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HemOnc Week 3 > ICL 11.2: Lymphoma II > Flashcards

ICL 11.2: Lymphoma II Flashcards

1
Q

what is the most common leukemia in adults?

A

CLL/SLL

Chronic lymphocytic leukemia/Small lymphocytic lymphoma

monoclonal small B-cells

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2
Q

what is the immunophenotype of CLL/SLL?

A

CD5+

CD19+

CD20+

CD23+

CD10-

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3
Q

what mutation causes CLL/SLL?

A

deletion of 13q, 11q, 17p or +12

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4
Q

what population is CLL/SLL most common in?

A

˜60 years old

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5
Q

what are the characteristics of CLL/SLL?

A

generalized lymphadenopathy & hepatosplenomegaly

slow, indolent course

hypogammaglobulinemia (infections)

there can be transformation to large cell lymphoma (Richter transformation) or PLL prolymphocytic leukemia

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6
Q

what does the blood smear of CLL/SLL look like?

A

high WBC count with lymphocytosis >4000

smudge cells

spherocytes

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7
Q

what is mantle cell lymphoma?

A

arising from mantle zone of lymphoid follicle

generalized lymphadenopathy

GI tract lymphomatous polyposis

small, mature-appear lymphocyte; condensed chromatin, small amount of cytoplasm and round to irregular nuclear contour

typically small lymphocyte lymphomas are indolent, mantle cell lymphoma is unusual and is clinically aggressive

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8
Q

what population is mantle cell lymphoma most common in?

A

M > F

˜60 years old

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9
Q

what mutation causes mantle cell lymphoma?

A

t(11;14)

this upregulates BCL1 gene which codes for cyclin D1, a protein involved in checkpoint between G1 and S phase, and subsequent uncontrolled proliferation

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10
Q

what is the immunophenotype for mantle cell lymphoma?

A

CD19+

CD20+

CD5+

CD23-

positive for cyclin D

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11
Q

what is the prognosis for mantle cell lymphoma?

A

poor prognosis

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12
Q

what does MALT stand for?

A

MALT = marginal zone lymphoma

also known as MALT or BALT

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13
Q

what is MALT?

A

Rare, indolent, nodal or extranodal lymphoma, especially stomach, lung, spleen

remain localized for long time and may
REGRESS if inciting agent is removed

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14
Q

what is the immunophenotype of MALT?

A

small B-cells negative for CD5, 23, 10

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15
Q

what mutations are associated with MALT?

A

some have t(11;18) or t(1;14) or t(14;18)

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16
Q

what diseases are associated with MALT?

A

MALT is associated with autoimmune or chronic inflammation

ex. Hashimoto thyroiditis, Sjogren disease
ex. stomach- Helicobacter pylori (gastric MALT) can be treated with antibiotics

MALT remains localized for a long time but can regress with the inciting agent is removed!

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17
Q

what infectious agent can cause MALT in the stomach? how do you treat it?

A

H. pylori

can treat with antibiotics like amoxicillin, clarithromycin, and PPI

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18
Q

what infectious agent can cause MALT in the ocular adenexa?

A

C. psittaci

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19
Q

what infectious agent can cause MALT in the small intestine?

A

C. jejuni

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20
Q

what infectious agent can cause MALT in the spleen?

A

HCV

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21
Q

what infectious agent can cause MALT in the skin?

A

borrelia burgdorferi

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22
Q

which lymphomas are small B-cell malignancies?

A
  1. CLL/SLL
  2. mantle cell
  3. MALT
  4. follicular lymphoma
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23
Q

what is the immunophenotype for CLL/SLL, mantle cell, MALT and follicular lymphoma? They’re the small B-cell lymphomas

A

CLL/SLL = CD5+, CD23+, CD10-

mantle cell = CD5+, CD23-, CD10-, cyclin+

marginal zone = CD5-, CD23-, CD10-

follicular lymphoma = CD10+

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24
Q

is Burkitt lymphoma HL or NHL?

A

NHL

it’s a very aggressive, high grade B-cell NHL

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25
Q

what pattern is Burkitt lymphoma and why?

A

Burkitt lymphoma has a short doubling time (shortest in all human cancers) = excessive tumor debris

it’s cleaned up by macrophages (tingible bodies).

they are light in background of dark blue – starry sky pattern

slide 17

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26
Q

what mutation is associated with Burkitt lymphoma?

A

t(8;14) 80% of cases

or t(2;8) 15%

or t(8;22) 5%

C-myc oncogene on 8, kappa light chain on 2, lambda light chain on 22, heavy chain on 14. c-myc is anti-apoptotic transcription factor that induces proliferation

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27
Q

what is the immunophenotype of Burkitt lymphoma?

A 
CD19+
CD20+
CD22+
CD10+
BCL6+
IgM+
TdT-
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28
Q

how do you differentiate Burkitt lymphoma from ALL?

A

ALL and BL look similar morphologically

TdT (terminal deoxynucleotidyl transferase is found in lymphoblasts

ALL is positive and BL is negative for TdT

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29
Q

what does the BM of Burkitt lymphoma patient look like?

A

blast-like cells with multiple cytoplasmic vacuoles

vacuoles make the cells look like there’s white holes in them

slide 18

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30
Q

what are the 3 types of Burkitt lymphoma?

A
  1. endemic African type
  2. sporadic American type
  3. HIV-associated
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31
Q

what is endemic African type Burkitt lymphoma?

A

large mass under jaw

all infected with EBV

age 4-7

50% of pediatric cancers in equatorial Africa

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32
Q

what is sporadic American type Burkitt lymphoma?

A

abdominal location esp. ileocecum

low EBV association

<35 years old

M»F

1-2% of all lymphomas

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33
Q

what is HIV-associated Burkitt lymphoma?

A

low EBV

young, immunodeficient adults

2nd most common HIV+ NHL

34
Q

which disease is associated with Burkitt lymphoma?

A

EBV

unclear EBV role in pathogenesis but EBV may induce B cell proliferation and expansion during which chromosomal translocations are likely to arise

given the equatorial location of endemic BL, the same location for malaria, there is some thought that malaria may prime B cells and make them more susceptible to EBV infections

35
Q

how do you treat Burkitt lymphoma?

A

high dose chemotherapy and should be started within 48 hours of diagnosis

often includes intrathecal treatment (methotrexate or cytarabine) as well as rituximab (anti-CD20)

36
Q

what does ALCL stand for?

A

Anaplastic large cell lymphoma

37
Q

which mutation is associated with ALCL?

A

t(2;5)

gene abnormality produces constitutively active tyrosine kinase

ALK gene rearrangement is diagnostic!

38
Q

what population is ALK most common in?

A

children/young adults

39
Q

what is ALK?

A

anaplastic, bizarre large cells

CD30+

40
Q

what is breast implant associated ALCL?

A

majority associated with textured implant

usually ALK negative, but has good prognosis

treatment is total capulectomy, removal of the implant

consider removing the contralateral implant

41
Q

what type of cell is common in ALCL?

A
  1. horseshoe nucleus with lots of cytoplasm
  2. embryo nucleus - looks like a kidney bean

slide 23

42
Q

what is mycosis fungoides/Sezary syndrome?

A

cutaneous T-helper CD4 lymphoma with slow, indolent course

skin resident effector memory T cells are the cells of origin of MF

cerebriform nucleus in sezary cells (slide 29)

skin changes-inflammatory (patch), plaque, tumor

collections of these cells in the epidermis = Pautrier microabscess (slide 27/28)

leukemic phase = Sezary syndrome, associated with generalized exfoliative erythroderma and usually only seen in older individuals

43
Q

what is Sezary syndrome?

A

associated with generalized exfoliative erythroderma and usually only seen in older individuals

characterized by the presence of atypical T cells in skin, lymph nodes and peripheral blood

thought to arise from thymus memory T cells while skin resident effector memory T cells are the cells of origin of MF

44
Q

what is extra nodal NK/T cell lymphoma?

A

nasal type, is a rare, fast-growing, highly aggressive type of NHL

it can start in T cells, but it develops most often in natural killer (NK) cells

this type of NHL is called extranodal because it develops in organs or tissues other than the lymph nodes

blood vessel wall destruction leads to extensive ischemic necrosis = angioinvasive and angiodestructive

45
Q

what disease is extra nodal NK/T-cell lymphoma associated with?

A

high EBV association

46
Q

what’s the immunophenotype of extra nodal NK/T-cell lymphoma?

A

+ EBV and usually CD56+, CD2+, CD3-

47
Q

how do you treat extra nodal NK/T-cell lymphoma?

A

combined chemo and radiation

48
Q

are T-cell lymphomas common?

A

no in the US

they also have a worse prognosis than B-cell lymphomas

49
Q

what are T-cell lymphomas?

A

not classified into small cell or large cell

skin involvement is common

normal T-cells are +CD2,3,5,7, and either CD4 or 8

but lymphomas commonly have antigen dropout and/or coexpression of CD4 and CD8 or loss of both CD4 and CD8

all have diffuse pattern

50
Q

what type of cells are involved in T-cel lymphomas?

A

all are peripheral (mature) T-cells except precursor T-ALL/lymphoblastic lymphoma

Tdt positive ONLY in lymphoblastic lymphoma

51
Q

what is the only TdT+ T-cell lymphoma?

A

precuror-T lymphoblastic leukemia/lymphoma = T-cell ALL

52
Q

what is adult T-cell leukemia/lymphoma caused by?

A

caused by HTLV-1 (human T-cell leukemia/lymphoma virus 1)

endemic in Japan, Caribbean basin

53
Q

what is the immunophenotype for adult T-cell leukemia/lymphoma ?

A

CD4+

54
Q

what are the characteristics of adult T-cell leukemia/lymphoma ?

A

caused by HTLV-1

leukemic variant is usually rapidly progressive and fatal

  • skin lesions
  • lymphadenopathy
  • lymphocytosis with cloverleaf or floret nucleus
  • hypercalcemia
55
Q

what does the blood smear of adult T-cell leukemia/lymphoma show?

A

lymphocytes with cloverleaf or floret nucleus

slide 37

nucleus literally looks like a 3 leaf clover

56
Q

what is large granular lymphocytic leukemia?

A

there’s T-cell (CD3+) or NK-cell (CD56+) variants

STAT3 mutation common = transcription factor

57
Q

what are some characterstics of large granular lymphocytic leukemia?

A

usually indolent course

lymphocytosis and splenomegaly

neutropenia and anemia

sometimes pure red cell aplasia in BM

some cases of Felty syndrome have underlying LGL

58
Q

what is pure red cell aplasia?

A

common in large granular lymphocytic leukemia (LGL)

Purered cell aplasia(PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow

59
Q

what is Felty syndrome?

A

some cases of Felty syndrome have underlying LGL

Felty syndrome(FS), also known as ‘‘super rheumatoid’’ disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and neutropenia, resulting in susceptibility to bacterial infections

60
Q

what mutation is associated with mantle cell lymphoma?

A

t(11;14)

BCL1 on chromosome 11, translocation to 14 increases expression

BCL1 codes overexpression of Cyclin D1

61
Q

what mutation is associated with follicular lymphoma?

A

t(14;18)

BCL2 – inhibits pro-apoptotic protiens, located on chromosome 18 and moves to heavy chain on 14

BCL2 over expression allows for lymphoma proliferation

62
Q

what mutation is associated with Burkitt lymphoma?

A

t(8;14)

c-myc (chromosome 8) over expression by translocation to heavy chain on 14

makes up 80% of BL translocation, other less common translocations include t(2;8) 15%; or t(8;22) 5%

63
Q

what mutation is associated with anaplastic large cell lymphoma?

A

t(2;5)

ALK gene rearrangements

64
Q

what are myelodysplastic syndromes?

A

hematopoiesis dysfunction that results in the bone marrow filled with dysplastic cells that can’t leave the marrow, resulting in systemic cytopenias

patients present with symptoms of bone marrow failure like weakness, fatigue, bleeding

65
Q

what disease are myelodysplastic syndrome patients at risk for?

A

high risk for transformation into AML

blast count must be from 0 – 19%

> 20% is AML

blast count can be prognostic

66
Q

how do you treat myelodysplastic syndromes?

A

treatment includes observation, chemotherapy, high dose combination chemotherapy and/or stem cell transplant

67
Q

what is multiple myeloma?

A

malignant proliferation of plasma cells in bone marrow

malignant plasma cells inhibit formation of normal polyclonal immunoglobulin = increase risk for infection

monoclonal IgG or IgA creates an M spike in SPEP - monoclonal immunoglobulin is closely correlated to amount of tumor mass present

68
Q

how do diagnose multiple myeloma?

A

Serum Protein Electrophoresis (SPEP)

Urine Protein Electrophoresis (UPEP)

Bone Marrow Biopsy (at least 10% plasma cells)

Blood Smear

Free Ligh Chain Assay (FLC)

Bone survey

Urine Immunofixation Electrophoresis (UIFE)

Serum immunofixation Electrophoresis (SIFE)

69
Q

what is UPEP looking for in MM?

A

Bence-Jones proteins

they’re light chain only proteins that are small enough to be filtered into the urine

70
Q

what three things are characteristic of MM?

A
  1. Russel bodies – large, eosinophilic, cytoplasmic inclusions thought to represent distended endoplasmic reticulum (slide 45)
  2. Dutcher bodies – Excess cytoplasmic immunoglobulin invaginating into the nucleus give the appearance of intranuclear inclusions (slide 47)
  3. Rouleaux Formation – increased immunoglobulin in serum interferes with normal repellant force that pushes red cells apart, allowing them to stack upon each other (slide 46)
71
Q

what is smoldering myeloma?

A

plasma cell disorder

> 10% plasma cells

M-spike, but no other end organ damage or biopsy proven amyloidosis to confirm MM

MGUS –> smoldering myeloma –> MM

72
Q

what is MGUS?

A

plasma cell disorder

< 10% plasma cells; small M-spike

no lytic bone lesions, anemia, kidney dysfunction,

at risk of developing MM,

since MGUS can lead to multiple myeloma at the rate of about 1.5% a year, yearly monitoring is recommended

MGUS –> smoldering myeloma –> MM

73
Q

what is LCDD vs. HCDD?

A

Light chain deposition disease (LCDD) – MM but not amyloidosis in kidney

Heavy chain deposition disease (HCDD – MM, produce both light and heavy immunoglobulin chains; unable to form amyloid fibrils so they don’t have hallmark histologic features of amyloidosis –identified via immunofluorescence

74
Q

what is LCDD?

A

light chain deposition disease

it’s the deposition of monoclonal light chains in multiple organs. It is a rare disease characterized by deposition of non-amyloid immunoglobulin light chains, which do not stain with Congo red

LCDD is categorized as a monoclonal immunoglobulin deposition disease

a single clone of plasma cells is responsible for overproduction of kappa chains and, very rarely, lambda chains

a monoclonal population of plasma cells can be detected in the bone marrow, and an altered serum-free light chain ratio is present

in 25% of patients, an abnormal serum free light chain ratio is noted, even without an abnormal finding with serum and or urine electrophoresis with immunofixation

incidence of LCDD is unknown. The median age at diagnosis is 58 years, and it is more common in M than F

75
Q

what is HCDD?

A

Heavy-chain deposition disease is the least common non-organized monoclonal immunoglobulin deposition disease

Unlike the more common LCDD an association of multiple myeloma or plasma cell dyscrasias with HCDD is less common, with only 7 of 23 (30%) reported cases being associated with development of demonstrable monoclonal plasmacytosis.

nodular glomerulosclerosis is the classic glomerular pattern of injury of all monoclonal immunoglobulin disorders, and the disease (though suspected on light microscopy) can be conclusively diagnosed only by an extended panel of immunofluorescence that includes antibodies against heavy-chain isotypes

76
Q

what is systemic light chain amyloidosis?

A

characterized by decreased number of monoclonal plasma cells in the BM compared to MM

amyloid has an affinity for visceral organs and causes end organ damages

Congo Red stain is used to identify amoyloid deposits

77
Q

what does Rituximab do?

A

target CD20

used for treatment of CLL, NHL

78
Q

what does Brentuximab Vedotin do?

A

target CD30

used for HL, primary cutaneous large cell lymphoma, CD30+ MF

79
Q

what does Bortezomib do?

A

proteasome inhibitor

used for tx of MM, amyloidosis

80
Q

what does Bendamustine do?

A

nitrogen mustard chemotherapy

used for tx of CLL, MM, NHL

HemOnc Week 3 (22 decks)

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