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WILLIAM'S HEMATOLOGY > 94 General Considerations of Lymphomas: Incidence Rates, Etiology, Diagnosis, Staging, and Primary Extranodal Disease > Flashcards

94 General Considerations of Lymphomas: Incidence Rates, Etiology, Diagnosis, Staging, and Primary Extranodal Disease Flashcards

1
Q

Lymphoma is more common in (men, women)

A

Men

The disease is more common in men with incidence rates of 23.9 and 16.2 in men and women, respectively.

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2
Q

The highest reported incidence rate is in

A

Israel

It is lowest of all in Asia

There are more cases of NHL in Asia than in the United States.

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3
Q

Incidence of different subtypes of NHL in different countries and continents

A

Follicular lymphoma: United States and Europe
Extranodal lymphoma: Japan
Burkitt lymphoma: sub-Saharan Africa
T-cell leukemia/lymphoma: southwest Japan, the southeastern United States, northeastern South America, and the Caribbean basin

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4
Q

An exponential increase in incidence in NHL among men and women occurs with increasing age ECXEPT for:

A

Lymphoblastic lymphoma: most commonly in children
Burkitt lymphoma : 20- to 64-year-old age group
Primary mediastinal B-cell lymphoma: median age of 35 years

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5
Q

Occupations with association with lymphoma

A

Crop farming but not animal farming, women’s hairdressers, cleaners, spray painters, carpenters and textile workers

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6
Q

Environmental factors and lymphoma

A

Cigarette smoking: NHL, follicular lymphoma
BMI above 30 kg/m2: DLBCL
Radiation exposure

Surprisingly, some studies have suggested an inverse relationship between an individual’s exposure to ultraviolet light and lymphoma incidence (especially DLBCL)

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7
Q

Infectious Agent: HTLV1

C-type RNA tumor virus

An acquired retrovirus that is not related to other known animal retroviruses

Also leads to a neurologic disorder called tropical spastic paraparesis

A

Adult T-cell leukemia/lymphoma (ATLL)

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8
Q

Infectious Agent: Epstein-Barr Virus

DNA virus in the herpesvirus family

A
  • Burkitt lymphoma
  • Posttransplantation lymphoma
  • HIV-associated lymphomas (immunodeficiency-related Burkitt lymphoma, primary CNS lymphoma, primary effusion lymphoma, the immunoblastic-plasmacytoid type of DLBCL, and oral cavity plasmablastic lymphoma)
  • Extranodal NK/T-cell lymphoma, nasal type
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9
Q

EBV binds to the _______

A

CD21 antigen (also the receptor for the C3d component of complement) on B lymphocytes

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10
Q

A three-step process in the development of Burkitt lymphoma has been proposed:

A

(a) EBV initiates a polyclonal proliferation of B cells;
(b) malaria or other infections further stimulate the proliferating B-cells; and
(c) the transforming B cells incur specific reciprocal translocations of chromosome 8 with chromosome 2, 14, or 22, resulting in a clonal expansion of B lymphocytes

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11
Q

Infectious Agent: Human Herpesvirus-8

In the homosexual population (mainly in the United States and Europe), HHV-8 is principally transmitted during repeated sexual contacts, whereas in Africa, it is mainly transmitted from mother to child and among siblings.

Saliva seems to play a major role in HHV-8 transmission.

A
  • Kaposi sarcoma
  • Castleman disease
  • Primary effusion lymphoma
  • Posttransplantation primary effusion lymphoma
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12
Q

Infectious Agent: Hepatitis B

A

DLBCL and follicular lymphoma

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13
Q

Infectious Agent: Hepatitis C

Associated with immunopathologic reactions, such as cryoglobulinemia, and frequently, clonality of infected B lymphocytes

A

DLBCL
Marginal zone lymphoma (MZL)
Lymphoplasmacytic lymphoma

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14
Q

Infectious Agent: Helicobacter pylori

Major cause of peptic ulceration and gastric cancer

A

Marginal zone mucosa-associated lymphoid tissue (MALT) lymphomas of the stomach

Transformation of a MALT or de novo DLBCL

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15
Q

Infectious Agent: Chlamydophila psittaci

A

Ocular adnexal lymphomas

The majority of ocular adnexal lymphomas are extranodal MALT lymphomas

Treated with doxycycline

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16
Q

Infectious Agent:Campylobacter jejuni

A

Immunoproliferative disease of the small intestine

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17
Q

Infectious Agent:Borrelia burgdorferi

A

B-cell lymphoma of the skin

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18
Q

TRUE OR FALSE

With the exception of common variable immunodeficiency and the syndromes present in childhood, many of the inherited immune deficiencies are X-chromosome linked; as a result, males are affected more commonly than females.

A

TRUE

With the exception of common variable immunodeficiency and the syndromes present in childhood, many of the inherited immune deficiencies are X-chromosome linked; as a result, males are affected more commonly than females.

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19
Q

This germline predisposition syndrome does not have an immunodeficiency phenotype. Rather, it is a nonsyndromic familial cancer syndrome transmitting susceptibility to mutations in p53. The cancers that occur in these families include lymphoma.

A

Li-Fraumeni syndrome

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20
Q

There is increased incidence of NHL in patients with rheumatological diseases receiving _______________ inhibitors

A

Tumor necrosis factor (TNF) inhibitors

The OR is 1.93 (95% CI, 1.16–3.20), and it is possible that the risk is higher with the TNF fusion protein etanercept than it is with inhibitory monoclonal antibodes.

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21
Q

Autoimmune disoreders: Sjögren syndrome

A

Parotid gland MZL (1000-fold increased risk)

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22
Q

Autoimmune disoreders: Hashimoto thyroiditis

A

Thyroid MALT lymphoma
MZL

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23
Q

Autoimmune disoreders: Lupus

A

MZL and DLBCL

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24
Q

Autoimmune disorders: Autoimmune hemolytic anemia

A

DLBCL

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25
Q

Autoimmune disorders: celiac disease, inflammatory bowel disease, and eczema

A

T-cell lymphoma

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26
Q

TRUE OR FALSE

The presence of such “B symptoms” has unfavorable prognostic significance in HL and NHL

A

FALSE

The presence of such “B symptoms” has unfavorable prognostic significance in HL, but these symptoms do not have independent prognostic significance for NHL

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27
Q

Recurrence of these symptoms after treatment may herald disease relapse of HL

A

Fatigue, rash, pruritus, and alcohol-induced pain

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28
Q

TRUE OR FALSE

During the physical examination, all enlarged lymph nodes (>1.5 cm) should be recorded.
Involved nodes typically are nontender, firm, and rubbery.

A

TRUE

During the physical examination, all enlarged lymph nodes (>1.5 cm) should be recorded.
Involved nodes typically are nontender, firm, and rubbery.

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29
Q

The single most important test in a lymphoma patient

A

Biopsy of affected tissue

Excisional lymph node biopsy of one of the largest nodes available, or generous incisional biopsy of an extranodal site.

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30
Q

When is core biopsy indicated

A

When the only sites of disease involvement are deep in the thorax or pelvis

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31
Q

TRUE OR FALSE

Narrow-diameter core biopsies or fine-needle aspiration alone should never be used as the sole method of establishing the initial diagnosis of lymphoma.

A

TRUE

Narrow-diameter core biopsies or fine-needle aspiration alone should never be used as the sole method of establishing the initial diagnosis of lymphoma.

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32
Q

FDG-avid lymphomas

A

HL, DLBCL, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, anaplastic large-cell lymphoma, and most subtypes of PTCL

ABDE(man-tEl)F-HP

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33
Q

Non FDG-avid lymphomas

Uses Contrast-enhanced CT

A

MZLs, chronic lymphocytic leukemia/small lymphocytic lymphoma, lymphoplasmacytic lymphoma/Waldenström macroglobulinemia, angioimmunoblastic T-cell lymphoma, mycosis fungoides, and cutaneous B-cell lymphomas

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34
Q

Preferred imaging for disease affecting the brain

A

Magnetic resonance imaging should be performed as well as a lumbar puncture

PET/CT scans are of limited value for the detection of disease affecting the brain

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35
Q

TRUE OR FALSE

Although marrow aspiration and biopsy have been standard in lymphoma staging in the past, the high sensitivity of PET/CT for marrow involvement has rendered these procedures less essential for patients with any kind of lymphoma who have negative PET/CT imaging of the bones and marrow.

A

FALSE

Although marrow aspiration and biopsy have been standard in lymphoma staging in the past, the high sensitivity of PET/CT for marrow involvement has rendered these procedures less essential for patients with HL and DLBCL who have negative PET/CT imaging of the bones and marrow.

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36
Q

Required diagnostics for full staging of other subtypes of lymphoma (aside from HL and DLBCL)

A

Single 2.5-cm core biopsy with flow cytometry and cytogenetics

37
Q

Important serum prognostic markers that should be assessed at baseline for most lymphomas

A

Lactate dehydrogenase and β2-microglobulin

38
Q

“B symptoms”

A
  • Fevers greater than 38.3 °C
  • Drenching night sweats
  • Unexplained weight loss of more than 10% of body mass over 6 months
39
Q

Bulky disease for HL

A

A nodal mass of ≥10 cm, or greater than one-third of the transthoracic diameter at any level of thoracic vertebrae

40
Q

Bulky disease for NHL

A

There is no consensus on the size of “bulk” for NHL with a suggestion that 6 cm may be optimal for follicular lymphoma.

Sizes between 6 cm and 10 cm have been advocated to define bulk for diffuse large B-cell lymphoma

41
Q

Indicate metabolic activity in tumor sites less than in the mediastinal blood pool, signifying complete metabolic response and CR

A

Deauville scores of 1–2

1- No significant FDG uptake in tumor site(s)above background
2- FDG uptake in tumor site(s) less than that in the mediastinal blood pool

42
Q

Indicate residual abnormal metabolic activity, representing treatment failure

A

Deauville scores of 4 or 5

4- FDG uptake in tumor site(s) moderately higher than in the liver
5- FDG uptake in tumor site(s) markedly higher than that in the liver and/or new FDG-avid lesions likely to be lymphoma

43
Q

Indicates metabolic activity greater than the mediastinum but less than the liver, it is indeterminant

A

Deauville score of 3

Most patients with HL or DLBCL who have a Deauville score of 3 at the end of treatment have a good outcome, but careful follow-up of such patients is required.

44
Q

Recommended follow-up for HL and DLBCL

A

Visits every 3 months during the first 2 years are recommended and then reduced to every 6 months for the next 3 years and annually thereafter

45
Q

Recommendation for followup imaging

A

NCCN: Contrast-enhanced CT imaging should not be performed more frequently than every 6 months for 2 years after the end of therapy for DLBCL and HL and then be discontinued

Lugano IWG guidelines: Discourage any surveillance CT imaging for asymptomatic patients with HL and DLBCL in CR at the end of therapy

46
Q

TRUE OR FALSE

There is general agreement that surveillance PET/CT imaging should not be carried out in asymptomatic patients in CR

A

TRUE

There is general agreement that surveillance PET/CT imaging should not be carried out in asymptomatic patients in CR

47
Q

Patients with incurable histologies require long-term follow-up and are usually observed every _____________

A

3–6 months

48
Q

Extranodal involvement that occurs as the only initial evidence of lymphoma after staging procedures

A

Primary extranodal lymphoma

49
Q

The histopathology of primary extranodal lymphoma is usually

A

MZL of MALT or DLBCL

50
Q

Usual treatment for Primary extranodal lymphoma

A
  • Immuno-chemotherapy multidrug regimen
  • Rituximab–cyclophosphamide, hydroxydaunorubicin, vincristine (Oncovin), and prednisone (RCHOP) or rituximab and bendamustine
51
Q

Use of radiotherapy in Primary extranodal lymphoma

A

Selected cases of extranodal lymphoma, other stage I–II lymphomas and for consolidation therapy of bulky adenopathy

52
Q

Primary CNS lymphomas are uncommon and almost always are of an aggressive histologic subtype, usually __________

A

DLBCL

There is a relatively high incidence of MYD88/CD79B mutations

53
Q

Treatment for Primary CNS lymphomas

A

Multiagent chemotherapy is usually given in younger fit patients with drugs that cross the blood–brain barrier such as a combination of methotrexate, thiotepa, cytarabine, and rituximab (MATRix)

Consolidation is often given with either an autologous stem cell transplant or whole-brain radiotherapy

54
Q

The most common orbital malignancy

A

Ophthalmic lymphoma

55
Q

The most frequent subtype of Ophthalmic lymphoma

A

MZL of MALT

56
Q

The most common site of ocular lesions

A

Periorbital soft tissues, particularly the conjunctival mucosal surfaces and the area surrounding the lacrimal gland

57
Q

The therapy for orbital MZL

A

Radiotherapy

58
Q

The therapy for orbital DLBCL

A

R-CHOP chemotherapy either alone or combined with local radiotherapy

59
Q

Intraocular lymphomas are a rare presentation of lymphoma of the eye. Most cases are

A

DLBCL

Lymphoma arising in the lacrimal sac was usually DLBCL

60
Q

The diagnosis of intraocular DLBCL is established by

A

Vitrectomy

61
Q

The therapy for intraocular DLBCL

A

High-dose methotrexate-based chemotherapy such as MATRix with or without intrathecal treatment and/or whole-brain and eye radiotherapy

62
Q

Localized NHL involving the nasal cavity and/or paranasal sinuses may be

A

DLBCL, T-cell lymphoma, or NK/T-cell lymphoma

63
Q

The predominant site of involvement in T-cell and NK/T-cell lymphoma

A

Nasal cavity

64
Q

Nasal NK/T lymphomas are typically treated with

A

L-asparaginase plus radiotherapy

65
Q

Patients with primary sinus lymphoma usually have

A

DLBCL

66
Q

The three main types of cutaneous B-cell lymphomas

A

Primary cutaneous marginal zone B-cell lymphoma
Primary cutaneous follicular center lymphoma
Primary cutaneous large B-cell lymphoma (leg type)

67
Q

Cutaneous B-cell lymphomas that are indolent types with an excellent prognosis

Treated primarily with nonaggressive therapies, including simple excision, glucocorticoid injections, or local radiotherapy.

A

Primary cutaneous marginal zone B-cell and primary cutaneous follicle center lymphoma

68
Q

A Cutaneous B-cell lymphoma with diffuse dermal infiltrate of neoplastic B-cells with extension to both the papillary dermis and the subcutaneous fat

Aggressive lymphoma that should be treated primarily with aggressive chemotherapy

A

Primary cutaneous large B-cell lymphoma (leg type)

69
Q

Primary pulmonary lymphoma histopathology is usually

A

Marginal zone B-cell lymphoma of the MALT or DLBCL

70
Q

A rare occurrence pulmonary lymphoma that may follow lung transplantation

A

Primary endobronchial lymphoma

71
Q

Most common location of lymphomatous masses in the heart

A

Right atrium

72
Q

Most cases of Primary cardiac lymphoma are

A

B-cell lymphoma

73
Q

The most common form of extranodal lymphoma, accounting for one-third of cases

A

Gastrointestinal lymphoma

74
Q

Most commonly involved site of the GI tract

A

Stomach

Followed by the small bowel, ileum, cecum, colon, and rectum

In the bowel, the small intestine, rectum, and colon may be involved, in that order of frequency

The intestinal location most often involved is the ileocecal region followed by small bowel, large bowel, and multiple intestinal sites.

75
Q

Diagnosis of Gastrointestinal lymphoma is made by

A

Endoscopic biopsy

76
Q

TRUE OR FALSE

GI Lymphomas: MALT lymphoma is common, but DLBCL also may arise de novo or may be found in the background of a MALT lymphoma.
If both subtypes of lymphoma are present, the treatment should be directed in treating MALT

A

FALSE

GI Lymphomas: MALT lymphoma is common, but DLBCL also may arise de novo or may be found in the background of a MALT lymphoma.
If both subtypes of lymphoma are present, the treatment should be directed at the large B-cell lymphoma

77
Q

The intestinal location most often involved is

A

Ileocecal region

78
Q

The most common disease location in the colon

A

Cecum

79
Q

TRUE OR FALSE

Primary lymphoma of the testes typically presents as a painless enlargement of the testis in an older man

A

TRUE

Primary lymphoma of the testes typically presents as a painless enlargement of the testis in an older man

80
Q

The histologic type usually of testicular lymphoma is

A

DLBCL

81
Q

Relapses of testicular lymphoma usually occurs in

A

Either the CNS or contralateral testicle

82
Q

The current international standard of care for testicular lymphoma

A

Orchiectomy, R-CHOP chemotherapy given every 21 days for six cycles, intrathecal methotrexate, and locoregional radiotherapy to the contralateral testicle

83
Q

Characteristic of primary renal lymphoma:

A

Bilateral enlargement of the kidneys without obstruction and other organ or nodal involvement and absence of other causes of renal failure

84
Q

When primary to the spleen, the lymphoma may be principally confined to the

A

Red pulp

85
Q

Histopathology of Primary splenic lymphoma

A

DLBCL or MZL

86
Q

Primary bone lymphoma may involve any bone but usually affects the

A

Long bones

87
Q

Histopathology of breast lymphoma

A

DLBCL

B-cell lymphoma 2 (BCL-2) gene expression is frequently present in the tumor cells.

88
Q

Breast lymphoma that may be the consequence of breast implants, especially those with a “rough” surface

A

T-cell anaplastic lymphoma of the breast

WILLIAM'S HEMATOLOGY (109 decks)

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