WBC

Question 1

Where are HSC located

Answer 1

yolk sac - 3rd week - blood cell progenitors
HSC first appear several weeks after (mesoderm)
then liver - 3rd month until before birth
birth until puberty - entire skeleton
after puberty - only axial skeleton

Question 2

two essential properties of HSC

Answer 2

pluripotent + self renewal

Question 3

describe : extramedullary hematopoiesis

Answer 3

during times of stress, HSC are mobilized from bone marrow into peripheral blood. LIVER/SPLEEN

Question 4

define : leukoerythroblastosis

Answer 4

immature precursors in peripheral blood

Question 5

normal bone marrow composition

Answer 5

1:1 – fat : hematopoietic

Question 6

how does bone marrow composition change in hypo vs hyper [plastic] states

Answer 6

more fat in hypo [aplastic anemia]

less fat in hyper [tumors,hemolytic anemia,leukemia]

Question 7

define : agranulocytosis + MCC

Answer 7

significant reduction in neutrophils + drug toxicity

Question 8

lymph nodes in acute vs chronic infections

Answer 8

painful vs painless

Question 9

3 types of myeloid neoplasia

Answer 9

1. myelodysplastic syndrome – ineffective hematopoiesis causes peripheral cytopenias
2. acute myeloid leukemia – increased precursors in bone marrow
3. chronic myeloproliferative disease – elevated peripheral blood counts of terminally differentiated cells

Question 10

3 viruses associated with lymphomas

Answer 10

1. EBC – burkitt lymphoma
2. HTLV-1. – adult T-cell leukemia/lymphoma
3. KSHV/HHV-8 – Kaposi sarcoma + B-cell lymphoma in pleural space

Question 11

3 major classes of lymphoid tumors

Answer 11

1. Hodgkins
2. Non-Hodgkins
3. Plasma cell

Question 12

MC plasma cell neoplasm

Answer 12

multiple myeloma

Question 13

cells in Hodgkins lymphoma

Answer 13

Reed Sternberg

Question 14

precursor B and T cell neoplasms

Answer 14

acute lymphoblastic leukemia/lymphoma (ALL)

1. B-ALLs - childhood acute “leukemia”
2. T-ALLs - adolescent thymic lymphomas

Question 15

MC cancer of children

Answer 15

ALL

Question 16

morphological comparison btw MYELOBLAST vs LYMPHOBLAST

Answer 16

lymphoblasts have more:

1. condensed chromatin
2. less conspicuous nucleoli
3. small amounts of cytoplasm
4. myeloperoxidase negative
5. PAS positive

Question 17

pathogenesis of ALL

Answer 17

chromosomal changes
MC is HYPERPLOIDY >50 chromosomes

increased # of -blast cells suppresses normal fxn of bone marrow leading to anemia, neutropenia, thrombocytopenia.
vomiting, headache, nerve palsies due to meningeal irritation

Question 18

which chromosome aberration is particularly bad in ALL?

Answer 18

t(9,22) Philadelphia chromosome

Question 19

Peripheral B-cell neoplasms

Answer 19

chronic lymphocytic leukemia (CLL)

small lymphocytic lymphoma (SLL)

Question 20

MC leukemia of adults in USA

Answer 20

CLL

Question 21

diagnostic criteria for CLL

Answer 21

absolute lymphocyte count >4000 per mm3

Question 22

what is seen in lymph nodes that is pathognomonic for CLL/SLL?
what is another cell seen?

Answer 22

proliferation centers

smudge cells

Question 23

immunophenotype of CLL/SLL

Answer 23

CD19 CD20 CD23 CD5 and surface Ig

Question 24

CLL/SLL can transform into?

Answer 24

1. prolymphocytic transformation
2. Richter syndrome (diffuse large B-cell lymphoma)
   bad prognosis if this happens

Question 25

MC indolent NHL in USA

Answer 25

follicular lymphoma | PAINLESS

Question 26

chromosomal translocation in follicular lymphoma

Answer 26

BCL2

Question 27

2 cells types seen in follicular lymphoma

Answer 27

1. centrocytes - small cleaved cells, scant cytoplasm ... majority of cells 2. centroblasts - larger cells, several nucleoli

Question 28

Immunophenotype of follicular lymphoma

Answer 28

CD19, CD20, BUT NOT CD5 | BCL2 + (usually negative in normal follicular)

Question 29

molecular pathogenesis of follicular lymphoma

Answer 29

14,18 translocation

Question 30

clinical features of follicular lymphoma

Answer 30

indolent generalized lymphadenopathy incurable

Question 31

most common form of NHL

Answer 31

diffuse large B-cell lymphoma ( DLBCL )

Question 32

molecular pathogenesis of DLBCL

Answer 32

BCL6 dysregulation is the major one | but also BCL2 can occur like in follicular

Question 33

2 important subtypes of DLBCL

Answer 33

immunodeficient large B-cell lymphoma - EBV | **primary effusion lymphoma -KSHV/HHV-8

Question 34

clinical features of DLBCL

Answer 34

rapidly enlarging mass at nodal or extranodal sites | commonly in WALDEYER ring

Question 35

what type of pattern is seen in Burkitt lymphoma

Answer 35

starry sky appearance | -apoptotic cells surrounded by pale macrophages

Question 36

molecular pathogenesis of Burkitt lymphoma

Answer 36

C-MYC on chromosome 8

Question 37

clinical features of Burkitt lymphoma

Answer 37

endemic- jaw | sporadic- ileocecum/peritoneum

Question 38

high levels of IgM that leads to hyperviscosity is aka

Answer 38

waldenstrom macroglobulinemia

Question 39

solitary myeloma aka

Answer 39

plasmacytoma

Question 40

myeloma that is lacks symptoms and has a high plasma M component

Answer 40

smoldering myeloma

Question 41

which interleukin is important for myeloma cells

Answer 41

IL-6

Question 42

what happens to the bone in multiple myeloma

Answer 42

increased RANKL activity leads to increased bone resorption -- hypercalcemia and pathologic fractures

Question 43

what do we see on xray in multiple myeloma

Answer 43

punched out defects ( skulls, ribs, vertebrae )

Question 44

what is M component

Answer 44

monoclonal Ig in the blood

Question 45

define: rouleaux formation

Answer 45

high levels of M proteins causes peripheral blood smears to stick together

Question 46

MC plasma cell dyscrasia

Answer 46

MGUS monoclonal gammopathy of uncertain significance elderly!

Question 47

immunophenotype seen in mantle cell lymphoma | molecular pathogenesis

Answer 47

cyclin D1 | 11;14