lymphoma
most common
great prognosis (not many Reed sternberg cells)
F=M
young adults
bx: bands of collagen, sclerosis, fibrosis + nodules

Answer 11

nodular sclerosing type

Question 12

hodgkin lymphoma
2nd most common
mix of lymphocytes + RS cells worse prognosis

Answer 12

mixed cellularity type

Question 13

hodgkin lymphoma
lots of RS cells
very poor prognosis

Answer 13

lymphocyte-depleted type

Question 14

types of non-hodgkin lymphoma (B cell)

Answer 14

diffuse large B cell lymphoma
lymphoblastic 
follicular lymphoma
burkitt lymphoma
mantle cell lymphoma
small lymphocytic lymphoma 
marginal cell lymphoma

Question 15

most common type of NHL in adults
most common type of lymphoma in US
elderly
t (14;18): 30%

Answer 15

diffuse large B cell lymphoma

Question 16

most common type of NHL in children

Answer 16

lymphoblastic lymphoma

Question 17

NHL

t(14;18): 90%

Answer 17

follicular lymphoma

Question 18

NHL
t(8;14): activates c-myc oncogene
“starry sky”: lymphocyte sheets (stars), some macrophages (sky) that have ingested apoptotic tumor cells

Answer 18

BurkiTT lymphoma (spells 8…14)

Question 19

types of burkitt lymphoma

Answer 19

endemic: associated with EBV - Africa - jaw lesion
sporadic: pelvis or abdomen
immunodeficiency-associated: HIV

Question 20

NHL
NHL
t (11;14): disrupts regulation of cyclin D →go into S phase quickly

Answer 20

mantle cell lymphoma

Question 21

NHL
lymphoma equivalent of CLL
difference: tumor cells in lymph nodes

Answer 21

small lymphocytic lymphoma

Question 22

NHL
arises in chronically inflamed tissue (outside lymph nodes)
associated with sjogren syndrome, hashimoto thyroiditis, H. pylori

Answer 22

marginal cell lymphoma

H. pylori = MALToma

Question 23

types of non-hodgkin lymphoma (T-cell)

Answer 23

adult T cell lymphoma
mycosis fungoides/sezary syndrome
intestinal (enteropathy-associated) T cell lymphoma

Question 24

NHL
caused by HTLV-1 (associated with IVDU)
cutaneous lesions

Answer 24

adult T cell lymphoma

Question 25

NHL cutaneous lesions if malignant T cells leave skin lesions → blood: sezary syndrome

Answer 25

mycosis fungoides/sezary syndrome

Question 26

NHL | associated with long term celiac disease (lymphocytes chronically in crypts)

Answer 26

intestinal (enteropathy-associated) T cell lymphoma

Question 27

leukemia classifications

Answer 27

acute vs chronic | lymphoid (lymphocytes) vs myeloid (granulocytes: NEB) tumor cells

Question 28

creates mutation bcr-abl (oncogene: mutated gene that encodes cancer, constitutively (always on) activated tyrosine kinase)

Answer 28

t(9;22): philadelphia chromosome

Question 29

drug inhibits bcr-able tyrosine kinase

Answer 29

imatinib

Question 30

activation of c-myc caused by

Answer 30

t(8;14)

Question 31

t(9;22) seen in

Answer 31

CML-always ALL- sometimes AML - rare

Question 32

t(8;14) seen in

Answer 32

BurkiTT lymphoma

Question 33

t(15;17) seen in

Answer 33

M3 type of AML: has lots of Auer rods

Question 34

drug to treat t(15;17) in M3 type of AML

Answer 34

all-trans retinoic acid

Question 35

t(18;21) seen in

Answer 35

AML (trisomy 21: extra copy of 21, greater risk of translocation)

Question 36

t(11;14) seen in

Answer 36

mantle cell lymphoma

Question 37

t(14;18) seen in

Answer 37

follicular lymphoma

Question 38

bcl-2 activation caused by

Answer 38

t(14;18)

Question 39

cyclin D1 activation caused by

Answer 39

t(11;14)

Question 40

``` ↑ WBC count (>30-40,000) as result of stressor (infection!, down syndrome, tetrology of fallot, maligancy, kawasaki disease, heat stroke) mostly neutrophils left shift (5-10% immature bands) ```

Answer 40

leukemoid reaction

Question 41

dysplasia of hematopoietic cells in myeloid tissue (like HPV) pre-luekemic state: risk of transformation to AML asymptomatic ↓CBC → bone marrow bx: dysplasia, disorderd hematopoiesis "Pseudo-pelger-huet anomaly": 2-lobed nuclei connected by thin strand tx: monitor, give GF or transfusions as needed

Answer 41

myelodysplastic syndrome

Question 42

myeloproliferative disorders: neoplastic transformation of single myeloid precursors → monoclonal proliferation of MATURE myeloid cells

Answer 42

polycythemia vera essential thrombocytosis myelofibrosis CML: neoplasm of mature myeloid cells

Question 43

from myeloid precursors

Answer 43

``` monocytes macrophages granulocytes: NEB erythrocytes megakaryocytes/PLT dendritic cells ```

Question 44

from lymphoid precursors

Answer 44

T cell B cell NK cell

Question 45

myeloid precursor cell acquires JAK2 mutation: constituitvely active janus kinase 2 → hematopoietic GF signaling to make more cells

Answer 45

polycythemia vera essential thrombocytosis myelofibrosis

Question 46

``` JAK2 mutation in red cell precursor ↑ RBC mass + normal/low EPO plethora: reddened face pruritis after hot bath/shower splenomegaly hyperviscous blood: headache erythromelalgia: hands/feet are painful, swollen due to episodic clots in vessels ```

Answer 46

polycythemia vera

Question 47

↑ PLT + low thrombopoietin | thrombosis or bleeding (defective PLTs)

Answer 47

essential thrombocytosis

Question 48

fibrosis + obliteration of marrow space | "tear drop" RBC

Answer 48

myelofibrosis vs adipocytes = aplastic anemia

Question 49

most common tumor that arises in bone if > 50 yo

Answer 49

multiple myeloma

Question 50

malignancy of plasma cells that arises in BM ↑ IgG (usually) or IgA lymph node bx: plasma cells (large eccentric nuclei, chromatin gives "clock face" appearance, cytoplasm outside nucleus cleared out "perinuclear hof") monoclonal Ab spike on serum protein electrophoresis (SPEP): Monoclonal M spike Bence Jones proteins: Ig light chains in urine detected on urine protein electrophoresis (UPEP) - BUT no ↑ protein on UA punched out lytic bone lesions on XRAY (swiss cheese) smear: RBC stacked like coins (rouleaux formation)

Answer 50

multiple myeloma

Question 51

4 clues, >40-50 yo: anemia: plasma cells interfere with production of other cell lines in BM renal insufficiency: Ab casts in urine back pain + hypercalcemia: secrete cytokines "local osteolytic factors" that stimulate osteoclasts, inhibit osteoblasts

Answer 51

multiple myeloma

Question 52

associations with multiple myeloma

Answer 52

↑ susceptibility: lymphocytes are defective/deficient | 1° amyloidosis (AL): deposition of Ig light chains

Question 53

``` M spike (IgM) associated with amyloidosis + hyperviscous symptoms NO lytic bone lesions ```

Answer 53

waldenstrom macroglobulinemia

Question 54

solid tumor of plasma cells 2 types: solitary plasmacytoma of bone or extramedullary plasmacytoma (usually head/neck, esp nose) NO lytic bone lesions

Answer 54

plasmacytoma

Question 55

monoclonal proliferation of plasma cells → production of monoclonal Ig (M spike) No symptoms: no end organ damage yet can progress to multiple myeloma (monitor)

Answer 55

monoclonal gammopathy of undetermined significance (MGUS)