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Heme > stuff 1 from saturday- lymphadenitis and non neoplasias > Flashcards

stuff 1 from saturday- lymphadenitis and non neoplasias Flashcards

1
Q

common feature of all forms is activation of MOs and CD8s

A

hemophagocytic lymphohistiocytosis

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2
Q

how does hemophagocytis lymphohistiocytosis happen and the nick name

A

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

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3
Q

familial HLH, and most common trigger

A

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

MCT: EBV

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4
Q

a child with cancer

A

most common: acute lymphoblastic leukemia or acute lymphoblastic lymphoma

highly aggressive

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5
Q

an adult with cancer

A

Chronic Lymphocytic Leukemia or Small LL

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6
Q

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

A

follicular lymphoma: most common indolent lymphoma of adults

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7
Q

BCL6 associated, BCL2 in 1/3.

A

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

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8
Q

strong ass w translos involving MYC

A

burkitt lymphoma, often latently infected by EBV

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9
Q

leukemias and NHLs more likely in adults

A
  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
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10
Q

leukemias and NHLs most likely in children

A

BALLs and TALLs

anaplastic large cell lymphoma (cytotoxic T cells)

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11
Q

teenagers-20 yos

A

thymic lymphomas: TALLs

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12
Q

peake around age 3

A

ALLs

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13
Q

notch 1 more likey found in a

A

t cell neoplasm in children

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14
Q

a child with a lymphoma- most likely genetic mutations

A

PAX5

E2A

EBF

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15
Q

a child with lymphoma (ALLs) most likely translocations

A

t(12;21) involving ET6 and RUNX1

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16
Q

cd1

A

thymocytes and langerhans

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17
Q

tDt +, peroxidase +, positive for glycogen

A

lymphoBLASTs, myeloBLASTs, lymphoblasts

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18
Q

symptoms of the ALLs, and note t cell specific ones

A

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)

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19
Q

factors inficating poor prognosis in ALLs

A
  1. age under 2 or presentation in adolescents/adulthood
  2. peripheral WBC above 100K
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20
Q

factors indicating a positive prognosis for ALLs

A
  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)
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21
Q

TRK mutations in ALLs

A

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

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22
Q

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

A

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

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23
Q

para-aortic nodes

A

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

24
Q

Germinal B cell lymphomas

A

burkitt, diffuse, follicular

25
Q

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

A

CLL: 60+ yo

26
Q

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

A

14; 18 t, follicular lymphoma

27
Q

pathonmemonic for CLL and SLL

A

PROLIFERATION CENTERS:

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

28
Q

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

A

kappa and lambad light chains; indicates neoplasia

29
Q

Most common deletions in CLL

A

11, triosmy 12, 13, 17

30
Q

what kind of chromosomal translocations are present in CLL?

A

none, or almost. very rate.

31
Q

ZAP 70 and Notch 1

32
Q

MLLT mutations

A

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

33
Q

seen in 90% of folliciular lymphomas

A

14;18 translo and MLL2 mutations

34
Q

T cell immunodeficiency associated B lymphoma: ignition.

A

EBV

This is a large B cell lymphoma subtype.

35
Q

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

A

children/young adults. burkitt’s

36
Q

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

A

upregulates enzymes required for the Warburg effect (tumor uses lactic acid and glycolysis to produce itself)

this is burkitt’s

37
Q

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

A

mantle cell lymphoma

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

38
Q

CD23+

39
Q

11;14 translocation

A

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

40
Q

overexpression of cyclin D1

A

mantle cell lymphoma

41
Q

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

A

mantle cell lymphoma

42
Q

the most likely of all NHL to spread

A

mantle cell lymphoma

43
Q

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

A

the mantle cell lymphoma will spread.

44
Q

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

A

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

45
Q

a continuum between reactive lymphoid hyperplasia and full blown lymphoma

A

marginal zone lymphoma

46
Q

does not begin as a monoclonal neoplasm but a polyclonal one

A

marginal zone lymphoma

47
Q

kidney shaped nucleus with bleb like extensions

A

hairy cell leukemia

48
Q

massive splenomegaly with respiratory issues

A

hairy cell leukemia

49
Q

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

A

hairy cell leukemia often presents with a “dry tap”

50
Q

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

A

hairy cell leukemia

51
Q

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

A

massive splenomegaly (splenic sequestration) and pancytopenia

52
Q

hepatic portal triads are often involved

A

hairy cell leukemia

53
Q

increased incidence of monocytopenia caused by______ in the context of hairy cell leukemia

A

mycobacterium avium

54
Q

TRAP

A

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

55
Q

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

A

hairy cell leukemia

Heme (24 decks)

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