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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
Immunophenotype of mantle cell lymphoma?
CD19, 20, 5 (23 and 10-)
26
Two mantle cell lymphoma variants? Worse Px?
Blastoid = BAD, pleomorphic; neither are curable -- BAD Px
27
Translocation associated with follicular lymphoma?
t(14;18) = BCL2 - anti-apoptotic Incurable but indolent Transformation to DLBCL in 30-50%
28
Two cell types seen in nodular architecture of follicular lymphoma?
centrocytes and centroblasts
29
Immunophenotype of follicular lymphoma?
CD19, 20, 10, 23 (NOT CD5)
30
Sex distribution in hairy cell leukemia?
males 4x females, >40
31
3 clinical characteristics of HCL?
- pancytopenia >50% - splenomegaly - marrow fibrosis ALSO fried egg cell appearance
32
Immunophenotype of HCL?
CD19, 20, brCD22, CC11c, CD25, CD103
33
Most common form of NHL? Who does it occur in?
- DLBCL - males, age 60, aggressive | - SLL and follicular lymphoma can transform into this
34
What proteins are overexpressed in DLBCL?
t(14;18) BCL2 (also follicular lymphoma) and BCL6 | BUT - do not co-occur
35
Immunophenotype of DLBCL?
CD19, 20, 10
36
Prognosis of two DLBCL types?
BCL2 - POOR | Better in germinal center type - BCL6, CD10
37
Presentation of DLBCL?
- rapidly enlarging mass at nodal or extranodal site | - BM involvement only seen late in disease
38
Translocation associated with Burkitt's?
t(8;14) = c-myc
39
Presentation of Burkitt's?
3 types; 2 associated with EBV; EXTRAnodal; starry sky appearance (macrophages)
40
What is used as a measure of myeloma cells in MM?
beta 2 microglobulin
41
BAD risk factors for MM?
ISS II, III del 13 or aneuploidy Hypodiploidy (like in ALL) t(4;14), t(14;16), del 17p by FISH
42
GOOD risk factors for MM?
hyperdiploidy (like in ALL) | t(11;14) - cyclin D1 (BCL1 like in mantle lymphoma)
43
Risk of transformation in smoldering myeloma?
10% risk/year during 1st 5 years
44
Waldenstrom macroglobulinemia is associated with which disease in adults?
Lymphoplasmacytic lymphoma
45
Two diagnostic characteristics of amyloidosis?
- beta pleated sheets | - congo red stain and apple green birefringence
46
Four common deposition-related clinical manifestations of amyloidosis?
- kidney glomeruli obliterated - heart conduct/contract issues - GI malabsorption, motility - tongue - speech, swallowing
47
3 main amyloidoses?
- 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
Transformation of MGUS?
1-5% per year
49
How does T-ALL differ from B-ALL?
- 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
Manifestations of CD4 T cell lymphoma of skin?
- 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
What disease is characterized by ALK+ t(2;5) and hallmark cells?
- Anaplastic large cell lymphoma - T cell - extranodal; high stage but curable; usually young, male - CD30+
52
What disease is characterized by prominent HEV's, polyclonal gammopathy, B Sx, GLAD/L/S/BM/Skin involvement, and EBV+ large cells?
angioimmunoblastic T cell lymphoma
53
Which lymphoma is highly associated with EBV, NK cells (CD56), and a lethal midline granuloma via tumor cell vascular invasion?
Extranodal NK/T cell lymphoma - more common in Asia, Central/South America
54
What are B symptoms?
fever, drenching night sweats, 10% weight loss
55
Presentation of lymphocyte predominance lymphoma?
- young men with isolated cervical or axillary LAD | - may recur, but not aggressive --- can transform to diffuse large cell NHL
56
Neoplastic cell in lymphocyte predominance lymphoma?
- popcorn/L&H cell | - mark like B cells - CD45+, CD20+, NO CD 15/30
57
Most common form/best prognosis of HL?
- Nodular Sclerosis
58
Presentation of nodular sclerosis lymphoma?
- deposition of collagen bands that divide LN's into circumscribed nodules - M=F, young adults, lower cervical, supraclavicular, and mediastinal LN's
59
What HL accounts for a quarter of cases? Presentation?
- 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
Least common/poorest Px HL?
lymphocyte depletion - but highest #cases with EBV = 90%
61
Which HL is characterized by cellular infiltrate of mostly reactive lymphocytes?
- lymphocyte-rich - 40% EBV - very good to excellent Px - can transform to HD
62
Presentation of lymphocyte depletion HL?
- paucity of lymphocytes + relative abundance of RSBC's - 90% EBV - mostly in elderly, HIV+ - advanced stages/systemic = poorest HL Px
63
Auer rods are diagnostic for which leukemia?
AML
64
Which translocation is associated with promyelocytic AML? Px?
- t(15;17) - distinctive granules, auer rods --- DIC risk - good if Tx early with ATRA (forces promyelocytes to differentiate)
65
Which two leukemias are associated with basophils?
AML with t(6;9) and CML
66
What cytogenetic abnormality is associated with abnormal eosinophilic AML? Px?
inv(16) - GOOD Px
67
AML with maturation (M2) and a good Px is associated with which translocation?
t(8;21)
68
Px and population of AML with t(9;11); 11q23 translocations?
children | better Px than 11q23 in ALL
69
Which AML translocation is associated with basophilia, dysplasia and a poor Px?
t(6;9)
70
Px of AML with myelodysplasia?
POOR
71
Two good mutations in AML?
NPM1 - fish mouth nuclear invaginations, monocytic diff CEBPA - most commonly AML without maturaiton
72
BAD mutation in AML? Intermediate combo?
FLT-3 FLT-3 + NPM1
73
What are two therapy related AML's? Prognosis?
- alkylating agents -- chromosome 5, 7 loss; 5-7 years after therapy - topoisomerase related -- 11q23; 2-3 years after Tx - POOR
74
Translocation in CML? Function?
t(9;22) = bcr-abl constitutively active protein kinase that increases prolif but doesn't block mat
75
Presentation and population of CML?
- 26-60 years - GRADUAL onset with SPLENOmegaly (extramed hematopoiesis - increased WBC, thrombocytosis in 50%
76
Poor CML outcome?
Progression into accelerated or blast phase after 3 years -- myeloid or lymphoid; more cyto abnormalities
77
Which myeloproliferative disease is associated with JAK2 in 95% of cases? 50%?
- polycythemia vera | - chronic idiopathic myelofibrosis and essenial thrombycythemia
78
Etiology of fibrosis in chronic idiopathic myelofibrosis? Presentation?
- 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
In polycythemia vera, how do you know that RBC proliferation is not in response to shortage?
low EPO
80
Presentation of polycythemia vera? Progression?
- increased WBC, RBC, platelets - Vascular congestion, hepatosplenomegaly, thrombosis etc. - gradual transition to SPENT fibrotic phase = CYTOPENIA - JAK2 in 95%
81
DDx for polycythemia vera?
secondary (altitude) and relative (dehydration) polycythemias
82
Presentation of essential thrombocythemia?
- platelets >600,000 - variable, bizarre appearance - enlarged mature megs, large platelets - indolent - 12-15 years - thrombotic and hemorrhagic episodes - JAK2 in 50%
83
Lymphocytosis and reactive lymphs.. think?
VIRUS
84
What is a leukemoid reaction?
high WBC with immature granulocytes

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