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Weir - Hematology > 3.26.14* Lymphomas > Flashcards

3.26.14* Lymphomas Flashcards

PPT* Lecture Notes* Reading (ch 19 and 20)* Pictures

1
Q

lymphoma

A

when malignancy lymphocytes accumulate in lymph nodes and cause lymphadenopathy. If they spill over into the blood, that is the leukemic phase)

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2
Q

lymphoma

A

a. clinical presentaiton- painless enlarged lymph nodes (mostly cervical), splenomegaly in 50% (maybe hepatomegaly)
b. PBS- normochromic/normocytic anemia. Neutrophilia, lymphopenia. Increased erythrocyte sedimentation rate, CRP, LDH.
c. lymph node- RS cells and mononuclear Hodgkin cells.

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3
Q

Types of Hodgkin lymphoma

A

i. Nodular sclerosis: collagen band encircle nodules in lymph node
ii. lymphocyte rich- scant R/S cells with multiple small lymphoctes
iii. mixed cellularity- R/S cell numerous with intermediate lymphocytes
iv. lymphocyte depleted- diffuse fibrosis disordered connective tissue

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4
Q

Non-Hodgkin lymphomas

A

a. clonal lymphoid tumors, 85% B cell and 15% T and NK cell. More irregular, more extranodal.
b. clinical presentation: lymphadenopathy, neutropenia/thrombocytopenia/anemia, infections, constitutional symptoms (fever, weight loss) if disseminated.

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5
Q

Non-Hodgkin lymphomas types

A 
Diffuse large B cell
Follicular lymphoma
CLL/SLL
MALT lymphoma
Mantle cell lymphoma
Primary mediastinal large B cell lymphoma
Burkitt
and others...
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6
Q

What distinguishes a reactive and malignant B cell lymphoma lymph node

A

the expression of either kappa or lambda light chains can confirm clonality and distinguish from a reactive node

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7
Q

t(14,18)

A

follicular lymphoma

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8
Q

t(11,14)

A

mantle cell lymphoma

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9
Q

t(8,14)

A

Burkitt lymphoma

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10
Q

t(2,5)

A

anaplastic large cell lymphoma

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11
Q

immunoperoxidase show

A

light chains in the lymph node to reveal monoclonal origin

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12
Q

Stage system for Hodgkin lymphoma

A

1 one area
2 two areas
3 areas on both sides of diaphragm
4 extranodal

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13
Q

when should treatment be done for lymphomas?

A

when there is significant organomegaly, anaemia, thrombocytopenia, neuropathy, amyloidosis or hyperviscosity.

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14
Q

What is the broadest effective drug treatment for lymphoma

A

Rituximab, anti-CD20

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15
Q

Marginal zone lymphomas

A

low grade, arise from marginal zone of B cell germinal follicles

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16
Q

Follicular lymphoma

A

a. 25% of NHL, average age 60.
b. genetics t(14,18) leading to constituitive BCL-2 expression (reduced apoptosis).
c. grading I or II depending on amount of centrocytes -> centroblasts). III is severe with marrow involvement. staging same as HL.

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17
Q

centrocytes v. centroblasts

A

During this process of rapid division and selection, B cells are known as centroblasts, and once they have stopped proliferating they are known as centrocytes.

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18
Q

Mantle cell lymphoma

A

a.
b. origin: derived from pre-germinal centre cells localized in the primary follicles or in the mantle region of secondary follicles.
c. immunophenotype: CD19, CD5, CD22, CD23A.
d. genetics: t(11,14) IgH-cyclin D1.

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19
Q

Immunophenotype of mantle cell lymphoma

A

CD19, CD5, CD22, CD23A.

Last two distinguish from CLL

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20
Q

Diffuse large B-cell lymphoma

A

a. cp: rapid lymphadenpathy, infiltration to marrow, GI, brain, spinal cord, kidneys.
b. genes: BCL-6 gene on chromosome 3, sometimes the BCL-2 translocation(20%).

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21
Q

Most common genetic abnormalities in diffuse large B-cell lymphoma

A

BCL-6 gene on chromosome 3, sometimes the BCL-2 translocation(20%).

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22
Q

Burkitt lymphoma genetic abnormality

A

overexpression of MYC t(8,14) in nearly all cases.

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23
Q

Burkitt lymphoma (endemic) clinical presentaiton

A

cp: massive lymphadenopathy often of jaw in children

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24
Q

Burkitt lymphoma (sporadic) clinical presentaiton

A

GI cancers

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25
Q

Burkitt lymphoma lymph node histology

A

“starry sky” due to large clear tingible body macrophages with homogenous background

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26
Q

Angioimmunoblastic lymphadenopathy

A

usually occurs in elderly patients with lymphadenopathy, hepatosplenomegaly, skin rashes and a polyclonal increase in serum IgG.

27
Q

Mycosis fungoides

A

chronic cutaneous T-cell lymphoma that presents with severe pruritus and psoriasis-like lesions. Ultimately, deeper organs are affected, particularly lymph nodes, spleen, liver and bone marrow.

28
Q

Sézary syndrome

A

dermatitis, erythroderma, generalized lymphadenopathy and circulating T-lymphoma cells. CD4s have a folded or cerebriform nuclear chromatin.

29
Q

Anaplastic large cell lymphoma

A

particularly common in children. The disease is CD30-and usually associated with the t(2; 5) (p23; q35) translocation. This leads to overexpression of anaplastic lymphoma kinase (ALK). It has an aggressive course characterized by systemic symptoms and extranodal involvement.

30
Q

T cell lymphoma types

A 
mycosis fungoides
Sezary (leukemic form of MF)
Angioimmunoblastic T cell lymphoma
Anaplastic large cell lymphoma
Adult T-cell leukemia/lymphoma
31
Q

What 4 major factors must a doctor consider when he/she decides if cure is possible for a patient with lymphoma?

A

tumor histology (indolent/aggressive, B or T cell)
tumor stage
condition of patient
available therapies

32
Q

*Name the most common indolent lymphoma. Aggressive lymphoma?

A

indolent:
aggressive: diffuse large B cell lymphoma

33
Q

*Be able to stage a patient with lymphoma

A 
(done usually by CAT scan or PET)
I. one node
II. two nodes
III. nodes on both sides of diaphragm
IV. organ involvement
34
Q

*What are the major treatment decision differences between indolent and aggressive lymphomas?

A

Aggressive lymphoma: treat with aggressive chemo/radiation immediately. Patients will often be cured. Often low stage.

Indolent lymphoma (low grade): wait to treat until symptomatic. Usually high stage. Less likely to be cured. Slow progression.

35
Q

*Understand the mechanism and sites of action of rituximab and ibrutinib.

A

Rituximab: anti-CD20 (complement, NK, and phagocytosis of B cell)
b. ibrutinib: anti-bruton tyrosine kinase (blocks BCR from signaling NFkB signalling)

36
Q

*What is the name of the most common cutaneous lymphoma and what cell can you find in the blood in some of these patients?

A

mycosis fungoides

37
Q

*What is a common complication of radiation therapy in young Hodgkin’s Disease women?

A

Solid breast tumors

38
Q

*Name two complications of CLL.

A 
Lymphocytosis
Lymphadenopathy
Splenomegaly
Anemia
Autoimmune cytopenias
Hypogammaglobulinemia
39
Q

*Name poor prognostic abnormalities in CLL.

A

High Stage
Increased rate of lymphocyte doubling time
Beta 2 macroglobulin (higher is worst)
Deletion 17p, TP53 mutations,11q deletion
Unmutated VH genes, ZAP-70, CD 38, (means less differentiated)

40
Q

*When is BMT used in Lymphomas?

A

relapse

41
Q

Clinical presentation of lymphoma

A

lymphadenopathy
night sweats
weight loss

42
Q

What virus is commonly associated with lymphoma after transplants?

A

EBV

43
Q

Complications of lymphoma

A

a. Lymphadenopathy obstruction
b. Replacement in bone marrow -> pancytopenia
c. Reduction in functional lymphocytes -> hypogammaglobulinemia
d. tumor productions: uric acid, calcium
e. paraneoplastic (AHA, ITP, neuropathy)

44
Q

What are the indolent lymphomas

A

follicular B cell
CLL
MALT lymphoma
Mycosis fungoides

45
Q

Lymphocytic grading system

A

by increasing numbers of centroblasts present, meaning that mutation happens earlier

46
Q

What are the aggressive lymphomas

A

Diffuse large B cell
Mantle cell
Peripheral T cell lymphomas (NOS, Angioimmunoblastic, anaplastic)

47
Q

What are the highly aggressive lymphomas

A

Burkitt’s

T cell lymphoblastic lymphoma

48
Q

What is included in the international prognostic index for aggressive NHL survival?

A 
A- age >60
P- performance status
L- LDH elevated
E- extranodal sites
S- Stage 3-4

positives increase risk or mortality

49
Q

Therapy for aggressive lymphoma

A

a. CHOP (chemo)
b. Antibody therapy (rituximab)
c. radio-immunotherapy
d. short course chemo + radiation
e. BMT for relapse

50
Q

What infections are associated with lymphoma?

A

HIV (primary CNS esp.)
HHV8 (leads to primary effusion lymphoma and Castleman’s)
HTLV-1 (T cell lymphoma)
EBV (Posttransplant lymphomas, nasopharyngeal)
H. Pylori (MALT lymphoma)

51
Q

Clinical manifestation of Mycosis Fungoides:

A

a. Indolent stage: Eczema
b. Invasive tumor stage: ulceration, Sezary syndrome with large numbers of B cell circulating in the blood causing erythrodermis.

52
Q

PBS of sezary syndrome in MF patient?

A

cerebriform large, clefted nuclei in T cells.

53
Q

Angioimmunoblastic T cell Lymphoma

A

Highly polymorphic background
Constitutional symptoms
Hypergammaglobulinemia
Autoimmune hemolysis

54
Q

Anaplastic Large Cell Lymphoma

A

30% childhood lymphomas
Young Adults
Mediastinal presentations
*Favorable if ALK+; unfavorable if ALK-

55
Q

Hodgkin’s Disease vs. NHL

A 
Different mode of progression
Same staging
Same staging tests
Early stage: treat with short course chemotherapy and focal radiation to area of disease or radiation alone or chemo alone
Late stage: treat with chemotherapy
ABVD
Good prognosis
56
Q

How does Hodgkin’s lymphoma usually progress?

A

Hodgkin usually progresses from one lymph node to surrounding. Makes good for radiation because it doesn’t skip around. These patients do better than NHL. Hodgkins has such a high cure rate, so we are very concerned about later diseases.

57
Q

Late complications of chemo Rx for Hodgkins

A 
Acute leukemia and myelodysplasia
Solid tumors-breast, lung, stomach, bone, soft tissues
Coronary artery disease
Radiation and chemotherapy pneumonitis
Infertility
Hypothyroidism
58
Q

Chronic Lymphocytic Leukemia

A

a. indolent, low grade B cell malignancy
b. clinical presentation: Usually patients > 50 yo. Often asymptomatic.
c. complications: Lymphocytosis
Lymphadenopathy
Splenomegaly
Anemia
Autoimmune cytopenias
Hypogammaglobulinemia

59
Q

Staging system of CLL

A

…

60
Q

Treatment of CLL

A

a, Chemo drugs

b. rituximab (CD20), alemtuzumab (CD52)
c. radiation
d. corticosteroids

(not done until CLL is problematic)

61
Q

Hairy cell leukemia

A

massive splenomegaly

62
Q

When do you do autologous transplant?

A

when the cancer will be responsive to chemo. The goal is to temporarily remove bone marrow so that intense chemo/radiation can be used. And get to a low enough burden where self immune system can do the rest.

63
Q

When do you do allogenic transplant?

A

When there is a problem with the patient’s own bone marrow, or you need a donor’s cells to fight the recipient’s leukemia.

Weir - Hematology (6 decks)

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