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Heme / Onc > SM_265b: Hodgkin's Disease and T Cell Disorders > Flashcards

SM_265b: Hodgkin's Disease and T Cell Disorders Flashcards

1
Q

Describe types of lymphoma

A

Lymphoma

  • Acute lymphoblastic leukemia / lymphoma: T-ALL, B-ALL
  • B cell ly,mphoma: CLL / SLL, follicular lymphoma, mantle cell lymphoma, marginal zone lymphoma, others
  • T cell and NK lymphoma
  • Hodgkin lymphoma
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2
Q

T cells mature in the ___

A

T cells mature in the thymus

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3
Q

Normal T cells are positive for pan T-cell antigens, mainly ___

A

Normal T cells are positive for pan T-cell antigens, mainly CD3+

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4
Q

Describe evaluation of T cell lymphoma

A

T cell lymphoma evaluation

  • Morphologic evaluation
  • Immunohistochemical stains
  • Flow cytometric immunophenotyping
  • Molecular and genetic analysis
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5
Q

____ is a protein product derived from a specific chromosomal translocation that is expressed in anaplastic large cell lymphoma

A

ALK is a protein product derived from a specific chromosomal translocation that is expressed in anaplastic large cell lymphoma

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6
Q

Describe classification of peripheral T and NK cell lymphomas

A

Classification of peripheral T and NK cell lymphomas

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7
Q

Describe peripheral T cell / NK cell lymphoma

A

Peripheral T cell / NK cell lymphoma

  • Uncommon
  • More common in Asia
  • Usually aggressive
  • Post-thymic T cells (NK cells are closely related)
  • Most T cell lymphomas involve lymph node, most NK cell lymphomas involve extranodal sites
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8
Q

Adult T-cell leukemia / lymphoma is caused by ___

A

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

  • Occurs only in adults and has long latency
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9
Q

Describe clinical features of adult T cell leukemia / lymphoma

A

Adult T cell leukemia / lymphoma

  • Acute variant (leukemic phase): marked elevation of WBC in peripheral blood, rash, generalized lymphadenopathy, hypercalcemia with or without lytic bone lesions, systemic symptoms
  • Lymphomatous variant: prominent lymphadenopathy without peripheral blood involvement, cutaneous lesions, systemic symptom, hypercalcemia less common
  • Chronic variant: skin lesion, atypical lymphocytes but less numerous on peripheral blood, no hypercalcemia
  • Smoldering variant: WBC count normal but abnormal lymphocytes, skin and pulmonary lesions, no hypercalcemia
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10
Q

Adult T cell leukemia / lymphoma has ___ survival

A

Adult T cell leukemia / lymphoma has short survival

  • Longer in chronic or smoldering form
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11
Q

Adult T cell leukemia / lymphoma shows peripheral blood lymphocytosis and lymphocytes show ____

A

Adult T cell leukemia / lymphoma shows peripheral blood lymphocytosis and lymphocytes show flower-shaped nuclei

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12
Q

Adult T cell leukemia / lymphoma arises from ___

A

Adult T cell leukemia / lymphoma arises from peripheral CD4+ T cells at various stages of maturation

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13
Q

Adult T cell leukemia / lymphoma immunophenotype is ___

A

Adult T cell leukemia / lymphoma immunophenotype is loss of pain T-cell antigen (CD7)

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14
Q

Adult T cell leukemia / lymphoma TCR rearrangement is ___

A

Adult T cell leukemia / lymphoma TCR rearrangement is clonal

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15
Q

Adult T cell leukemia / lymphoma genetics are ___

A

Adult T cell leukemia / lymphoma genetics are no specific abnormality

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16
Q

Anaplastic large cell lymphoma presents with ____ and often in ____

A

Anaplastic large cell lymphoma presents with B symptoms (fevers, night sweats, and unintentional weight loss) and often in advanced stages

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17
Q

ALK+ anaplastic large cell lymphoma is more common in ___ and has ___ prognosis

A

ALK+ anaplastic large cell lymphoma is more common in pediatric patients and has favorable prognosis

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18
Q

ALK- anaplastic large cell lymphoma is more common in ___ and has ___ prognosis

A

ALK- anaplastic large cell lymphoma is more common in elderly and has poor prognosis

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19
Q

ALK+ anaplastic large cell lymphoma involves ___, most commonly ___

A

ALK+ anaplastic large cell lymphoma involves translocations involving ALK gene, most commonly t(2;5)

  • ALK gene encodes a tyrosine kinase receptor in chromosome 2p
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20
Q

Anaplastic large cell lymphoma appears as ___, which are ___

A

Anaplastic large cell lymphoma appears as hallmark cells, which are large cells with horse shoe shaped nuclei

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21
Q

Anaplastic large cell lymphomas are strongly positive for ___

A

Anaplastic large cell lymphomas are strongly positive for CD30

22
Q

ALK+ anaplastic large cell lymphoma involves ___ due to ___

A

ALK+ anaplastic large cell lymphoma involves overexpression of ALK protein due to t(2;5)

  • Tyrosine kinase receptor
  • t(2;5) is fusion of ALK with NPM
23
Q

Endonasal NK / T cell lymphoma nasal type is almost always ___

A

Endonasal NK / T cell lymphoma nasal type is almost always EBV+

24
Q

Endonasal NK / T cell lymphoma nasal type most commonly occurs in ___ and prognosis is ___

A

Endonasal NK / T cell lymphoma nasal type most commonly occurs in nasal cavity and prognosis is poor

25
Q

Endonasal NK / T cell lymphoma nasal type morphology is ___ and ___

A

Endonasal NK / T cell lymphoma nasal type morphology is often angiocentric with prominent necrosis with variable cytological features

26
Q

Endonasal NK / T cell lymphoma nasal type immunophenotype is ____ and ____

A

Endonasal NK / T cell lymphoma nasal type immunophenotype is NK phenotype and EBV+

  • NK phenotype: surface CD3-, cytoplasmic CD3+, CD56+, and cytotoxic molecules+
  • TCR gene rearrangement negative
27
Q

___ is the most common primary cutaneous T cell lymphoma

A

Mycosis fungoides is the most common primary cutaneous T cell lymphoma

28
Q

Mycosis fungoides involves ____

A

Mycosis fungoides involves infiltration of small/medium sized T cells with cerebriform nuclei

  • Originates from CD4+ T cells
  • Abnormal phenotype: loss of pan T cell antigen CD7
29
Q

Mycosis fungoides abnormal phenotype is ___

A

Mycosis fungoides abnormal phenotype is loss of pan T cell antigen CD7

30
Q

Mycosis fungoides has ____ clinical course

A

Mycosis fungoides has indolent clinical course with slow progression

31
Q

Pautrier microabscess occurs in ___

A

Pautrier microabscess occurs in mycosis fungoides

32
Q

Sezary syndrome has triad of ____, ____, and ____

A

Sezary syndrome has triad of

  • Erythroderma
  • Generalized lymphadenopathy
  • Peripheral blood involvement by neoplastic cells with cerebriform (convoluted) nuclei (Sezary cells(
33
Q

Erythroderma, generalized lymphadenopathy, and peripheral blood involvement by neoplastic cells with cerebriform nuclei occur in ___

A

Erythroderma, generalized lymphadenopathy, and peripheral blood involvement by neoplastic cells with cerebriform nuclei occur in Sezary syndrome

35
Q

Peripheral T cell lymphoma not otherwise specified is ___

A

Peripheral T cell lymphoma not otherwise specified is peripheral T cell lymphomas that do not meet criteria for specifically defined subtypes

  • Adults, 25-30% of all peripheral T cell lymphomas
36
Q

Peripheral T cell lymphoma not otherwise specified presents with ____ and ____ and has ____ clinical course

A

Peripheral T cell lymphoma not otherwise specified presents with lymphadenopathy and constitutional symptoms (fevers, night sweats, and weight loss) and has aggressive clinical course

37
Q

Describe morphology, cell of origin, and immunophenotype of peripheral T cell lymphoma not otherwise specified

A

Peripheral T cell lymphoma not otherwise specified

  • Morphology: extremely broad cytological spectrum
  • Cell of origin: peripheral T cells in various stages of transformation
  • Immunophenotypes: mostly CD4+, EBV usually negative
38
Q

Describe classificiation of Hodgkin lymphoma

A

Hodgkin lymphoma

  • Nodular lymphocyte predominant
  • Classical: nodular sclerosis HL, mixed cellularity HL, lymphocyte rich classical HL, lymphocyte depleted HL
39
Q

Nodular lymphocyte predominant Hodgkin lymphoma is a ___ with ___ prognosis

A

Nodular lymphocyte predominant Hodgkin lymphoma is a B cell neoplasm with preservation of B cell gene expression with good prognosis

  • Men, 30-50 years old
  • Fairly frequent relapse, rarely progresses to large B cell lymphoma
40
Q

Nodular lymphocyte predominant Hodgkin lymphoma involves ___

A

Nodular lymphocyte predominant Hodgkin lymphoma involves localized lymphadenopathy

  • Cervical, axillary, inguinal lymph nodes
41
Q

Classical Hodgkin lymphoma has ___ age distribution

A

Classical Hodgkin lymphoma has bimodal age distribution

42
Q

Classical Hodgkin lymphoma has immunophenotype of ___

A

Classical Hodgkin lymphoma has immunophenotype of CD3-, CD20-, CD30+, and CD15+

43
Q

Classical Hodgkin lymphoma has B cell lineage of ___

A

Classical Hodgkin lymphoma has B cell lineage of PAX5+ (B cell transcription factor)

44
Q

Classical Hodgkin lymphoma involves ___

A

Classical Hodgkin lymphoma involves Reed-Sternberg cells

45
Q

Nodular sclerosis classic Hodgkin lymphoma commonyl has ___

A

Nodular sclerosis classic Hodgkin lymphoma commonyl has mediastinal involvement

46
Q

Nodular sclerosis classical Hodgkin lymphoma appears as ___ on microscopy

A

Nodular sclerosis classical Hodgkin lymphoma appears as lymph node tissue separated by fibrous bands into nodules on microscopy

  • Gross: nodules
47
Q

___ has bilobed nuclei with prominant nucleoli

A

Reed-Sternberg cell has bilobed nuclei with prominant nucleoli

48
Q

This is ___

A

Reed-Sternberg cell

(classical Hodgkin lymphoma)

49
Q

Reed-Sternberg cell phenotype is ___

A

Reed-Sternberg cell phenotype is CD30+, CD15+, PAX5+, CD3-, and CD20-

50
Q

Extranodal NK / T cell lymphoma nasal type ___, ___, ___, and ___

A

Extranodal NK / T cell lymphoma nasal type occur more commonly in Asians, are EBV+, have a specific cytogenetic abnormality, and have aggressive clinical course

51
Q

This is ___

A

Anaplastic large cell lymphoma

52
Q

This is ___

A

Classical Hodgkin lymphoma, nodular sclerosis type

Heme / Onc (41 decks)

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