stuff 1 from saturday- lymphadenitis and non neoplasias

Question 1

common feature of all forms is activation of MOs and CD8s

Answer 1

hemophagocytic lymphohistiocytosis

Question 2

how does hemophagocytis lymphohistiocytosis happen and the nick name

Answer 2

MOs eat up blood cell progenitors in marrow and formed elements in circulation. cytokine storm –> mediators suppress hematopoiesis and –> systemic inflammation occurs “shock like syndrome”

cytopenia, thrombocytopenias

macrophage activation syndrome

Question 3

familial HLH, and most common trigger

Answer 3

familial hemophagocytic lymphohistiocytosis: CD8 and NK cells cant deploy cytotoxic granules–> acute febrile illness, organomegaly, hemophagocytosis of BM

MCT: EBV

Question 4

a child with cancer

Answer 4

most common: acute lymphoblastic leukemia or acute lymphoblastic lymphoma

highly aggressive

Question 5

an adult with cancer

Answer 5

Chronic Lymphocytic Leukemia or Small LL

Question 6

most cases associated with the 14; 18 translo and overexpression of BCL2

Answer 6

follicular lymphoma: most common indolent lymphoma of adults

Question 7

BCL6 associated, BCL2 in 1/3.

Answer 7

most common lymphoma of adults: diffuse Large b cell lymphoma, may arise from follicular lymphoma

Question 8

strong ass w translos involving MYC

Answer 8

burkitt lymphoma, often latently infected by EBV

Question 9

leukemias and NHLs more likely in adults

Answer 9

1. diffuse large b cell lymphoma
2. folliciular lymphoma
3. hairy cell lymphoma
4. mantle cell lymphoma
5. Mult Myel/solitary plasmacytoma
6. small lymphocytic lymphoma
7. extranodal marginal zone lymphoma
8. almost all T /NK cell neoplasms except for anaplastic large cell lymphoma

Question 10

leukemias and NHLs most likely in children

Answer 10

BALLs and TALLs

anaplastic large cell lymphoma (cytotoxic T cells)

Question 11

teenagers-20 yos

Answer 11

thymic lymphomas: TALLs

Question 12

peake around age 3

Answer 12

ALLs

Question 13

notch 1 more likey found in a

Answer 13

t cell neoplasm in children

Question 14

a child with a lymphoma- most likely genetic mutations

Answer 14

PAX5

E2A

EBF

Question 15

a child with lymphoma (ALLs) most likely translocations

Answer 15

t(12;21) involving ET6 and RUNX1

Question 16

cd1

Answer 16

thymocytes and langerhans

Question 17

tDt +, peroxidase +, positive for glycogen

Answer 17

lymphoBLASTs, myeloBLASTs, lymphoblasts

Question 18

symptoms of the ALLs, and note t cell specific ones

Answer 18

symptoms related to reduced marrow- anemia, fever, neutropenia

symptoms related to mass effect: bone pain, LAD, splenomegaly, hepatomegaly, testicular enlargement.

in TALLs specifically: vessel compression (SVC syndrome) and meningeal spread (headaches)

Question 19

factors inficating poor prognosis in ALLs

Answer 19

1. age under 2 or presentation in adolescents/adulthood
2. peripheral WBC above 100K

Question 20

factors indicating a positive prognosis for ALLs

Answer 20

1. diagnosed between 2-10
2. low peripheral WBC count
3. hyperdiploidy
4. trisomy of chroms 4/7/10
5. presence of t(12;21)

Question 21

TRK mutations in ALLs

Answer 21

9;22 philly chrom translation. notable because most of the ALLs are transcription factor mutations.

Question 22

starry sky appearance associated with what mutation and in what groups and in what kind of cells does this appear

Answer 22

8;14 translo in burkitts and ass w/70% adults and 30 of childhood NHLs, reactive histiocytosis

Question 23

para-aortic nodes

Answer 23

american type burkitt lymphoma, mostly adults but also 30% of children w/NHL

Question 24

Germinal B cell lymphomas

Answer 24

burkitt, diffuse, follicular

Question 25

BCL-2 (-), CD5: how old is this person likely?

Answer 25

CLL: 60+ yo

Question 26

BCL2+, CD5- : what is a hallmark mutation in this group?

Answer 26

14; 18 t, follicular lymphoma

Question 27

pathonmemonic for CLL and SLL

Answer 27

**PROLIFERATION CENTERS:** diffuse effaced LN by infiltrate with small lymphocytes, admixed with activated lymphocytes father in loose aggregates referred to as proliferation centers.

Question 28

how can we determine B cell monoclonality? what does that indicate?

Answer 28

kappa and lambad light chains; indicates neoplasia

Question 29

Most common deletions in CLL

Answer 29

11, triosmy 12, 13, 17

Question 30

what kind of chromosomal translocations are present in CLL?

Answer 30

none, or almost. very rate.

Question 31

ZAP 70 and Notch 1

Answer 31

CLL

Question 32

MLLT mutations

Answer 32

histone methyltransferase = regulates gene expression. present in up to 90% of follicular lymphomas

Question 33

seen in 90% of folliciular lymphomas

Answer 33

14;18 translo and MLL2 mutations

Question 34

T cell immunodeficiency associated B lymphoma: ignition.

Answer 34

EBV This is a large B cell lymphoma subtype.

Question 35

BCL2 (-), BCL6+, royal lue cytoplasm containing clear cytoplasmic vacuoles. how old is the patient, probably?

Answer 35

children/young adults. burkitt's

Question 36

all forms are highly ass w/translos of MYC gene on chrom 8. what does this do?

Answer 36

upregulates enzymes required for the Warburg effect (tumor uses lactic acid and glycolysis to produce itself) this is burkitt's

Question 37

a man in his 40s or 50s presents with painless lymphadenopathy and massive splenomegaly. Lymphoma cells are CD20+, but **CD23-**

Answer 37

mantle cell lymphoma CD23- distingushies it from CLL/SLL, which is CD23+

Question 38

CD23+

Answer 38

CLL/SLL

Question 39

11;14 translocation

Answer 39

involves the IgH locus on chrom 14 and cyclin D on chrom 11: overexpression of cyclin D1

Question 40

overexpression of cyclin D1

Answer 40

mantle cell lymphoma

Question 41

reactive germinal centers with a nodular appearance. diffusely efface node. proliferations consist of homogenous populations of small lymphocytes with irregular to occasionally deeply clefted nuclear contous. no centroblasts or centrocytes

Answer 41

mantle cell lymphoma

Question 42

the most likely of all NHL to spread

Answer 42

mantle cell lymphoma

Question 43

big spleen, polyp like lesions in the gut. what is your concern?

Answer 43

the mantle cell lymphoma will spread.

Question 44

patient has massive hepatosplenomegaly and complains that his gluten anti IFN medication isn't working

Answer 44

he has mantle cell lymphoma, lymphomatoid polyposis, and was misdiagnosed with ulcerative collitis.

Question 45

a continuum between reactive lymphoid hyperplasia and full blown lymphoma

Answer 45

marginal zone lymphoma

Question 46

does not begin as a monoclonal neoplasm but a polyclonal one

Answer 46

marginal zone lymphoma

Question 47

kidney shaped nucleus with bleb like extensions

Answer 47

hairy cell leukemia

Question 48

massive splenomegaly with respiratory issues

Answer 48

hairy cell leukemia

Question 49

doctor can't seem to get a marrow sample from a patient whose primary sample returned CD11c +, CD25+, CD103+, annexin A1+

Answer 49

hairy cell leukemia often presents with a "dry tap"

Question 50

90% SHOW activating point mutations with serine/threonine kinase BRAF

Answer 50

hairy cell leukemia

Question 51

what two characteristics of hairy cell leukemia would you expect to find on patients 50% of the time?

Answer 51

massive splenomegaly (splenic sequestration) and pancytopenia

Question 52

hepatic portal triads are often involved

Answer 52

hairy cell leukemia

Question 53

increased incidence of monocytopenia caused by\_\_\_\_\_\_ in the context of hairy cell leukemia

Answer 53

mycobacterium avium

Question 54

TRAP

Answer 54

pathoma says hairy cell leukemia is positive for tartrate resistance acid phosphatase (TRAP)

Question 55

seen best under a phase contrast microscope, and the marrow is involved by a diffuse interstitial infiltrate, and splenic red pulp is highly infiltrated, leading to obliteration of the white pulp beefy red appearance

Answer 55

hairy cell leukemia