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WILLIAM'S HEMATOLOGY > 103 Mature T-Cell and Natural Killer Cell Lymphomas > Flashcards

103 Mature T-Cell and Natural Killer Cell Lymphomas Flashcards

1
Q

Whereas B-cell lymphomas are often characterized by a specific immunophenotypic profile, T-cell lymphomas are often characterized by

A

Antigen aberrancy

In establishing the diagnosis of T-cell NHL, it is important to exclude a reactive process

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2
Q

Serologic testing for this virus is particularly important in establishing a new diagnosis of PTCL in a person from an endemic area

INITITAL WORKUP

In addition to physical examination, initial evaluation should include systemic imaging (preferably positron emission tomography with computed tomography [PET-CT] or CT of the chest, abdomen, and pelvis), marrow aspirate or biopsy, and laboratory evaluation

A

Human T-cell lymphotrophic virus (HTLV)-1

Because adult T-cell leukemia/lymphoma (ATL) represents approximately 9% of PTCL in such areas, is associated with a different prognosis, and usually requires alternative therapy.

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3
Q

Prognostic index for T-cell lymphoma (PIT) includes

A

Age, performance status, LDH level, and marrow involvement

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4
Q

IPTCLP score

A

Albumin, performance status, stage, and absolute neutrophil count

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5
Q

Most commonly used backbone for initial therapy of patients with PTCL

A

CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)

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6
Q

Teatment for patients with peripheral T-cell lymphomas with CD30 expression

A

Brentuximab vedotin (BV)

Clinical trial: ECHELON 2

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7
Q

The toxicity profile of BV-CHP was similar to CHOP but was associated with:

A

Increased risk of diarrhea

Less frequent resolution of neuropathy

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8
Q

Agents approved by FDA for relapsed/refractory PTCL

A
  • Romidepsin
  • Belinostat
  • Pralatrexate
  • BV
  • Allogeneic transplantation
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9
Q

Characterized by PTCL-NOS that expresses two or more TFH cell markers (ICOS, CXCL13, CD10, BCL6, PD1, SAP, CCR5) by immunohistochemistry

A

PTCL-NOS with T-follicular helper (TFH) phenotype

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10
Q

Translocation of ALK-negative ALCL possibly associated with a better prognosis

This mutation typically leads to reduced expression of the DUSP22 gene, which likely functions as a tumor suppressor

A

t(6;7)

Presence of p63 by immunohistochemistry delineated a higher risk population of ALK-negative ALCL.

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11
Q

A translocation producing an ITK-SYK fusion gene

Suggest a potential role for SYK inhibitors

A

t(5;9)

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12
Q

An ALK inhibitor, demonstrated significant activity in pediatric patients with relapsed ALK-positive ALCL

A

Crizotinib

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13
Q

Drugs with have preferential activity and duration of response in patients with AITL

A

Histone deacetylase (HDAC) inhibitors, such as belinostat and romidepsin

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14
Q

Both PTCL-NOS with TFH phenotype and AITL have recurrent mutations in genes involved in chromatin modification including ____

A

TET2

TET2 is involved in epigenetic control of transcription through DNA methylation and inactivating mutations of this gene were first identified in myeloid malignancies

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15
Q

The most common of the mature T-cell neoplasms, representing approximately 25% to 30% of the total cases of T-cell lymphoma in Western countries.

A

PTCL-NOS

Malignant cells are often characterized by antigen aberrancy

Deletion of one or more pan–T-cell antigens is frequently observed, along with rearrangements of the TCR.

Pruritus, peripheral eosinophilia, hypercalcemia, and hemophagocytic syndrome

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16
Q

The median age of diagnosis of PTCL-NOS is 60 years with a_______ predominance (2:1).

A

Male

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17
Q

The majority of cases of PTCL NOS arise in

A

Lymph nodes

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18
Q

The immunophenotype is typically that of a mature T cell expressing either a CD4 or CD8 phenotype, most commonly ______

A

CD4

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19
Q

Initially described as angioimmunoblastic lymphadenopathy with dysproteinemia

A

Angioimmunoblastic T-cell lymphoma

The male-to-female ratio is approximately 1:1, and the median age at diagnosis is approximately 65–70 years.

Common features at presentation include B symptoms (fever, drenching sweats, and weight loss), generalized lymphadenopathy, rash, polyclonal hypergammaglobulinemia, blood eosinophilia, and autoimmune hemolytic anemia (direct Coombs positive).

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20
Q

Characterized histologically by effacement of the normal lymphoid architecture with a pleomorphic cellular infiltrate and proliferation of small arborizing blood vessels

Loss of germinal centers and extensive intranodal neovascularization and expansion of the follicular dendritic cell meshwork (CD21).

A

ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA

Scattered EBV+ B cells are almost always present

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21
Q

Marker typical for AITL, in approximately 90% of cases.

A

CD10

The malignant cells are CD4+ αβ T-cells with TCR-β and -γ rearrangements

Abnormal karyotypes involving the X chromosome and chromosomes 1, 3, and 5 are frequently found in AITL.

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22
Q

Most patients with AITL are treated with

A

CHOP-based regimens

The optimal initial therapy for patients with AITL remains unclear

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23
Q

A CD30+ peripheral T-cell neoplasm, provisionally subdivided into those that are ALK positive (60–70%) or negative (30–40%)

A

ANAPLASTIC LARGE CELL LYMPHOMA

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24
Q

Ninety percent of children with ALCL are ALK ________

A

ALK positive

The median age of ALK-positive ALCL is 30–40 years old.

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25
Q

Patients with ALK-_________ ALCL tend to be younger and have a better performance status and lower serum LDH levels.

The most chemosensitive of the T-cell lymphomas

A

ALK-positive

Approximately 8% to 12% of patients with ALK-positive ALCL have morphologic evidence of marrow involvement.

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26
Q

Patients with ALK-_________ ALCL tend to be older, with higher LDH values and a worse performance status

A

ALK-negative

Extranodal presentations are more common in the ALK-negative population, and include sites such as the marrow, liver, lung, and skin but rarely the CNS

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27
Q

This disorder is characterized by ALK-negative ALCL associated with textured saline and silicone breast implants.

A

Breast implant–associated anaplastic large cell lymphoma

The vast majority of patients present swelling of the breast associated with a seroma and presented with an effusion within the breast and less frequently with a distinct breast mass.

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28
Q

Three morphologic variants of ALCL based on the size of the neoplastic and admixed reactive cells.

A
  • Common type
  • Small cell variant
  • Lymphohistiocytic variant
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29
Q

ALK-positive ALCL is characterized by a nonrandom_________ translocation resulting in fusion of the NPM and ALK genes.

A

t(2;5) (p23;q35)

ALK immunoreactivity is a highly specific marker for this disease.

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30
Q

Pan-T antigens

A

CD2, CD2, CD4, CD5, and CD7

31
Q

Genes are typically overexpressed in ALK-positive ALCL

A

CEBPB, PTPN12, SERPINA1, and BCL6

32
Q

Genes are typically overexpressed in ALK-negative ALCL

A

CCR7, CNTFR, interleukin (IL)-21, and IL-22

33
Q

Associated with favorable prognosis among ALK-negative ALCL similar to ALK-positive ALCL

A

DUSP22

CD56

But those with TP63 expression are associated with an especially poor prognosis

34
Q

The most chemosensitive of the T-cell lymphomas

A

ALK-positive ALCL

35
Q

Previous treatment for ALCL

A

CHOP-based therapy

36
Q

The new standard of care in patients with previously untreated ALK-positive or ALK-negative ALCL

A

BV-CHP

37
Q

An inhibitor of ALK tyrosine kinase that is FDA approved for the treatment of ALK-positive non–small cell lung cancer, has demonstrated encouraging responses in small series of ALK-positive ALCL, leading to ongoing trials in relapsed/refractory ALCL

A

Crizotinib

38
Q

ALK-_________ ALCL typically presents with unfavorable features, including stages III and IV disease, B symptoms, high IPI scores, high serum LDH levels, and expression of TP63.

A

ALK-negative ALCL

39
Q

A mature T-cell lymphoma that presents within the gastrointestinal (GI) tract.

A

Enteropathy-associated intestinal T-cell lymphoma (EATL)

The median patient age at diagnosis is 55–65 years with a slight male predisposition.

40
Q

EATL comprises 60% to 80% of cases, is most common in patients who have underlying_______________, and is strongly associated with the human leukocyte antigen____________

A

Celiac disease

(HLA)-DQ2 haplotype

41
Q

Present in 20% to 40% of cases, is less frequently associated with celiac sprue and the HLA-DQ2 haplotype.

A

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL)

42
Q

EATL typically presents with

A

Acute abdominal symptoms

Ulcerative lesions of the jejunum or ileum

43
Q

The two types of EATL are recognized on the basis of the specific immunophenotype:

A

EATL: CD56 negative

MEITL: CD56 positive

44
Q

The histology of EATL demonstrates

A

Medium to large tumor cells with round or angulated vesicular nuclei, prominent nucleoli, and pale-staining cytoplasm

45
Q

EATL characteristically demonstrates the following immunophenotype:

A

Positive for CD3, CD7, CD103, and usually CD30 ; perforin, granzyme B, and TIA-1

Negative for CD4, CD5, CD8, and CD56

46
Q

MEITL characteristically demonstrates the following immunophenotype:

A

Positive for CD3, CD8, CD56, TCR-γ/δ or TCR-α/β

Negative for CD4, CD30

47
Q

Treatment used most widely for both EATL and MEITL

A

CHOP chemotherapy

48
Q

Adverse prognostic factors with EATL

A
  • History of celiac sprue
  • A large tumor mass (≥5 cm) at diagnosis
  • An elevated LDH
  • A nonambulatory performance status
49
Q

ADULT T-CELL LEUKEMIA/LYMPHOMA is an uncommon lymphoproliferative neoplasm of mature _____________T-cells

A

CD4+CD25+ T-cells

50
Q

ATL is caused by infection with the

A

Retrovirus HTLV-1

HTLV-1 is transmitted through breastfeeding, blood products, and unprotected sexual intercourse.

51
Q

ATL has cells classically have a leukemic _____________ appearance

A

“flower-cell” appearance

52
Q

At least ____% of circulating abnormal T lymphocytes are required to diagnose ATL in patients without histologically proven tumor lesions.

A

5%

53
Q

The mean age of patients with adult T-cell leukemia/lymphoma is 62 years, _________________ predominance.

A

Without a gender predominance

54
Q

Clinical variants of ATL

A
  • Acute variant 60%
  • Lymphoma variant 20%
  • Chronic
  • Smoldering
55
Q

Variant of ATL that presents with a predominance of skin lesions or lung infiltration without visceral or marrow disease and minimal blood involvement (<5% of lymphocytes).

A

Smoldering form of adult T-cell leukemia/lymphoma

56
Q

Variant of ATL that present with leukocytosis with lymphadenopathy and organomegaly without an elevated LDH level or visceral involvement.

A

Chronic ATL

57
Q

Characteristic of cells of ATL

A

Highly lobulated nuclei (“clover leaf” or “flower-cell” appearances) with condensed nuclear chromatin, inconspicuous nucleoli, and a mature helper T-lymphocyte immunophenotype.

In approximately 20% of cases, nuclear lobulation is less pronounced, and the cells may be difficult to distinguish from Sézary cells.

58
Q

Immunophenotype of ATL

A

Positive: CD2, CD4 and CD5, CD45RO, CD29, and TCR-αβ;

Negative for CD7, CD8, and CD26; and show reduced CD3 expression

The lymphocytic activation markers HLA-DP, -DQ, and -DR and IL-2Rα (CD25) are always present, whereas terminal deoxy-nucleotidyl transferase is typically absent.

59
Q

The prognosis in ATL remains poor with a median survival time of less than ________ year for patients with aggressive subtypes

A

1 year

Inadequacy of CHOP in this disease

The role of antiviral therapy remains controversial.

60
Q

Drug has been approved in Japan for the treatment of relapsed or refractory adult T-cell leukemia-lymphoma and is compendium listed in the United States by the National Comprehensive Cancer Network for the same patient population.

A

Mogamulizumab (anti-CCR4 monoclonal antibody)

61
Q

A rare lymphoma that infiltrates the spleen, liver, and marrow

In the majority of cases, cells consist of mature γ/δ T cells; however, α/β HSTCL has also been reported.

A

Hepatosplenic T-cell lymphoma (HSTCL)

HSTCL is a rare lymphoma, representing 3% of all T-cell lymphomas.

This disease typically occurs in young men at a median age of 35 years.

The optimal therapy for HSTCL is not known.

62
Q

Risk factors for Hepatosplenic T-cell lymphoma (HSTCL)

A
  • Immunosuppression after solid-organ transplantation or
  • Use of anti–tumor necrosis factor-α or thiopurine agents
63
Q

Common presentation of HSTCL

A

Isolated hepatosplenomegaly without lymphadenopathy

Frequently accompanied by cytopenias, B symptoms, and an elevated serum LDH level.

64
Q

Immunophenotype of Hepatosplenic T-cell lymphoma (HSTCL)

A

Positive: CD3, CD56, and TCR-δ

Negative for CD4 and usually CD8

Clonal rearrangement of the TCR-γ gene is usually present, and in most cases, the lymphoma cells have an isochromosome 7q [i7(q10)] along with trisomy 8, which also may be seen in the αβ variant of this disease.

65
Q

Other name for Extranodal natural killer (NK)/T-cell lymphoma (ENKTL), nasal type

A

Lethal midline granuloma, malignant granuloma, or angiocentric lymphoma

ENKTL represents approximately 2% to 9% of T-cell lymphomas.
The disease typically afflicts middle-aged men, with a median age of 50 years at diagnosis, but it may also affect children.

66
Q

TRUE OR FALSE

ENKTLs are almost exclusively extranodal.

A

TRUE

ENKTLs are almost exclusively extranodal.

Initial sites involved are often the nose and nasopharynx and occasionally the paranasal sinuses, tonsils, Waldeyer ring, and oropharynx.

67
Q

Histopathology of ENKTL

A

Angiocentric plesiomorphic small- or medium-size atypical lymphoid cells with vascular invasion and ischemic tissue necrosis.

68
Q

Immunophenotype of ENKTLs

A

Positive: CD2 and CD7 ; CD16, CD56, cytoplasmic CD3ε, and CD57

Negative for surface CD3

69
Q

Treatment for localized NK-/T-cell lymphoma

A

Combined chemotherapy and radiation therapy

70
Q

Drug that has a major single-agent activity in NK-/T-cell lymphomas and is incorporated into most modern regimens for this disease.

A

L-Asparaginase

In patients with localized ENKTL, it is standard to consolidate asparaginase-based chemotherapy with radiation therapy.

REGIMENS
SMILE (dexamethasone, methotrexate, ifosfamide, Lasparaginase, and etoposide)
Gemcitabine, oxaliplatin, and Lasparaginase (GELOX)

71
Q

A primary cutaneous T-cell lymphoma presenting with painful subcutaneous nodules.

Subcutaneous nodules that typically begin in the extremities

A

Subcutaneous panniculitis-like T-cell lymphoma (SPTL)

A disorder of adults with an average age at diagnosis in the mid to late 30s, although cases have also been reported in children.

SPTL has a female predominance with a male-to-female ratio of 0.5.144

72
Q

Characteristics of lesions of Subcutaneous panniculitis-like T-cell lymphoma (SPTL)

A

Atypical lymphoid cells and reactive histiocytes with admixed adipose tissue often associated with coagulation necrosis.

73
Q

Histologically, the cells of SPTL express an ________ phenotype.

A

α/β phenotype

The γ/δ phenotype of this disease is now classified as cutaneous γ/δ T-cell lymphoma.

Composed of mature CD8+ αβ cytotoxic T cells that express TIA-1, granzymes, and perforin genes.

74
Q

An anti–IL-2 receptor antibody coupled to diphtheria toxin, has been reported in two patients with evidence of activity in SPTCL

A

Denileukin diftitox

WILLIAM'S HEMATOLOGY (109 decks)

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