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Heme > Lec 16 Pathology of Lymphoma > Flashcards

Lec 16 Pathology of Lymphoma Flashcards

1
Q

What is structure of normal lymph nodes? And which type of cells in each part?

A

cortical follicles = B lymphocytes
paracortex = T lymphocytes
subscapular and medullary sinuses = histiocytes

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2
Q

What does follicular hyperplasia of lymph node suggest?

A

suggests B cell proliferative response

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3
Q

How do you distinguish follicular hyperplasia from follicular lymphoma?

A

greater variation in size and shape of follicles
preserved lymph node architecture
greater cellular heterogeneity

only malignant lymphoma will demonstarte light chain restriction = either lambda or kappa but not both b/c its polyclonal

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4
Q

What are some specific disease associated with follicular hyperplasia?

A
  • syphilis
  • rheumatoid arthritis
  • giant lymph node hyperplasia [castlemans disease]
  • HIV
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5
Q

What does diffuse lymphoid hyperplasia suggest?

A

expansion of T cells; associated with large number of immunoblasts = fully transformed large lymphoid cells with prominent central nucleoli and abundant cytoplasm

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6
Q

What are some common causes of diffuse lymphoid hyperplasia?

A
  • post vaccination and viral
  • dilantin + drug sensitivity
  • angioimmunoblastic lymphadenopathy
  • dermatopathic lymphadenitis
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7
Q

What is angioimmunoblastic lymphadenopathy [AILD]?

A

have fever, anemia, polyclonal hyperglobulinemia

1/3 may progress to overt lymphoma

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8
Q

What are two specific causes of lymph node mixed hyperplasia?

A
  • mononucleosis

- acute toxoplasmosis

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9
Q

What are clinical signs of infectious mononucleosis?

A

fever, pharyngitis, cervical lymphadenopathy

atypical lymphocytes on peripheral blood

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10
Q

What do you see on peripheral smear in EBV?

A

circulating atypical T cells reacting against EBV infected B cells

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11
Q

What are the two compartments of the spleen?

A

white pulp = periarteriolar lymphoid sheeth and follicles

red pulp = cords and sinusids = a macrophage filter for removing abnormal cellular elements from the blood

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12
Q

What is gauchers disease?

A

glucoserebroside accumulation in macrophages secondar to genetic deficiency of glucocerebrosidase

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13
Q

What is neiman pick?

A

sphingomyelin accumulation secondary to sphingomyelinase deficiency

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14
Q

What defines malignant lymphoma?

A

clonal proliferation of lymphocytes confined to lymph nodes or spleen

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15
Q

What is role of EBV in lymphoma?

A

associated with development of burkitt’s lymphoma, post-transplant lymphoproliferative disorders [PTLDs], and hodgkins lymphoma

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16
Q

What is PTLD?

A

post trasnplant lymphoproliferative disorder –> constitutive activation of nuclear transcription factor NF-kappaB

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17
Q

What is role of HTLV1 in lymphoma?

A

associated with adult T cell leukemia/lymphoma

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18
Q

Where is HTLV1 endemic?

A

Japan, west africa, carribean

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19
Q

What is role of HHV8 in lymphoma?

A

associated wtih kaposi sarcoma

also plays a role in primary effusion lymphomas in immunosuppressed pts

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20
Q

What is role of helicobacter pylori in lymphoma?

A

associated with MALT = mucosal associated lymphatic tissue lymphoma [aka extranodal marginal zone B cell lymphoma]

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21
Q

What oncogene and translocation in burkitt’s lymphoma?

A

c-myc oncogene

t(8;14) = translocation of c-myc [8] and heavy chain Ig [14]

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22
Q

What oncogene and translocation in follicular lymphoma?

A

bcl-2 oncogene

t(14;18) = translocation of heavy chain Ig [14] and bcl-2 [18]

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23
Q

What oncogene and translocation in mantle cell lymphoma?

A

bcl-1 [cyclin D1]

t(11;14) = translocation cyclin D1 [11] and heavy chain Ig [14]

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24
Q

What oncogene and translocation in diffuse large B cell lymphoma?

A

bcl-2 oncogene

t(14;18) = translocation of heavy chain Ig [14] and bcl-2 [18]

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25
Q

What are the common features of non-hodgkins lymphoma?

A
  • effacement of node architecture
  • capsular invasion
  • monoclonality
  • involvement of other sites
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26
Q

What is typical clinical presentation of non-hodgkins lymphoma?

A

painless lymphadenopathy
rubbery lymph nodes; non-tender and moveable

more advance –> fever, night sweats, weight loss

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27
Q

What are some signs of extranodal involvement in lymphoma?

A
  • cytopenia due to BM involvement [myelophthisis]
  • bone pain
  • neuro findings
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28
Q

What are the 4 low grade lymphomas?

A
  • follicular
  • marginal zone
  • MALT
  • small lymphocytic
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29
Q

What are characteristics of low grade lymphomas?

A
  • have severeal years
  • almost always have systemic disease and BM invovlement
  • relaspe common
  • not curable
30
Q

What is richters transformation?

A

low grade lymphomas transform to intermediate or high grade

31
Q

What are the 5 intermediate/high grade lymphomas?

A
  • diffuse large B cell
  • mantle cell
  • anaplastic large cell
  • burkitts
  • lymphoblastic
32
Q

What are characteristics of intermediate/high grade lymphomas?

A
  • more rapidly progress

- potentially curable with chemo

33
Q

How do you diagnose lymphoma type?

A

excisional biopsy of lymph node [rather than fine needle aspirate]

34
Q

What are characteristics of small lymphocytic lymphoma?

A

proliferation well differentiated small B lymphocytes = tissue phase of CLL

disease of the elderly

35
Q

What are characteristics of follicular lymphoma?

A

proliferation of B lymphocytes arising from lymphoid follicle

36
Q

What is morphology of follicular lymphoma?

A

have cells with nuclear clefts = small cleaved follicular center cells

37
Q

What is typical presentations of follicular lymphoma?

A

painless waxing and waning lymphadenopathy

38
Q

What is MALT lymphoma?

A

mucosa associated lymphoid tissue lymphoma

characteristically involved GI

39
Q

What are characteristics of diffuse large cell lymphoma?

A

tumor of large transformed lymphocytes resembling blasts; have few cell markers

increased frequency in immunodeficient

40
Q

Who gets diffuse large B cell lymphoma?

A

usually older adults; but 20% in kids

41
Q

Is diffuse large b cell lymphoma curable?

A

yes with aggressive chemotherapy

42
Q

What is characteristic of T lymphoblastic lymphoma?

A

seen in male adolescents
anterior mediastinal mass
can have CNS/BM involvement and leukemic conversion

43
Q

What markers seen in T lymphoblastic lymphoma?

A

TdT and + E rosette

44
Q

What is burkitts lymphoma?

A

small non-cleaved B lymphocytes

45
Q

Who gets burkitts lymphoma?

A

adolescents or young adults

46
Q

What are the 3 types of burkitts lymphoma?

A
  • endemic = african; in kids + EBV; large jaw tumors
  • non-endemic [western] = older children; less frequently EBV; abdomen/pelvis involvement
  • HIV assocaited
47
Q

What is characteristic appearance of Burkitts lymphoma?

A

starry sky = clear areas of macrophages ingesting dead cells

48
Q

What is mycosis fungoides / sezary syndrome?

A
  • T cell lymphomas that involve the skin = cutaneous T cell lymphoma

sezary has diffuse skin involvement and circulating lymphoma cells

49
Q

What are characteristics of adult T cell lymphoma?

A

see lytic bone lesions, hypercalcemia, rash

circulating abnormal flower cells w/ T cell markers

50
Q

What is difference in prognosis lymphomas that express characteristics of germinal center B cells vs activated B cells?

A

better prognosis when express germinal center B cell markers

51
Q

WHat is use of bendamustine?

A

treat low grade lymphomas es in combination with rituximab

52
Q

What is action/use of rituximab?

A

monoclonal antibody binds CD20; used to treat B cell lymphoma esp low grade

53
Q

What is most common regimen for high grade lymphomas?

A

Cytoxan
Hydroxyldanorubicin [adriamycin]
Oncovin [vincristine]
Prednisone

CHOP

54
Q

Who gets hodgkins lymphoma?

A

bimodal = young adults and > 55 yo; associated with EBV; more common in men except nodular sclerosing type

significantly associated with defects in cell-mediated immunity

55
Q

What is pathogenesis of hodgkins lymphoma?

A

have localized single group of nodes wtih contiguous spread

56
Q

Is prognosis better for hodgkins or non-hodgkins lymphoma?

A

better for hodgkins

57
Q

How do you diagnose hodgkins lymphoma?

A

presence of reed-sternberg cells on excisional biopsy of affected node AND normal small lymphocytes, lots of inflammatory reactive cells

58
Q

What is morphology of reed steinberg cells?

A

binucleated, large inclusion like esoinophilic nucleoli, express CD15 and CD30

lack expression of normal B cell markers like CD19 and CD20

59
Q

What type of hodgkins lymphoma expresses CD2?

A

nodular lymphocyte predominant subtype

60
Q

What are chracteristics of nodular sclerosing hodgkins lymphoma?

A
  • affects men and women equally
  • have birefringent collagen bands, lacunar cells = mononuclear RS cells that look like they are surrounded by halo
  • lots of eosinophils
61
Q

What types of cells make up majority in hodgkins lymphoma?

A

majority of cells are reactive

62
Q

WHat is nodular lymphocyte predominant hodgkins?

A

have few RS cells
more indolent than classical
express B cell antigens CD19 and CD20 and have germinal center specific BCL6

rarely epxress CD15 and CD30; few RS cells

63
Q

What are the 4 tpes of classical hodgkins lymphoma?

A
  • nodular sclerosing = have lacunar cells
  • lymphocyte rich = least aggressive
  • mixed cellularity = lots of RS cells
  • lymphocyte depleted = more aggressive
64
Q

What is normal presentation of hodgkins lymphoma?

A

asymptomatic lymph node enlargement usually in neck

B symptoms [fever, night sweats, weight loss]

65
Q

What do you see on lab in hodgkins lymphoma?

A

eosinophils + monocytes
lymhpocytopenia in more advanced disease
ESR elevated

66
Q

Who are most favorable prognostically in hodgkins lymphoma?

A
  • early stage
  • less than 50 yo
  • without large mediastinal adenopathy
  • ESR less than 50
  • no B symptoms
67
Q

What is typical treatment of early stage hodgkins?

A

combo chemo —> 2-4 cycles of ABVD

68
Q

What is typical treatment of advanced stage hodgkins?

A

6-8 cycles of ABVD

69
Q

What is ABVD therapy?

A
  • adriamycin
  • bleomycin
  • vinblastine
  • dacarbazine
70
Q

What is the name of the monoclonal antibody drug against CD30?

A

brentuximab vedotin = anti-tubulin agent linked to CD30 antibody

Heme (20 decks)

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