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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

(52 cards)

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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 ___

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A

Anaplastic large cell lymphomas are strongly positive for CD30

22
Q

ALK+ anaplastic large cell lymphoma involves ___ due to ___

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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 ___

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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 ___

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A

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

25
Endonasal NK / T cell lymphoma nasal type morphology is ___ and \_\_\_
Endonasal NK / T cell lymphoma nasal type morphology is often angiocentric with prominent necrosis with variable cytological features
26
Endonasal NK / T cell lymphoma nasal type immunophenotype is ____ and \_\_\_\_
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
\_\_\_ is the most common primary cutaneous T cell lymphoma
Mycosis fungoides is the most common primary cutaneous T cell lymphoma
28
Mycosis fungoides involves \_\_\_\_
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
Mycosis fungoides abnormal phenotype is \_\_\_
Mycosis fungoides abnormal phenotype is loss of pan T cell antigen CD7
30
Mycosis fungoides has ____ clinical course
Mycosis fungoides has indolent clinical course with slow progression
31
Pautrier microabscess occurs in \_\_\_
Pautrier microabscess occurs in mycosis fungoides
32
Sezary syndrome has triad of \_\_\_\_, \_\_\_\_, and \_\_\_\_
Sezary syndrome has triad of * Erythroderma * Generalized lymphadenopathy * Peripheral blood involvement by neoplastic cells with cerebriform (convoluted) nuclei (Sezary cells(
33
Erythroderma, generalized lymphadenopathy, and peripheral blood involvement by neoplastic cells with cerebriform nuclei occur in \_\_\_
Erythroderma, generalized lymphadenopathy, and peripheral blood involvement by neoplastic cells with cerebriform nuclei occur in Sezary syndrome
34
35
Peripheral T cell lymphoma not otherwise specified is \_\_\_
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
Peripheral T cell lymphoma not otherwise specified presents with ____ and ____ and has ____ clinical course
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
Describe morphology, cell of origin, and immunophenotype of peripheral T cell lymphoma not otherwise specified
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
Describe classificiation of Hodgkin lymphoma
Hodgkin lymphoma * Nodular lymphocyte predominant * Classical: nodular sclerosis HL, mixed cellularity HL, lymphocyte rich classical HL, lymphocyte depleted HL
39
Nodular lymphocyte predominant Hodgkin lymphoma is a ___ with ___ prognosis
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
Nodular lymphocyte predominant Hodgkin lymphoma involves \_\_\_
Nodular lymphocyte predominant Hodgkin lymphoma involves localized lymphadenopathy * Cervical, axillary, inguinal lymph nodes
41
Classical Hodgkin lymphoma has ___ age distribution
Classical Hodgkin lymphoma has bimodal age distribution
42
Classical Hodgkin lymphoma has immunophenotype of \_\_\_
Classical Hodgkin lymphoma has immunophenotype of CD3-, CD20-, CD30+, and CD15+
43
Classical Hodgkin lymphoma has B cell lineage of \_\_\_
Classical Hodgkin lymphoma has B cell lineage of PAX5+ (B cell transcription factor)
44
Classical Hodgkin lymphoma involves \_\_\_
Classical Hodgkin lymphoma involves Reed-Sternberg cells
45
Nodular sclerosis classic Hodgkin lymphoma commonyl has \_\_\_
Nodular sclerosis classic Hodgkin lymphoma commonyl has mediastinal involvement
46
Nodular sclerosis classical Hodgkin lymphoma appears as ___ on microscopy
Nodular sclerosis classical Hodgkin lymphoma appears as lymph node tissue separated by fibrous bands into nodules on microscopy * Gross: nodules
47
\_\_\_ has bilobed nuclei with prominant nucleoli
Reed-Sternberg cell has bilobed nuclei with prominant nucleoli
48
This is \_\_\_
Reed-Sternberg cell (classical Hodgkin lymphoma)
49
Reed-Sternberg cell phenotype is \_\_\_
Reed-Sternberg cell phenotype is CD30+, CD15+, PAX5+, CD3-, and CD20-
50
Extranodal NK / T cell lymphoma nasal type \_\_\_, \_\_\_, \_\_\_, and \_\_\_
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
This is \_\_\_
Anaplastic large cell lymphoma
52
This is \_\_\_
Classical Hodgkin lymphoma, nodular sclerosis type

Heme / Onc (41 decks)

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