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Hem/Onc > Leukemias and Lymphomas > Flashcards

Leukemias and Lymphomas Flashcards

1
Q

Which two Burkitt subsets are associated with EBV?

A

African/endemic (100%) and HIV-associaed (30-40%)

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2
Q

Which lymphoma is associated with HHV8?

A

malignant effusion B cell lymphoma

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3
Q

What CD’s are associated with RSBC’s?

A

CD15, CD30

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4
Q

Which markers are associated with immature cells?

A

CD34, TdT

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5
Q

What CD’s are usually associated with pre-B cells?

A

CD10, 19, 20

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6
Q

Which CD is associated with activated, mature B cells?

A

CD23

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7
Q

Which T cell antigen is associated with B cell CLL/SLL?

A

CD5

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8
Q

T cell CD’s?

A

1, 3, 4, 5, 8

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9
Q

What is CD45 associated with?

A

leukocyte common antigen

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10
Q

What is the most common cancer in children?

A

B-ALL

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11
Q

What population is usually affected by ALL?

A

65, HIspanic males

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12
Q

Spread of ALL?

A

bone pain, CNS, testes, LAD, hepatosplenomegaly

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13
Q

Hyperdiploidy and t(12,21) associated with good Px in what disease?

A

ALL, also age 2-10, low WBC at Dx

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14
Q

Bad Px factors for ALL?

A

hypodiploidy, t(9;22) (worse in AML than CML), t(4;11) = MLL or 11q23 MLL

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15
Q

Most common leukemia of adults in western world?

A

CLL/SLL

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16
Q

Morphology of CLL/SLL?

A

diffusely invaded LN’s with proliferative centers or larger, active lymphocytes; smudge cells in peripheral blood

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17
Q

Immunophenotype of CLL/SLL?

A

CD19, 20, 5, 23; NOT CD10

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18
Q

Manifestation of progression of CLL/SLL?

A

Richter’s syndrome - BAD, progression to DLBCL

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19
Q

CD38, ZAP70, Del 11q22-23, 17p13 associated with poor Px in?

A

CLL/SLL

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20
Q

Which leukemia characteristically disrupts normal immune function?

A

CLL/SLL - hypogammaglobulinemia = bact infections

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21
Q

What cells cause problems in lymphoplasmacytic lymphoma?

A

substantial fraction of tumor cells terminally diff into plasma cells and secrete monoclonal IgM –> waldenstrom macroglobulinemia (congestion, eye issues) — incurable, progressive

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22
Q

There are splenic, nodal and extranodal marginal zone lymphomas. Extranodal is the most common. Most common manifestation?

A
  • MALT - monocytoid appearance, reactive GC’s

- often arise within tissues involved by chronic inflamm disorders (Sjogren, H. pylori)

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23
Q

In MALT lymphoma, what translocation provides resistance to antimicrobial Rx as in H. pylori associated MALT?

A

t(11;18)

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24
Q

What disease is associated with t(11;14)? Oncoprotein?

A
  • mantle cell lymphoma (MIMICKER)

- BCL1 - pro-proliferation

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25
Q

Immunophenotype of mantle cell lymphoma?

A

CD19, 20, 5 (23 and 10-)

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26
Q

Two mantle cell lymphoma variants? Worse Px?

A

Blastoid = BAD, pleomorphic; neither are curable – BAD Px

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27
Q

Translocation associated with follicular lymphoma?

A

t(14;18) = BCL2 - anti-apoptotic
Incurable but indolent
Transformation to DLBCL in 30-50%

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28
Q

Two cell types seen in nodular architecture of follicular lymphoma?

A

centrocytes and centroblasts

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29
Q

Immunophenotype of follicular lymphoma?

A

CD19, 20, 10, 23 (NOT CD5)

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30
Q

Sex distribution in hairy cell leukemia?

A

males 4x females, >40

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31
Q

3 clinical characteristics of HCL?

A
  • pancytopenia >50%
  • splenomegaly
  • marrow fibrosis
    ALSO fried egg cell appearance
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32
Q

Immunophenotype of HCL?

A

CD19, 20, brCD22, CC11c, CD25, CD103

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33
Q

Most common form of NHL? Who does it occur in?

A
  • DLBCL - males, age 60, aggressive

- SLL and follicular lymphoma can transform into this

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34
Q

What proteins are overexpressed in DLBCL?

A

t(14;18) BCL2 (also follicular lymphoma) and BCL6

BUT - do not co-occur

35
Q

Immunophenotype of DLBCL?

A

CD19, 20, 10

36
Q

Prognosis of two DLBCL types?

A

BCL2 - POOR

Better in germinal center type - BCL6, CD10

37
Q

Presentation of DLBCL?

A
  • rapidly enlarging mass at nodal or extranodal site

- BM involvement only seen late in disease

38
Q

Translocation associated with Burkitt’s?

A

t(8;14) = c-myc

39
Q

Presentation of Burkitt’s?

A

3 types; 2 associated with EBV; EXTRAnodal; starry sky appearance (macrophages)

40
Q

What is used as a measure of myeloma cells in MM?

A

beta 2 microglobulin

41
Q

BAD risk factors for MM?

A

ISS II, III
del 13 or aneuploidy
Hypodiploidy (like in ALL)
t(4;14), t(14;16), del 17p by FISH

42
Q

GOOD risk factors for MM?

A

hyperdiploidy (like in ALL)

t(11;14) - cyclin D1 (BCL1 like in mantle lymphoma)

43
Q

Risk of transformation in smoldering myeloma?

A

10% risk/year during 1st 5 years

44
Q

Waldenstrom macroglobulinemia is associated with which disease in adults?

A

Lymphoplasmacytic lymphoma

45
Q

Two diagnostic characteristics of amyloidosis?

A
  • beta pleated sheets

- congo red stain and apple green birefringence

46
Q

Four common deposition-related clinical manifestations of amyloidosis?

A
  • kidney glomeruli obliterated
  • heart conduct/contract issues
  • GI malabsorption, motility
  • tongue - speech, swallowing
47
Q

3 main amyloidoses?

A
  • AL - light chains; most common; multiple organ involvement; BAD = cardiac involvement
  • AA - serum protein AA; made by liver (acute phase reactant) - chronic inflammation (arthritis); 97% renal dysfunction; CARDIAC RARE
  • Abeta - Alz disease; beta protein precursor in CNS
  • B2 microglobulin - hemodialysis related; carpal tunnel/joints/synovium
48
Q

Transformation of MGUS?

A

1-5% per year

49
Q

How does T-ALL differ from B-ALL?

A
  • manifests in adolescent males as thymic lymphoma
  • mediastinal thymic masses occur in 50-70% of patients –> compression of large vessels, airways
  • more likely to be associated with LAD, splenomegaly
50
Q

Manifestations of CD4 T cell lymphoma of skin?

A
  • cerebriform nuclei, pautrier microabscesses
  • Mycosis fungiodes - early Ds in epiderm/dermis (plaques, patches, ulcerating tumors); may progress to LN/BM
  • Sezary - exfoliative erythroderma + blood involvement
51
Q

What disease is characterized by ALK+ t(2;5) and hallmark cells?

A
  • Anaplastic large cell lymphoma - T cell
  • extranodal; high stage but curable; usually young, male
  • CD30+
52
Q

What disease is characterized by prominent HEV’s, polyclonal gammopathy, B Sx, GLAD/L/S/BM/Skin involvement, and EBV+ large cells?

A

angioimmunoblastic T cell lymphoma

53
Q

Which lymphoma is highly associated with EBV, NK cells (CD56), and a lethal midline granuloma via tumor cell vascular invasion?

A

Extranodal NK/T cell lymphoma - more common in Asia, Central/South America

54
Q

What are B symptoms?

A

fever, drenching night sweats, 10% weight loss

55
Q

Presentation of lymphocyte predominance lymphoma?

A
  • young men with isolated cervical or axillary LAD

- may recur, but not aggressive — can transform to diffuse large cell NHL

56
Q

Neoplastic cell in lymphocyte predominance lymphoma?

A
  • popcorn/L&H cell

- mark like B cells - CD45+, CD20+, NO CD 15/30

57
Q

Most common form/best prognosis of HL?

A
  • Nodular Sclerosis
58
Q

Presentation of nodular sclerosis lymphoma?

A
  • deposition of collagen bands that divide LN’s into circumscribed nodules
  • M=F, young adults, lower cervical, supraclavicular, and mediastinal LN’s
59
Q

What HL accounts for a quarter of cases? Presentation?

A
  • mixed cellularity
  • diffuse effacement of LN’s with heterogeneous cellular infiltrate with plentiful RSBC (70% associated with EBV)
  • older, systemic, advanced stage - BUT still goo Px
60
Q

Least common/poorest Px HL?

A

lymphocyte depletion - but highest #cases with EBV = 90%

61
Q

Which HL is characterized by cellular infiltrate of mostly reactive lymphocytes?

A
  • lymphocyte-rich
  • 40% EBV
  • very good to excellent Px
  • can transform to HD
62
Q

Presentation of lymphocyte depletion HL?

A
  • paucity of lymphocytes + relative abundance of RSBC’s
  • 90% EBV
  • mostly in elderly, HIV+
  • advanced stages/systemic = poorest HL Px
63
Q

Auer rods are diagnostic for which leukemia?

A

AML

64
Q

Which translocation is associated with promyelocytic AML? Px?

A
  • t(15;17)
  • distinctive granules, auer rods — DIC risk
  • good if Tx early with ATRA (forces promyelocytes to differentiate)
65
Q

Which two leukemias are associated with basophils?

A

AML with t(6;9) and CML

66
Q

What cytogenetic abnormality is associated with abnormal eosinophilic AML? Px?

A

inv(16) - GOOD Px

67
Q

AML with maturation (M2) and a good Px is associated with which translocation?

A

t(8;21)

68
Q

Px and population of AML with t(9;11); 11q23 translocations?

A

children

better Px than 11q23 in ALL

69
Q

Which AML translocation is associated with basophilia, dysplasia and a poor Px?

A

t(6;9)

70
Q

Px of AML with myelodysplasia?

A

POOR

71
Q

Two good mutations in AML?

A

NPM1 - fish mouth nuclear invaginations, monocytic diff

CEBPA - most commonly AML without maturaiton

72
Q

BAD mutation in AML? Intermediate combo?

A

FLT-3

FLT-3 + NPM1

73
Q

What are two therapy related AML’s? Prognosis?

A
  • alkylating agents – chromosome 5, 7 loss; 5-7 years after therapy
  • topoisomerase related – 11q23; 2-3 years after Tx
  • POOR
74
Q

Translocation in CML? Function?

A

t(9;22) = bcr-abl constitutively active protein kinase that increases prolif but doesn’t block mat

75
Q

Presentation and population of CML?

A
  • 26-60 years
  • GRADUAL onset with SPLENOmegaly (extramed hematopoiesis
  • increased WBC, thrombocytosis in 50%
76
Q

Poor CML outcome?

A

Progression into accelerated or blast phase after 3 years – myeloid or lymphoid; more cyto abnormalities

77
Q

Which myeloproliferative disease is associated with JAK2 in 95% of cases? 50%?

A
  • polycythemia vera

- chronic idiopathic myelofibrosis and essenial thrombycythemia

78
Q

Etiology of fibrosis in chronic idiopathic myelofibrosis? Presentation?

A
  • fibrosis related to GF’s released by abl megakaryocytic
  • extramed hematopoiesis, splenomegaly
  • anemia with tear drop cells
  • Sx mostly from marrow failure
  • Adults, 3-5 year survival
  • JAK2 in 50%
79
Q

In polycythemia vera, how do you know that RBC proliferation is not in response to shortage?

A

low EPO

80
Q

Presentation of polycythemia vera? Progression?

A
  • increased WBC, RBC, platelets
  • Vascular congestion, hepatosplenomegaly, thrombosis etc.
  • gradual transition to SPENT fibrotic phase = CYTOPENIA
  • JAK2 in 95%
81
Q

DDx for polycythemia vera?

A

secondary (altitude) and relative (dehydration) polycythemias

82
Q

Presentation of essential thrombocythemia?

A
  • platelets >600,000 - variable, bizarre appearance
  • enlarged mature megs, large platelets
  • indolent - 12-15 years
  • thrombotic and hemorrhagic episodes
  • JAK2 in 50%
83
Q

Lymphocytosis and reactive lymphs.. think?

A

VIRUS

84
Q

What is a leukemoid reaction?

A

high WBC with immature granulocytes

Hem/Onc (2 decks)

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