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WILLIAM'S HEMATOLOGY > 95 Pathology of Lymphomas > Flashcards

95 Pathology of Lymphomas Flashcards

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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
The cells retain expression of germinal center markers **(BCL6, CD10, LMO2)**, and demonstrate a nodular architecture
Follicular lymphoma (FL)
26
FL can be graded **(grades 1–3)** based on the number of centroblasts present within the nodules.
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).
27
Mutation common in Follicular lymphoma (FL)
t(14;18)(q32;q21) involving rearrangement of the **BCL2** gene
28
A helpful tool to distinguish FL from reactive follicles
Expression of BCL2 protein Reactive follicles are BCL2- negative
29
Variant of FL usually lacks the BCL2 rearrangement and is characterized by **large localized tumors in the inguinal region**
Diffuse variant of FL
30
Characteristics of testicular FL
* High cytological grade, has a good prognosis, with surgical excision * Lacks a BCL2 translocation * Higher frequency in children
31
Characteristics of pediatric type FL
* 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
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.
Marginal zone lymphomas (MZL)
33
Based on the site of presentation, MZLs can be divided into:
(a) extranodal MZL of mucosa-associated lymphoid tissue (MALT) (b) splenic MZL (c) nodal MZL
34
The most common type of MZL and arise in mucosal sites subject to longstanding chronic inflammation
Extranodal MZL of the MALT type
35
Four translocations associated with the **MALT subtype of MZL**
* t(11;18)(q21;q21) * t(1;14)(p22;q32) * t(14;18)(q32;q21) * t(3;14)(p14.1;q32)
36
**Splenic MZL** carries these mutations
7q deletion NOTCH2 and KLF2.31 ## Footnote **Nodal MZL do not have any of the aforementioned genetic alterations**
37
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
Large B-cell lymphomas (LBCLs)
38
The most common type of Large B-cell lymphoma (LBCL)
**DLBCL not otherwise specified (DLBCL, NOS)** 25-30% OTHERS: Primary mediastinal LBCL, Primary CNS, primary cutaneous, leg type, or intravascular lymphoma
39
Frequent mutation in **primary CNS, primary cutaneous, leg type, or intravascular lymphoma**
MYD88/CD79a
40
Large B-cell lymphoma (LBCL) are associated with infectious agents such as
Epstein-Barr virus (DLBCL, EBV+) or Human herpesvirus 8 (primary effusion lymphoma)
41
# TRUE OR FALSE Patients with a ABC-subtype usually have a significantly better prognosis compared with the GCB-subtype.
FALSE Patients with a **GCB-subtyp**e usually have a significantly **better prognosis** compared with the ABC-subtype.
42
Main mutations in DLBCL based on cell of origin
* 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
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**)
High-Grade B-Cell Lymphoma
44
MORPH: large cells with abundant cytoplasm, associated with diffuse fibrosis
Primary mediastinal large B-cell lymphoma (PMBL)
45
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)
Burkitt lymphoma (BL)
46
BL arises in three epidemiologic settings:
* Endemic * Sporadic * In the context of immunodeficiency
47
Mutation in BL
translocations involving the MYC gene on chromosome 8 t(8;14)(q24;q32) ## Footnote Positive for **CD10** and **BCL6**; negative or focally weakly positive for BCL2; growth fraction near 100% as determined by **Ki67** staining
48
The most common subtype of mature T-cell lymphomas
Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS)
49
Presents with marked systemic symptoms and **polyclonal hypergammaglobulinemia**, and arises from **follicular helper T-cells**
Angioimmunoblastic T-cell lymphoma
50
Originates in the small bowel from a background of **celiac disease**
Enteropathy-associated T-cell lymphoma
51
An **Epstein-Barr virus (EBV)–associated** neoplasm commonly involving the **nasal cavity**
Extranodal NK-/T-cell lymphoma, nasal type
52
MORPH: medium-to-large pleomorphic cells, with some cases displaying characteristic “hallmark” cells with horseshoe- or kidney-shaped nuclei and a perinuclear eosinophilic region
Anaplastic large-cell lymphoma (ALCL)
53
ALCL is characterized by uniform, strong expression of _____
CD30
54
ALCL is divided into two entities based on the expression of
Anaplastic lymphoma kinase (ALK)
55
ALCL type most often seen in the **first three decades** of life and has a **favorable** prognosis
ALK-positive ALCL
56
The most common translocation in ALCL
t(2;5) (p23;q35) Involving the **nucleophosmin gene** on **chromosome 5**
57
ALCL tend to occur in **older** patients and have a more **aggressive** behavior
ALK-negative ALCL ## Footnote Different genetic abnormalities have been identified in these cases involving **DUSP22** (with a better prognosis) and **TP63** (with a worse prognosis) genes.
58
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
Reed-Sternberg cell ## Footnote The mononucleated forms are referred to as **“Hodgkin cells.”**
59
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
CLASSICAL HODGKIN LYMPHOMA
60
Reed-Sternberg cells express_____ in almost all cases of classical Hodgkin lymphoma and _____in the majority
CD30 CD15
61
Classical Hodgkin lymphoma is associated with EBV in _________ of cases.
20% to 40%
62
The most common subtype of classical Hodgkin lymphoma
Nodular sclerosis variant
63
MORPH: broad collagen bands dividing the tumor into nodules and by the presence of “lacunar” cells, Reed-Sternberg cells with retraction artifact
Nodular sclerosis variant
64
The second most common subtype classical Hodgkin lymphoma
Mixed cellularity variant
65
MORPH: abundant Reed-Sternberg cells in a mixed inflammatory background *without* the broad collagen bands seen in nodular sclerosis
Mixed cellularity variant
66
MORPH: small number of Reed-Sternberg cells in a background of small lymphocytes with absent or rare eosinophils and neutrophils
Lymphocyte-rich variant ## Footnote **confused with nodular LP Hodgkin lymphoma, as well as T cell–rich B-cell lymphoma
67
MORPH: increased number of large, atypical cells, commonly with bizarre multinucleated cells, with a minor reactive component
Lymphocyte-depleted variant ## Footnote **overlap morphologically with ALCL or DLBCL
68
MORPH: large with a single nucleus that contains multilobated or folded features, with nucleoli (also called “**popcorn cells**”)
Nodular lymphocyte-predominant (LP) Hodgkin lymphoma
69
Main difference of Nodular lymphocyte-predominant (LP) Hodgkin lymphoma from classical HL
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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