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Oncology > 7 - Hodgkin Lymphomas and T Cell Lymphomas > Flashcards

7 - Hodgkin Lymphomas and T Cell Lymphomas Flashcards

1
Q

T Cell Origin Story

A

Born in the bone marrow
Migrate to Thymus
Mature & enter peripheral blood flow

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2
Q

Peripheral T-Cell Lymphomas (PTCL)

A

Rare (5 - 10% of Non-Hodgkin Lymphoma in US)
More common in Asia
No standard treatment regimens
Range from indolent to highly aggressive
Generally worse prognosis than B-Cell Counterparts

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3
Q

PTCL Subtypes

A

PTCL-NOS (Not Otherwise Specified) - Catchall
Anaplastic Large Cell Lymphoma (ALCL) - ALK+/-
Angioimmunoblastic Lymphoma

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4
Q

Median overall survival for most subtypes of PTCL

A

1 - 3 years
5 year survival is approximately 26%
Exception: ALK+ ALCL (5-year survival of 65-90%)

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5
Q

Peripheral T-Cell Lymphoma Not Otherwise Specified (PTCL-NOS)

A

Waste basket diagnosis
Often presents with rash
Uniformly poor prognosis
No standard treatment regimens

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6
Q

PTCL-NOS - Treatment

A 
No standard treatment regimens
CHOP offers poor outcome
Etoposide
Gemcitabine
Transplant in first remission
HDAC inhibitors
Pralatrexate
Campath
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7
Q

PTCL-NOS - Morphology & Immunohistochemistry

A

TCR Rearrangements
Loss of T-Cell surface markers
No specific cytogenetic abnormalities

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8
Q

Anaplastic Large Cell T-Cell Lymphoma - 2 Subtypes

A

ALK+

ALK-

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9
Q

Anaplastic Large Cell T-Cell Lymphoma - ALK+

A

Younger age
Better prognosis
t(2;5)

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10
Q

Anaplastic Large Cell T-Cell Lymphoma - ALK-

A

Older age

Poor prognosis

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11
Q

Anaplastic Large Cell T-Cell Lymphoma - Morphology

A

CD30+
Allows us to use Brentuximab for targeted therapy!
Horseshoe-shaped nuclei

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12
Q

Angioimmunoblastic T-Cell Lymphoma

A

Often long latency to definitive diagnosis
Presents with autoimmune syndromes
Hemolytic anemia and thrombocytopenia
Rash

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13
Q

Angioimmunoblastic T-Cell Lymphoma - Morphology

A

Arborization of vessels
Polymorphous infiltrate (Plasma Cells, Monoclonal B-Cells)
Follicular dendritic T-Cell origin
TCR genes rearranged 75 - 90%
EBV and HHV-6 genomes amy be present in reactive B Cells

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14
Q

Angioimmunoblastic T-Cell Lymphoma - Immunohistochemistry

A 
CD3+
CD4+
BCL6+
CD10+
CD2-
CD3-
CD5-
CD7-
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15
Q

Angioimmunoblastic T-Cell Lymphoma - Treatment

A

HDAC Inhibitors
Immunosuppressants
Gemcitabine
Campath (Anti-CD52)

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16
Q

Extranodal NK/T-Cell Lymphoma - Nasal Type

A

Most common in Asia

Always clonal EBV+

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17
Q

Extranodal NK/T-Cell Lymphoma - Nasal Type - Morphology

A

Large Azurophilic Granules

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18
Q

Extranodal NK/T-Cell Lymphoma - Nasal Type - Immunohistochemistry

A

CD56+
CD3-
TCR rearrangement-

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19
Q

Extranodal NK/T-Cell Lymphoma - Nasal Type - Treatment

A

Combined XRT and high does chemotherapy

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20
Q

Hepatosplenic T-Cell Lymphoma - Presentation

A

Hepatosplenomegaly (due to infiltration of sinusoids of liver, spleen)
Coombs negative hemolytic anemia
Purpura/Rash
Hemophagocytic syndrome

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21
Q

Hepatosplenic T-Cell Lymphoma - Morphology

A

Small to medium size

Transforms to blastic variant

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22
Q

Hepatosplenic T-Cell Lymphoma - Immunohistochemistry

A 
CD2+
CD3+
CD7+
CD4-
CD5-
CD8-
TCR Rearrangement +
Usually gamma-delta, but few alpha-beta
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23
Q

Hepatosplenic T-Cell Lymphoma - Treatment

A

No standard therapy

Very poor prognosis

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24
Q

Subcutaneous Panniculitis-Like T-Cell Lymphoma - Morphology

A

Subcutaneous Infiltrate
Atypical Lymphocytes
Involves fat lobules
Dermis and epidermis

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25
Q

Subcutaneous Panniculitis-Like T-Cell Lymphoma

A

Hemophagocytic Syndrome

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26
Q

Subcutaneous Panniculitis-Like T-Cell Lymphoma - Immunohistochemistry

A 
CD3+
CD8+
CD4-
CD56-
α/β TCR+
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27
Q

Enteropathy Associated T-Cell Lymphoma - Presentation

A

Worsening malabsorption in patients with Celiac Disease
Associted with HLA-DRQ
Similar to MALT with H. Pylori
Often the lymphoma goes away if you treat the celiac disease

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28
Q

Enteropathy Associated T-Cell Lymphoma - Treatment

A

Surgery
High Chemo
Allo Stem Cell Transplant

29
Q

Mycosis Fungoides

A

Cutaneous T-Cell Lymphoma
Indolent Lymphoma (delayed diagnosis, history of eczema)
Skin lesions can progress

30
Q

Mycosis Fungoides - Progression of skin lesions

A

Patch-Plaque stage
Tumor (minority)
Lymph node involvement
Sezary syndrome

31
Q

Mycosis Fungoides - Sezary Syndrome

A

Systemic involvement

Peripheral blood findings

32
Q

Mycosis Fungoides - Treatment

A 
Topical Glucocorticoids (can be curative)
XRT
PUVA
Topical chemotherapy
Phototherapy
Photopheresis
Retinoids
HDAC inhibitors
Chemotherapy
Transplant
33
Q

Adult T Cell Leukemia/Lymphoma (ATLL)

A

NOT the same as T-Cell ALL
Driven by HTLV-1
Transmitted by breastfeeding, blood transfusions, needle sharing, sexual intercourse
Long latency (10 - 30 years)

34
Q

ATLL - Morphology

A

Flower Cell

35
Q

ATLL - Immunohistochemistry

A

TAX+
CD4+
CD25+
CD30+ Occasionally

36
Q

HTLV-1 ATLL - Presenting Symptoms

A

Lymphadenopathy
Rash
Hypercalcemia
CNS involvement (32%)

37
Q

HTLV-1 ATLL - Epi

A

26% Jamaican heritage
18% Dominican Republic
7% American born AA

38
Q

HTLV-1 ATLL - Therapy

A

Combination Chemotherapy:
EPOCH, HyperCVAD
Intrathecal prophylaxis

Anti-viral therapy:
Zidovudine + IFN

Transplant

Novel Therapies

39
Q

HTLV-1 ATLL - Novel Therapies

A 
Anti-CD25 & Anti CCR4 Antibodies
HDAC Inhibitors
Pralatrexate
Bortezomib
Lenalidomide
40
Q

HTLV-1 ATLL - Overall Survival

A 
WEEKS!!!! Hella aggro
Acute Subtype - 19 weeks
Lymphomatous Subtype - 37 weeks
Chronic Subtype - 89 weeks
Smoldering Subtype - Not reached
41
Q

Managing PTCL-NOS Algorithm

A 
Confirm hematopathology (Check for CD30, HTLV-1)
Clinical Trial OR Etoposide-containing Combination Chemo
If complete remission, autologous stem cell transplant
If partial remission, relapse or worse:
Vorinostat
Romidepsin
belinostat
Pralatrexate
Berntuximab
Clinical Trial
42
Q

HDAC Inhibitors approved for T-Cell Lymphoma

A

Vorinostat
Romidepsin
Belinostat

43
Q

HDAC Inhibitor - Mechanism of action

A 
Inhibiting the de-acetylation of histones
Enforces the acetylation of histones
Leads to open chromatin
Transcriptional activation of tumor suppressors:
p53
HSP90
NFkB
STAT3
44
Q

Pralatrexate

A

Novel anti-folate drug
Significant improvements in overall survival
Marked activity in relapsed T-Cell Lymphoma
Overall response rate 29%

45
Q

Hematologic Malignancies Expressing CD30

A

Hodgkin Lymphoma

Systematic Anaplastic Large Cell Lymphoma (sALCL)

46
Q

CD30

A

Member of the TNFR superfamily

Found at variable levels on the surface of different B-Cell and T-Cell lymphomas

47
Q

Brentuximab Vedotin - Mechanism of action

A

Antibody-Drug Conjugant (ADC)
Ab to CD30 bound to Monomethyl Auristatin E (MMAE) a potent antimicrotubule agent

Binds to CD30
Drug gets endocytosed
Protease-cleavable linker releases MMAE into lysosome
MMAE disrupts microtubule network

48
Q

Brentuximab Vedotin - Side effect

A

Neuropathy

49
Q

Brentuximab Vedotin in Relapsed/Refractory Peripheral T-Cell Lymphoma - Response

A

Nearly everyone responded

97% patients achieved tumor reduction

50
Q

Hodgkin Lymphoma

A

Lymphoid neoplasm defined by presence of Reed-Sternberg (RS) cells in a reactive infiltrate
9,290 estimated new cases in 2013
1,180 deaths estimated

51
Q

Hodgkin Lymphoma - Morphology

A

Reed Sternberg Cells
Background of Eosinophils & Macrophages
Cell of origin - Now thought to be B-Cell, likely post-Germinal center

52
Q

Hodgkin Lymphoma - Subtypes

A

Nodular Sclerosis - Most common, thick fibrous bands
Mixed Cellularity - Most often in HIV
Lymphocyte Rich
Lymphocyte Depletedq

53
Q

Hodgkin Lymphoma - Immunohistochemistry

A

CD30+
CD15+
PAX5+
Often EBV+ → NFkB activation

54
Q

Hodgkin Lymphoma - Presentation

A

Young Adults
Spreads in contiguous fashion
Large mediastinal mass

B-Symptoms:
Pruritus
Pel Ebstein Fevers
Painful nodes with alcohol consumption

55
Q

Advanced Hodgkin’s - International Prognostic Score (IPS) Points

A

Age ≥ 45
Gender Male
Albumin

56
Q

Hodgkin’s Lymphoma - Treatment

A

ABVD every 2 weeks

Doxorubicin (Adriamycin)
Bleomycin
Vinblastine
Dacarbazine

57
Q

Doxorubicin (Adriamycin) - Mechanism of action

A

Anthracycline

58
Q

Doxorubicin (Adriamycin) - Side Effect

A

Cardiotoxicity

59
Q

Bleomycin - Mechanism of action

A

Antineoplastic

60
Q

Bleomycin - Side Effect

A

Pulmonary Fibrosis

61
Q

Vinblastine - Mechanism of action

A

Anti-microtubule

62
Q

Vinblastine - Side Effect

A

Neuropathy

63
Q

Dacarbazine - Mechanism of action

A

Alkylating Agent

64
Q

Dacarbazine - Side Effect

65
Q

Hodgkin’s Lymphoma - Pre-Treatment & Considerations

A

First evaluate cardiac & pulmonary function:
ECHO
PFTs with DLCO

of cycles given depends partly on stage and partly on IPS. Anywhere from 2 - 6 cycles

Treatment is highly emetogenic, so prescribe anti-emetics

Not all patients lose their hair

66
Q

Hodgkin’s Lymphoma - Role of Radiation

A

Early-Stage
Involved field XRT can be used to decrease ABVD cycles to 2 - 4

Bulky Disease (mediastinal mass > 10cm or > 1/3 of chest width
XRT can be used as a consolidation
Improved rate of survival

Risk
Late toxicities:
Secondary malignancies (breast cancer)
Pulmonary fibrosis
Early CAD
67
Q

Hodgkin’s Lymphoma - PET- after 2 cycles

A

Will likely do very well. This trumps IPS score.

68
Q

Hodgkin’s Lymphoma - PET+ after 2 cucles

A

Will likely relapse

69
Q

Hodgkin’s Lymphoma - Plan at relapse

A

Salvage Chemotherapy
Leads to responses in 60 - 90%

Treatment regimens:
ICE
DHAP
ESHAP
Gemcitabine-based

Oncology (23 decks)

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