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WILLIAM'S HEMATOLOGY > 100 Marginal Zone B-Cell Lymphomas > Flashcards

100 Marginal Zone B-Cell Lymphomas Flashcards

1
Q

Most frequently involved anatomic compartment in Marginal zone lymphomas (MZLs)

A

Spleen and the mucosa-associated lymphoid tissues (MALT)

Lymph nodes may also be involved, albeit rarely

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2
Q

Three distinct subtypes of MZL

A
  • Extranodal MZL of MALT type (also termed extranodal marginal zone lymphoma [EMZL])
  • Splenic marginal zone lymphoma (SMZL)
  • Nodal marginal zone lymphoma (NMZL)

In addition, three provisional entities are recognized by the current WHO classification: pediatric nodal MZL, splenic diffuse red pulp lymphoma, and hairy-cell leukemia variant.

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3
Q

In adults, MZLs account for approximately _____of all non-Hodgkin lymphomas (NHLs)

A

10%

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4
Q

The most frequent MZL, being the third most frequent NHL and representing approximately 7.5% of all B-cell neoplasms

A

MALT lymphoma

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5
Q

TRUE OR FALSE

MALT lymphoma mostly affects middle-aged adults, at a median age of 60 years, with a slight female preponderance and often in association with chronic antigenic stimulation, either as a consequence of a chronic infection or a gastric autoimmune disease

A

TRUE

MALT lymphoma mostly affects middle-aged adults, at a median age of 60 years, with a slight female preponderance and often in association with chronic antigenic stimulation, either as a consequence of a chronic infection or a gastric autoimmune disease

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6
Q

Sites of EMZL or MALT lymphoma

A

Gut, the nasopharynx, and the lung, salivary glands, the ocular adnexa, the skin, the thyroid, the genitourinary tract

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7
Q

MALT lymphoma accounts for at least _____ of primary gastric lymphomas

A

50%

Middle-aged adults, with a female predominance
Common in the Northeastern part of Italy, an area where there is an area of high prevalence of Helicobacter pylori infection

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8
Q

Ocular and adnexal MZLs are associated with infection by

A

Chlamydophila psittaci

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9
Q

Microbial species thought to be associated with MALT lymphoma

A

H. pylori and Helicobacter heilmannii, Campylobacter jejuni, Borrelia burgdorferi, and Chlamydophila psittaci

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10
Q

Helicobacter pylori can be demonstrated by

Helicobacter pylori has been documented as the etiologic agent in more than 90% of patients with gastric MALT lymphoma

A

Histology, PCR, or urea breath test, culture

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11
Q

An infection by hepatitis C virus (HCV) can be documented in at least ____ of gastric and ____of nongastric MALT lymphomas

it is also associated with SMZL and NMZL

A

Half of gastric MALT lymphomas

One-third of nongastric MALT lymphomas

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12
Q

Associated Autoimmune Diseases

Sjögren syndrome :
Hashimoto thyroiditis:

A

Sjögren syndrome :EMZL of the salivary gland (44x)
Hashimoto thyroiditis: thyroid lymphoma (70x)

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13
Q

A key transcription factor that regulates the expression of several survival- and proliferation-related genes in B cells during immune responses and has the potential to promote an uncontrolled B-cell proliferation

A

NF-κB

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14
Q

The inactivation of this protein, a negative controller of the NF-κB pathway, is frequently encountered in EMZL

A

TNF-α–induced protein 3 (TNFAIP3)

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15
Q

The most common structural chromosomal abnormality in MALT lymphoma

A

t(11;18)(q21;q21) translocation

Demonstrated in 15% to 40% of the cases, especially in gastric and lung MALT lymphoma

Other chromosomal translocations include t(14;18)(q32;q21), t(1;14)(p22;q32) and t(3;14)(p13;q32), all involving the immunoglobulin heavy chain variable region (IGHV) gene on chromosome 14, and displaying a specific anatomic distribution

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16
Q

EMZL mostly presents as Ann Arbor classification stage _______

A

Stage IE disease

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17
Q

The most commonly involved organ in EMZL, accounting for approximately one-third of cases.

A

Stomach

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18
Q

Most frequently involved portion of the stomach in EMZL

A

Antrum

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19
Q

Macroscopic features of Gastric MALT lymphoma

A

Intragastric nodularities, enlarged rugal folds, thickened gastric walls, irregularly shaped superficial erosions, and shallow ulcers

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20
Q

MALT lymphomas of the ocular adnexa most often arise in the __________

A

Orbit (40% of cases)

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21
Q

The ______________is the site of origin in approximately 35% to 40% of the cases of MALT lymphomas of the ocular adnexa

A

Conjunctiva

The conjunctiva is the site of origin in approximately 35% to 40% of the cases, with bilateral involvement observed in nearly 15% of patients.

More rarely, the lymphoma originates from the lacrimal gland (10% of the cases) or the eyelid.

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22
Q

Cutaneous MALT lymphoma generally presents with papules, plaques, or nodules mainly involving the __________________

A

Trunk and the upper limbs

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23
Q

Morphology: highly characteristic of MALT lymphoma, particularly gastric MALT lymphoma, although they are not pathognomonic

A

Invasion or necrotic destruction of the glandular epithelium by infiltrating lymphoma cells

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24
Q

Immunohistochemistry of MALT lymphoma

A

CD20+, CD79a+, CD21+, and CD35+
Expressing immunoglobulin (Ig) M (less often IgA or IgG), with an immunoglobulin light-chain restriction

Negative: CD5, CD23, and CD10

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25
Q

The outcome of H. pylori eradication must be confirmed by

A

Urea breath test at least 6 weeks after eradication therapy and at least 2 weeks after proton pump inhibitors withdrawal

26
Q

Helicobacter pylori eradication therapy

A

Proton pump inhibitor, in association with either amoxicillin or metronidazole, and clarithromycin for 10–14 days

27
Q

Gastrectomy can be considered in gastric MALT lymphoma in the ff cases

A
  • Major hemorrhage
  • Massive infiltration of the gastric walls (with an enhanced risk of perforation during chemotherapy)
  • Pyloric stenosis
28
Q

Treatment options for nongastric MALT lymphoma and gastric MALT lymphoma patients who fail to respond to H. pylori eradication or who have no evidence of H. pylori infection

A

Involved-field radiation therapy (25–35 Gy)

Chemotherapy or rituximab immunotherapy, or a combination of both

29
Q

TRUE OR FALSE

Patients with the t(11;18) translocation are also usually responsive to alkylating drugs if administered as single agents

A

FALSE

Patients with the t(11;18) translocation are also usually unresponsive to alkylating drugs if administered as single agents

30
Q

Treatment options for patients with the t(11;18) translocation

A

Fludarabine with rituximab

Rituximab and chlorambucil

31
Q

Radioimmunotherapy used in patients with MALT lymphoma, being feasible and safe either at disease onset or at relapse

A

90Y-ibritumomab tiuxetan

32
Q

A tetracycline antibiotic, has been evaluated as the upfront treatment of patients with stage I ocular or adnexal MZL, showing activity against C. psittaci and inducing lymphoma regression

A

Doxycycline

33
Q

TRUE OR FALSE

MALT lymphoma has a favorable outcome, with a 5-year overall survival greater than 85% in most series

A

TRUE

MALT lymphoma has a favorable outcome, with a 5-year overall survival greater than 85% in most series

The reported median time to progression seems to be more favorable for GI MALT lymphomas than non-GI lymphomas; however, no significant differences in overall survival have been demonstrated between the two disease subgroups.

34
Q

3 parameters used in the MALT Lymphoma International Prognostic Index

A
  • Age 70 years or older
  • Elevated LDH
  • Ann Arbor stage III/IV

Three risk groups (low, 0 points; intermediate, 1 point; high, at least 2 points)

35
Q

Histologic transformation to diffuse large B-cell lymphoma of MALT Lymphoma occurs in approximately _____%

A

6% to 10%

36
Q

Risk factors for disease transformation to diffuse large B-cell lymphoma of MALT Lymphoma

A
  • Elevated LDH
  • Failure to achieve a complete remission after first-line course
  • Involvement of more than four nodal sites
37
Q

A mature B-cell neoplasm arising from postgerminal center lymphocytes and involving the white pulp follicles of the spleen, the splenic hilar lymph nodes, the marrow, and often the blood, showing characteristic neoplastic lymphoid elements referred to as “villous lymphocytes.”

A

SPLENIC MARGINAL ZONE LYMPHOMA

It accounts for 1% to 2% of all lymphomas, with a median age at diagnosis of nearly 65 years (range, 30–90 years) and a slight male predominance.

38
Q

SMZL is associated with this chronic viral infection

A

HCV

Result of the interaction of the viral E2 glycoprotein with CD81 on B cells

39
Q

SMZL associated with chronic HCV infection is often associated with

A

Mixed cryoglobulinemia

Cryoglobulinemic vasculitis

40
Q

Most frequent cytogenetic abnormalities in SMZL patients

A

Complete or partial 3q trisomy (30–80% of cases) and gains of 12q (15–20% of patients)

41
Q

The most significant karyotype aberrations, considered typical of SMZL, are ____________, which are reported in nearly 40% of the cases

A

Deletions or translocations involving 7q32

42
Q

Most significant clinical characteristics of SMZL

A

Asymptomatic splenomegaly and cytopenias

43
Q

In CBC,______________ is always present, and basophilic villous cells in blood may also be found

A

Lymphocytosis

Lymphocytosis is always present, and basophilic villous cells in blood may also be found

44
Q

Morphology of SMZL

A

A micronodular lymphoid infiltrate of small lymphocytes typically surrounds and replaces the splenic white pulp germinal centers in SMZL, with involvement of the red pulp also consistently observed

45
Q

Immunohistochemistry of SMZL

A

CD20+, CD23−, CD38−, CD5−, CD10−, cyclin D1−, IgD+

46
Q

Distinction of both follicular lymphoma and mantle cell lymphoma from SMZL

A

Expression of both CD5 and cyclin D1 by mantle cell lymphoma

CD10 Expression of follicular lymphoma

47
Q

Distinction of lymphoplasmacytic lymphoma (LPL) from SMZL

A

The presence of a very large IgM paraprotein spike (>10 g/L) favors the diagnosis of LPL rather than SMZL, though small IgM paraprotein spikes may also been seen in SMZL (usually <10 g/L).

48
Q

Treatment indications in SMZL

A

Symptomatic SMZL patients with massive splenomegaly causing pain, early satiety or

Significant cytopenias, defined as
* hemoglobin less than 100 g/L
* platelets less than 80,000/μL, or
* neutrophils less than 1000/μL

49
Q

Frontline treatment for SMZL

  • Symptomatic SMZL patients with massive splenomegaly causing pain, early satiety or
  • Significant cytopenias, defined as hemoglobin less than 100 g/L, platelets less than 80,000/μL, or neutrophils less than 1000/μ
A

Splenectomy

Does not influence marrow infiltration or blood lymphocytosis

50
Q

Systemic therapy in SMZL is required when:

A
  • Major contraindications to surgery exist,
  • Older adult patients
  • Those who relapse or progress after splenectomy
  • Advanced disseminated nodal disease
  • High-grade transformation
51
Q

Options for systemic therapy in SMZL

A

Rituximab, used both as a single agent or combined with chemotherapy

Chemotherapy regimens are based on alkylating agents (eg, chlorambucil or cyclophosphamide), fludarabine, or bendamustine

52
Q

Three variables used for prognostication in SMZL

A
  • Hemoglobin concentration (< 120 g/L)
  • LDH (if elevated at diagnosis)
  • Albumin levels (<35 g/L)

Low-risk group (no risk factors): 88% 5-year cause-specific survival rate
Intermediate-risk group (one risk factor): 73% 5-year cause-specific survival rate
Hiigh risk group (at least two risk factors): 50% cause-specific survival rate

53
Q

Nodal MZL is a mature, postgerminal center B cell lymphoma accounting for _________ % of all lymphomas, with a median age at onset of between 50 and 60 years

A

Less than 2%

54
Q

TRUE OR FALSE

The association with autoimmune phenomena is strong for Nodal MZL, in contrast to other MZLs

A

FALSE

The association with autoimmune phenomena is weak for Nodal MZL, in contrast to other MZLs

55
Q

Constitute a common marker of NMZL, occurring in 20% to 25% of patients and are shared with patients presenting with EMZL

A

Gain of several regions of chromosome 3

More than three-quarters of patients harbor somatic IGHV gene mutations and show a biased use of IGHV segments 3 and 4.

No typical cytogenetic aberrations have been demonstrated for NMZL

56
Q

The majority of patients affected by NMZL present with ____________

A

Disseminated peripheral and abdominal nodal involvement

wlaa dapat extranodal at spleen, puro nodes!

Marrow infiltration is seen in fewer than half of the patients, and blood involvement is rare.

57
Q

TRUE OR FALSE

In NMZL, extranodal disease is absent by definition, and the presence of splenomegaly should suggest a diagnosis of SMZL.

A

TRUE

In NMZL, extranodal disease is absent by definition, and the presence of splenomegaly should suggest a diagnosis of SMZL.

58
Q

Type of NMZL that arises in adolescent patients, with a striking predominance in males.

Atypical cells within lymph nodes show a marked pleomorphism and an interfollicular distribution with the marginal zones considerably expanded.

A

Pediatric NMZL

Patients generally present with localized stage I disease, which can be managed conservatively (surgical excision and observation), with low rates of disease recurrence.

59
Q

Treatment for limited-stage disease NMZL

A

Surgery and radiotherapy

No specific treatment consensus guidelines have been developed for NMZL, and patients are generally well managed using treatment paradigms developed for follicular lymphoma and other indolent NHLs.

60
Q

Treatment for symptomatic advanced stage disease NMZL

A

Immunochemotherapy

Rituximab may be combined with cyclophosphamide, vincristine, and prednisone (R-CVP);
Fludarabine-containing and anthracycline-containing regimens (eg, R-CHOP, which is based on cyclophosphamide, doxorubicin, vincristine and prednisone); or
Bendamustine

61
Q

Immunomodulatory and targeted agents used in immunomodulatory

A

Lenalidomide
Bortezomib
Ibrutinib
Inhibition of the Phosphatidylinositol 3-Kinase Pathway (Idelalisib, Duvelisib, Copanlisib, Parsaclisib, Umbralisib)

WILLIAM'S HEMATOLOGY (109 decks)

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