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SBL > Pathology of White Blood Cells, Lymph Nodes, Spleen, and Thymus (Part 2 of 4) > Flashcards

Pathology of White Blood Cells, Lymph Nodes, Spleen, and Thymus (Part 2 of 4) Flashcards

1
Q

What are the three categories of White cell neoplasia?

A

lymphoid neoplasia, myeloid neoplasia, and histiocytic neoplasia

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2
Q

what are the five broad types of lymphoid neoplasia?

A

B-cell, T-cell, Plasma cell, Hodgkin, NK cell

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3
Q

what are the 3 types of myeloid neoplasia?

A

Acute myeloid leukemia, myelodysplasia, myeloproliferative neoplasia

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4
Q

what is an example of histiocytic neoplasia?

A

Langerhans Cell histiocytosis

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5
Q

what viral infections are common contributors to white cell neoplasms? (3)

A

HTLV-1, EBV, HHV-8

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6
Q

what chronic bacterial infection is a common contributor to white cell neoplasms?

A

H. pylori infections

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7
Q

what is the difference between leukemia and lymphoma?

A

leukemia involves the bone marrow/blood and lymphoma involves the lymph nodes

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8
Q

what is the most common cancer in kids?

A

acute lymphoblastic leukemia

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9
Q

how does ALL present?/ symptoms (6)

A

sudden onset of symptoms/ fever, fatigue, bleeding/bruising, pain, headache, n/v

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10
Q

What occurs in acute lymphoblastic leukemia (ALL)?

A

there are mutations to lymphoid stem cells to create a “leukemic stem cell” with self renewal capacity and simultaneous maturation arrest–> the lymphoid stem cells are shunted to form lymphoblasts

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11
Q

what might happen if the bone marrow is densely “packed” with leukemic cells?

A

they may not aspirate “sludgy”

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12
Q

why do you see a fever in ALL?

A

you’ve wiped out the granulocytes/neutrophils and there is an opportunistic infection

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13
Q

why do you see fatigue in ALL patients?

A

they are very anemic

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14
Q

why do you see bleeding/bruising in ALL patients?

A

they have no platelets

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15
Q

why do patients with ALL experience pain?

A

there is expansion of the leukemic cells inside their bone marrow

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16
Q

why do patients with ALL experience headaches, nausea/vomiting?

A

there are CNS manifestations bc these leukemia will go to the CNS- so there can actually be meningeal involvement

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17
Q

What is the morphology of ALL cells?

A

large cells, dark nuclear chromatin, inconspicuous nucleoli, scant agranular cytoplasm

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18
Q

When trying to diagnose a patient with ALL, what would you look for on flow cytometry?

A

TdT: if positive you can identify that these are prolymphocytes; because either B cells or T cells can have ALL, you need to determine whether it is a B cell or a T cell ALL; B-cell: CD 19, CD20, CD10, Pax-5; T cell: CD1-5, CD7, and CD8

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19
Q

what is the general treatment for ALL?

A

chemotherapy, intrathecal chemotherapy

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20
Q

who has a better prognosis when diagnosed with ALL? (4)

A

age 2-10, low peripheral WBC count, hyperdiploidy, t(12;21)

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21
Q

who has a worse prognosis when diagnosed with ALL? (3)

A

age <2, adolescence/adulthood, high WBC count (>100K)

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22
Q

what is the common demographic for chronic lymphocytic/small lymphocytic lymphoma (CLL//SLL)?

A

patients are typically older and may be asymptomatic or fatigued

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23
Q

what does a typical peripheral smear show in a patient with CLL/SLL?

A

lymphocytosis with small mature lymphocytes; smudge cells may be seen

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24
Q

what are the particular features that present in CLL/SLL? (think flow cytometry)

A

By flow, a clonal (kappa-restricted) population of CD19, CD5+ cells

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25
Q

what is the aggressive phenomena associated with CLL/SLL?

A

richter transformation

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26
Q

what drives the transformation of CLL/SLL into an aggressive tumor?

A

new mutations involving TP53 or MYC

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27
Q

what is follicular lymphoma?

A

a pattern of neoplastic lymphocytes that expand from the follicle center (germinal center)

28
Q

what does follicular lymphoma resemble and what does this mean?

A

resembles follicular hyperplasia; so further testing is needed

29
Q

what are the vast majority of follicular lymphomas associated with? (translocation wise)

A

a classic translocation: t(14;18)

30
Q

what does the translocation (14;18) do?

A

places an IgH gene next to a BCL-2

31
Q

what is bcl-2?

A

an apoptosis suppressor

32
Q

what happens when bcl-2 is placed next to IgH?

A

there is overexpression of bcl-2- so you get less apoptosis- you have now enabled IgH to drive a neoplastic process

33
Q

how can you stain for something you think is follicular lymphoma?

A

there will be a positive brown staining for bcl-2

34
Q

How is follicular lymphoma graded?

A

graded from 1-3; higher grades correlate with higher proportion of large centroblasts

35
Q

what is grade 3 follicular lymphoma related to?

A

diffuse large B cell lymphoma

36
Q

what is diffuse large b cell lymphoma?

A

when large masses form in the lymph nodes or in organs like the spleen; aggressive and fast growing

37
Q

what are the two different types of diffuse large b cell lymphomas?

A

immunodeficiency-related LCL and primary effusion lymphoma

38
Q

what are immunodeficiency-related LCLs linked to?

A

EBV activation

39
Q

what is primary effusion lymphoma associated with and where does it occur?

A

associated with HHV-8 and it occurs in plural fluid

40
Q

what somatic mutation is associated with DLBCLs?

A

BCL-6

41
Q

what is the classic morphology of Burkitt lymphoma?

A

the neoplasm is a B cell neoplasm and it grows in this diffuse sheet

42
Q

what is the cellular composition in Burkitt lymphoma?

A

diffuse growth of intermediate-sized cells with numerous tingible body macrophages

43
Q

what translocation is associated with Burkitt lymphoma?

A

8;14 translocation (IgH/MYC)

44
Q

what occurs in an 8;14 translocation?

A

it allows for an upregulated MYC oncogene to be immediately adjacent to IgH

45
Q

what marker can be used when trying to diagnose Burkitt lymphoma?

A

Ki-67 proliferation marker

46
Q

what are the three different types of Burkitt lymphoma?

A

endemic african burkitt lymphoma, sporadic, and HIV-related

47
Q

what is the endemic african burkitt lymphoma associated with??

A

EBV; mandibular and maxillary growth

48
Q

what is mantle cell lymphoma?

A

malignancy of B cells resembling those in the mantle layer

49
Q

what translocation is associated with mantle cell lymphoma?

A

t(11;14)

50
Q

what occurs in the translocation 11;14?

A

places an IgH next to cyclin D1

51
Q

what is the driver for mantle cell lymphoma?

A

cyclin D1

52
Q

what can you stain for to make the diagnosis of mantle cell lymphoma?

A

an immunostain for cyclin D1

53
Q

where can marginal zone lymphomas arise?

A

in LNs or in extranodal sites (MALT-omas)

54
Q

what are extranodal MALTomas associated with?

A

chronic inflammation

55
Q

what is a gastric maltoma associated with?

A

H pylori infection

56
Q

what can hashimoto thyroiditis result in?

A

mantle zone lymphoma of the thyroid

57
Q

what can be associated with salivary gland marginal zone lymphoma?

A

chronic sialadenitis

58
Q

what does the bone marrow look like in a patient with hairy cell leukemia?

A

back-to-back cells; the hairy cytoplasm keeps a distance between cells

59
Q

what effect does hairy cell leukemia have on the bone marrow?

A

it creates reticulin fibrosis, which can lead to a dry tap on bone marrow aspiration- so patients with hairy cell leukemia have low blood counts

60
Q

what is a common side effect of hairy cell leukemia?

A

splenic involvement is very striking with typical massive splenomegaly

61
Q

what are the markers for hairy cell leukemia?

A

CD25 and CD11c

62
Q

CLL/SLL will show particular features; what is an interesting marker that you can see in association with this particular neoplasm?

A

CD5

63
Q

How can you differentiate between follicular lymphoma and follicular hyperplasia?

A

flow cytometry isn’t a bad idea, but there is something more commonly done: genetic testing for translocation 14;18

64
Q

what is the purpose of tingible-body macrophages?

A

they clear apoptotic debris due to the extreme cell turn over

65
Q

What do patients with hairy cell leukemia present with?

A

splenomegaly but mostly pancytopenia

66
Q

what can flow cytometry demonstrate?

A

clonality and light chain restriction

SBL (14 decks)

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