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WILLIAM'S HEMATOLOGY > 95 Pathology of Lymphomas > Flashcards

95 Pathology of Lymphomas Flashcards

1
Q

Four main groups of lymphoid malignancies

A
  • Precursor B- and T-cell neoplasms
  • Mature B-cell neoplasms
  • Mature T- and natural killer (NK)-cell neoplasms
  • Hodgkin lymphomas
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2
Q

The cornerstone of lymphoma diagnosis of lymphoma where assessment of lymph node architecture and cell morphology is performed

A

Hematoxylin-eosin stain

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3
Q

Are neoplasms of precursor lymphoid cells committed to the B or T lineage.

A

Lymphoblastic leukemia/lymphomas

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4
Q

Most cases of lymphoblastic lymphoma are of _______lineage

A

T-cell lineage

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5
Q

Common site of involvement of lymphoblastic lymphoma

A

Mediastinum

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6
Q

MORPH: small- to intermediate-size cells with finely dispersed nuclear chromatin, inconspicuous nucleoli, and scant cytoplasm

A

PRECURSOR LYMPHOID NEOPLASMS

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7
Q

Immature markers of lymphoblastic neoplasms:

A
  • Terminal deoxynucleotide transferase, CD1a, and/or CD99
  • B Cell: CD19, CD79, and CD22
  • T Cell: CD3 and CD7
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8
Q

A neoplasm composed of monomorphic mature B lymphocytes that coexpress CD5 and CD23

A

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)

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9
Q

Disease presents with monoclonal B-lymphocyte counts greater than or equal to 5 × 109/L in the blood

A

CLL

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10
Q

Present clinically with lymphadenopathy or tissue disease with a number of blood monoclonal lymphocytes less than 5 × 109/L

A

SLL

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11
Q

Immunophenotype of CLL/SLL

A

CD5 and CD23 expression, dim expression of CD20, and weak surface clonal immunoglobulin chains

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12
Q

Epigenetic subtype of CLL and prognosis

A
  • Naïve B lymphocytes (naïvelike): unfavorable
  • Memory B lymphocytes (memorylike): favorable
  • Intermediate: intermediate
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13
Q

Most common genetic alterations in CLL

A
  • deletions in 13q14.3
  • trisomy 12
  • deletions of 11q22-24 (ataxia-telangiectasia mutated gene [ATM])
  • del 17p13 (the gene coding for tumor protein p53 [TP53])
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14
Q

Associated with transformation/Richter syndrome

A

TP53 and NOTCH1 mutations, MYC translocations, and homozygous deletions of CDKN2A

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15
Q

A neoplasm of small B lymphocytes with plasmacytoid features, usually involving marrow and sometimes spleen, lymph nodes, and, less often, blood

A

Lymphoplasmacytic lymphoma

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16
Q

Lymphoplasmacytic lymphoma is often associated with this monoclonal immunoglobulin that can cause hyperviscosity or cryoglobulinemia (Waldenström macroglobulinemia)

A

Monoclonal immunoglobulin M serum protein

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17
Q

A commonly recurring and highlyspecific feature of Waldenström macroglobulinemia

A

MYD88

Mutations in CXCR4 are present in a minority of cases

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18
Q

MORPH: small lymphocytes and cells with plasmacytoid features (eccentric nuclei and bluish cytoplasm)

A

LYMPHOPLASMACYTIC LYMPHOMA

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19
Q

A mature B-cell lymphoma typically composed of monomorphic small- to medium-size lymphocytes with irregular nuclei that express CD5 but are negative for CD23

A

Mantle cell lymphoma (MCL)

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20
Q

It most commonly has a diffuse growth pattern, but it can show annodular or, more rarely, a mantle zone pattern

A

Mantle cell lymphoma (MCL)

associated with gastrointestinal tract (lymphomatous polyposis)

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21
Q

Translocation in Mantle cell lymphoma (MCL)

A

t(11;14)(q13;q32) translocation which results in the expression of cyclin D1

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22
Q

Protein whose expression is also highly specific to MCL, can be used as an immunohistochemical marker to diagnose cases that are negative for cyclin D1

A

SOX11

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23
Q

Condition where Cyclin D1–positive lymphocytes in mantle zones can be an incidental finding in reactive lymphoid follicles

A

In situ mantle cell neoplasia

Very indolent behavior and do not require treatment

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24
Q

A neoplasm composed of germinal center B cells (GCBs) with a variable mixture of centrocytes and centroblasts

A

Follicular lymphoma (FL)

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25
Q

The cells retain expression of germinal center markers (BCL6, CD10, LMO2), and demonstrate a nodular architecture

A

Follicular lymphoma (FL)

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26
Q

FL can be graded (grades 1–3) based on the number of centroblasts present within the nodules.

A

Both grade 1 and grade 2 tumors are indolent, and distinguishing between them is generally not helpful.

Grade 3 lesions (>15 centroblasts per high-power microscopic field) can be further divided into:
- grade 3A (centroblasts and centrocytes both present)
- grade 3B (depletion of small centrocytes).

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27
Q

Mutation common in Follicular lymphoma (FL)

A

t(14;18)(q32;q21)
involving rearrangement of the BCL2 gene

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28
Q

A helpful tool to distinguish FL from reactive follicles

A

Expression of BCL2 protein

Reactive follicles are BCL2- negative

29
Q

Variant of FL usually lacks the BCL2 rearrangement and is characterized by large localized tumors in the inguinal region

A

Diffuse variant of FL

30
Q

Characteristics of testicular FL

A
  • High cytological grade, has a good prognosis, with surgical excision
  • Lacks a BCL2 translocation
  • Higher frequency in children
31
Q

Characteristics of pediatric type FL

A
  • BCL2-negative
  • Do not carry the t(14;18)
  • Show low genetic complexity
  • Recurrent alterations of TNFRSF14 and MAP2K1 mutations
  • Prognosis is excellent achieving complete remission after removal of the affected lymph node
32
Q

Characterized by a proliferation of various types of B cells, in variable proportion: small lymphocytes, lymphocytes with centrocytic morphology, lymphocytes with abundant pale cytoplasm (monocytoid B cells), and plasma cells.

A

Marginal zone lymphomas (MZL)

33
Q

Based on the site of presentation, MZLs can be divided into:

A

(a) extranodal MZL of mucosa-associated lymphoid tissue (MALT)
(b) splenic MZL
(c) nodal MZL

34
Q

The most common type of MZL and arise in mucosal sites subject to longstanding chronic inflammation

A

Extranodal MZL of the MALT type

35
Q

Four translocations associated with the MALT subtype of MZL

A
  • t(11;18)(q21;q21)
  • t(1;14)(p22;q32)
  • t(14;18)(q32;q21)
  • t(3;14)(p14.1;q32)
36
Q

Splenic MZL carries these mutations

A

7q deletion

NOTCH2 and KLF2.31

Nodal MZL do not have any of the aforementioned genetic alterations

37
Q

Characterized by the presence of large (nuclear size more than twice the normal lymphocyte, or equal to or larger than a normal macrophage) B lymphocytes that usually resemble centroblasts or immunoblasts

A

Large B-cell lymphomas (LBCLs)

38
Q

The most common type of Large B-cell lymphoma (LBCL)

A

DLBCL not otherwise specified (DLBCL, NOS) 25-30%

OTHERS:
Primary mediastinal LBCL, Primary CNS, primary cutaneous, leg type, or intravascular lymphoma

39
Q

Frequent mutation in primary CNS, primary cutaneous, leg type, or intravascular lymphoma

A

MYD88/CD79a

40
Q

Large B-cell lymphoma (LBCL) are associated with infectious agents such as

A

Epstein-Barr virus (DLBCL, EBV+) or
Human herpesvirus 8 (primary effusion lymphoma)

41
Q

TRUE OR FALSE

Patients with a ABC-subtype usually have a significantly better prognosis compared with the GCB-subtype.

A

FALSE

Patients with a GCB-subtype usually have a significantly better prognosis compared with the ABC-subtype.

42
Q

Main mutations in DLBCL based on cell of origin

A
  • GCB:EZH2/BCL2 translocations and histone genes/PI3k pathway
  • ABC: MYD88/CD79a, NOTCH1, and inactivation of tumor suppressor genes
  • Unclassified DLBCL: NOTCH2/BCL6 translocations
43
Q

Term reserved for LBCLs of variable morphology with MYC rearrangement in combination with BCL2 and/or BCL6 (referred to as a “double-hit” [DH] or “triple-hit” [TH] lymphomas)

A

High-Grade B-Cell Lymphoma

44
Q

MORPH: large cells with abundant cytoplasm, associated with diffuse fibrosis

A

Primary mediastinal large B-cell lymphoma (PMBL)

45
Q

MORPH: diffuse infiltrate of intermediate-size cells with a high mitotic rate and a “starry sky” appearance caused by numerous macrophages that have engulfed apoptotic debris (known as tingible body macrophages)

A

Burkitt lymphoma (BL)

46
Q

BL arises in three epidemiologic settings:

A
  • Endemic
  • Sporadic
  • In the context of immunodeficiency
47
Q

Mutation in BL

A

translocations involving the MYC gene on chromosome 8
t(8;14)(q24;q32)

Positive for CD10 and BCL6; negative or focally weakly positive for BCL2; growth fraction near 100% as determined by Ki67 staining

48
Q

The most common subtype of mature T-cell lymphomas

A

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS)

49
Q

Presents with marked systemic symptoms and polyclonal hypergammaglobulinemia, and arises from follicular helper T-cells

A

Angioimmunoblastic T-cell lymphoma

50
Q

Originates in the small bowel from a background of celiac disease

A

Enteropathy-associated T-cell lymphoma

51
Q

An Epstein-Barr virus (EBV)–associated neoplasm commonly involving the nasal cavity

A

Extranodal NK-/T-cell lymphoma, nasal type

52
Q

MORPH: medium-to-large pleomorphic cells, with some cases displaying characteristic “hallmark” cells with horseshoe- or kidney-shaped nuclei and a perinuclear eosinophilic region

A

Anaplastic large-cell lymphoma (ALCL)

53
Q

ALCL is characterized by uniform, strong expression of _____

A

CD30

54
Q

ALCL is divided into two entities based on the expression of

A

Anaplastic lymphoma kinase (ALK)

55
Q

ALCL type most often seen in the first three decades of life and has a favorable prognosis

A

ALK-positive ALCL

56
Q

The most common translocation in ALCL

A

t(2;5) (p23;q35)

Involving the nucleophosmin gene on chromosome 5

57
Q

ALCL tend to occur in older patients and have a more aggressive behavior

A

ALK-negative ALCL

Different genetic abnormalities have been identified in these cases involving DUSP22 (with a better prognosis) and TP63 (with a worse prognosis) genes.

58
Q

The characteristic tumor cell in classical Hodgkin lymphoma

Large, with abundant cytoplasm and often two or more nuclei or nuclear lobes, each of which contains a large eosinophilic nucleolus

A

Reed-Sternberg cell

The mononucleated forms are referred to as “Hodgkin cells.”

59
Q

MORPH:Large, with abundant cytoplasm and often two or more nuclei or nuclear lobes, each of which contains a large eosinophilic nucleolus; must be found in an appropriate background, consisting of a variable polymorphous reactive infiltrate of predominantly T lymphocytes and other inflammatory and accessory cells

A

CLASSICAL HODGKIN LYMPHOMA

60
Q

Reed-Sternberg cells express_____ in almost all cases of classical Hodgkin lymphoma and _____in the majority

A

CD30
CD15

61
Q

Classical Hodgkin lymphoma is associated with EBV in _________ of cases.

A

20% to 40%

62
Q

The most common subtype of classical Hodgkin lymphoma

A

Nodular sclerosis variant

63
Q

MORPH: broad collagen bands dividing the tumor into nodules and by the presence of “lacunar” cells, Reed-Sternberg cells with retraction artifact

A

Nodular sclerosis variant

64
Q

The second most common subtype classical Hodgkin lymphoma

A

Mixed cellularity variant

65
Q

MORPH: abundant Reed-Sternberg cells in a mixed inflammatory background without the broad collagen bands seen in nodular sclerosis

A

Mixed cellularity variant

66
Q

MORPH: small number of Reed-Sternberg cells in a background of small lymphocytes with absent or rare eosinophils and neutrophils

A

Lymphocyte-rich variant

**confused with nodular LP Hodgkin lymphoma, as well as T cell–rich B-cell lymphoma

67
Q

MORPH: increased number of large, atypical cells, commonly with bizarre multinucleated cells, with a minor reactive component

A

Lymphocyte-depleted variant

**overlap morphologically with ALCL or DLBCL

68
Q

MORPH: large with a single nucleus that contains multilobated or folded features, with nucleoli (also called “popcorn cells”)

A

Nodular lymphocyte-predominant (LP) Hodgkin lymphoma

69
Q

Main difference of Nodular lymphocyte-predominant (LP) Hodgkin lymphoma from classical HL

A

Retain expression of CD45 and B-lineage markers (CD20, CD79a, PAX-5, immunoglobulins)

Negative for CD15 and CD30

WILLIAM'S HEMATOLOGY (109 decks)

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