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BIIC Cancers and Pharmo > Lymphomas > Flashcards

Lymphomas Flashcards

1
Q

Lymphoid neoplasms can be due to what 3 classes of cancer?

A
  1. Hodgkin’s and non-Hodgkin’s Lymphoma (masses on lymph nodes)
  2. Leukemia (bone marrow and peripheral blood)
  3. Plasma cell dyscrasisia (arise within bones and cause systemic symptoms while producing complete or partially incomplete monoclonal immunoglobin)
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2
Q

Myeloid neoplasms can be due to which cancerous diseases? (3)

A
  1. Leukemias
  2. Myelodysplastic syndromes
  3. Myeloproliferative neoplasms
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3
Q

______ ______ ______ manifest as histiocytic neoplasms.

A

Langerhans cell histocytosis

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4
Q

All neoplasms have the capacity to ______ to ______ ______ and other tissues such as ______, spleen, and ______ ______.

A

Spread; lymph nodes; liver; bone marrow

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5
Q

Non-Hodgkins Lymphoma precursor B and T neoplasms are due to ______ ______ ______ (______) and lymphoma composed of ______ B+T cells.

A

Acute lymphoblastic leukemia (ALL); immature

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6
Q

Non-Hodgkins Lymphoma peripheral B-cell neoplasms can be due to ______ ______ _____ (______) or ______ ______ ______ (______).

A

Chronic lymphocytic leukemia (CLL); Small lymphocytic lymphoma (SLL).

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7
Q

CLL is composed of cells mostly found in ______ ______.

A

Peripheral blood

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8
Q

SLL is composed of cells found in ______ ______.

A

Lymphatic tissue

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9
Q

What is FCCL?

A

Follicular Lymphoma

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10
Q

FCCL is the ______ ______ ______ of non-Hodgkin’s lymphoma in the US. Accounting for ______% of cases.

A

Most common form; 40

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11
Q

Translocation of FCCL?

A

T (14;18) which causes fusion of the BCL2 gene on chromosome 18 to the IgH locus on chromosome 14

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12
Q

What does translocation T(14;18) do?

A

Causes overexpression of BCL2 protein, inhibiting apoptosis.

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13
Q

Characteristics of FCCL morphology? (3)

A
  1. Increased nodular proliferation
  2. Larger centrocytes with cleaved nuclei, indentation, and condensed chromatin
  3. Centroblasts can be seen as large cells with vesicular chromatin and multiple, indistinct nucleoli
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14
Q

B cell markers of FCCL?

A

CD20, CD10, and BCL6.
BCL2 is expressed in more than 85% of cases.
Bone marrow is involved in 80% of cases.

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15
Q

Clinical features of FCCL? (7)

A
  1. Adults older than 50
  2. Affects males and females equally
  3. Usually painless
  4. No cure
  5. Median survival rate: 10 yrs after diagnosis
  6. Symptomatic and bulky diseases are treated with cytotoxic drugs and rituximab (anti-CD20)
  7. In 30-40% of cases, FCCL progresses to diffuse large B cell lymphoma; survival rate decreases to 1 yr.
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16
Q

What is MCL?

A

Mantle cell lymphoma

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17
Q

MCL consists of cells that resemble ______ ______ ______ found in the mantle zones of lymphoid follicles.

A

Naive B cells

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18
Q

MCL translocation?

A

T (11;14) which causes the fusion of the cyclin D1 gene to the IgH locus

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19
Q

What does the translocation T(11;14) do?

A

Causes the overexpression of cyclin D1 and leads to growth by promoting progression of cells from the G1 stage to the S stage.

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20
Q

Characteristics of MCL morphology? (5)

A
  1. Lymph nodes are in a diffuse or vaguely nodular pattern
  2. Tumor cells are larger with an irregular nucleus and scant cytoplasm
  3. Bone marrow is involved in most cases
  4. Peripheral blood is involved in 20% of cases
  5. Can arise in the GI tract as lymphomatous polyposis
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21
Q

B cell markers of MCL?

A

CD20 and CD5

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22
Q

Clinical features of MCL? (5)

A
  1. Most common in men younger than 50 yrs
  2. Contributes to 6% of total non-Hodgkin’s cases
  3. Presents with lymphadenopathy and fatigue
  4. Aggressive and incurable
  5. Survival rate is 4-6 years after diagnosis
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23
Q

What is EMZL?

A

Extranodal Marginal Zone Lymphoma

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24
Q

EMZL is an ______ B cell tumor that arises in _______ _______. It is sustained by _______ ______.

A

Indolent; epithelial tissues; chronic inflammation

25
Q

Triggers of chronic inflammation in EMZL include autoimmune disorders, _______ syndrome, Hashimoto’s thyroiditis, and ______ infection.

A

Sjogren’s; H. pylori

26
Q

In EMZL, H. pylori is associated with the _______ marginal zone. Eradication of H. pylori with antibiotics can lead to ______ of tumor cells. Without H. pylori, tumor cells ______ ______ have inflammatory ______ to ______.

A

Gastric; regression; will not; cytokines; survive

27
Q

In EMZL, chronic gastritis caused by H. pylori causes ______ of Ag-B cells.

A

Proliferation

28
Q

B cell clones depend on antigen __-______ _____ for growth and survival in EMZL.

A

T-helper cells

29
Q

Greater tumor cell autonomy and growth result from continued _______ _______ in EMZL.

A

Clonal evolution

30
Q

Characteristics of EMZL morphology?

A

Lymphoepithelial lesions.

31
Q

B cell markers in EMZL?

A

CD20 and IgM

32
Q

Clinical features of EMZL? (3)

A
  1. Swollen salivary glands, thyroid, and orbit
  2. Often discovered incidentally due to H. pylori gastritis or imaging
  3. Cured by simple excision followed by radiotherapy
33
Q

What is DLBCL?

A

Diffuse Large B cell Lymphoma

34
Q

DLBCL is the ______ _______ lymphoma in adults. ______% of total non-Hodgkin’s cases.

A

Most common; 35

35
Q

What are the 2 types of DLBCL and what are their associated genetic pathogeneses?

A
  1. Rearrangement in the BCL6 gene on 3q27 and an activating point mutation in the BCL6 promoter region, leading to increased production of the BCL6 protein
  2. T(14;18) involving BCL2 leading to the overexpression of the BCL2 protein
    * These changes in BCL6 and BCL2 do not occur together, hence, two types of DLBCL.
36
Q

Characteristics of DLBCL morphology?

A

Diffuse large B cell lymphoma with diffuse growth pattern.

Cell appearances can vary.

37
Q

B cell markers of DLBCL?

A

All types express CD20, IgM, and IgG.

CD10 and BCL6 are variably expressed.

38
Q

What are the 3 subtypes of DLBCL?

A
  1. EBV: occurs in the setting of AIDS, immunosuppression, transplant recipients, or the elderly
  2. Kaposi Sarcoma Herpesvirus: (HHV-8) associated with rare effusion in the pleural cavity, pericardium, and peritoneum
  3. Mediastinal Large B Cells: predominantly occurs in young women. Can spread to abdominal viscera and the CNS.
39
Q

Clinical features of DLBCL?

A
  1. Predominately affects individuals over age 60
  2. Results in rapidly enlarging symptomatic masses at one or several sites
  3. Extranodal presentations are common, especially in the GI tract
  4. Without treatment, the cancer is aggressive and fatal
  5. When treated with anti-CD20 (rituximab), 60-80% of individuals achieved remission and 50% of those remained cancer-free
40
Q

Where is Burkitt Lymphoma seen?

A

Endemic in Africa, but also presents in the US.

There are clinical and virological differences between them.

41
Q

Pathogenesis of Burkitt Lymphoma? (6)

A
  1. Translocation of MYC gene on chromosome 8 leads to overexpression of the MYC transcription factor, causing changes in Warburg metabolism (aerobic glycolysis)
  2. T(8;14): fuses with the IgH gene on chromosome 14
  3. There are also translocations involving kappa and gamma light chains
  4. Fastest growing human tumor
  5. In most endemic cases and 20% of sporadic cases, cells also become infected with EBV
  6. In endemic areas, other diseases like malaria impair immune competence allowing for B cell proliferation; T cells eliminate most infected B cells, but those that survive become lymphoma cells
42
Q

Characteristics of Burkitt Lymphoma morphology? (2)

A
  1. Tumor cells are intermediate in size, round, with 2-5 nucleoli
  2. Presence of tissue macrophages containing ingested debris leads to a “starry-sky” pattern
43
Q

B cell markers of Burkitt Lymphoma?

A

CD20, IgM, CD10, and BCL6

44
Q

Clinical features of Burkitt Lymphoma?

A
  1. Mainly affects children and young adults
  2. Arises at extranodal sites: maxillary and mandibular masses seen in endemic areas; abdominal tumors more common in the US
  3. Highly aggressive
  4. Very intensive chemotherapy can cure the disease
45
Q

Generalized characteristics of Hodgkin’s Lymphoma? (2)

A
  1. Reed-Sternberg Cells (RS cells): giant tumor cells with a muti-lobate nucleus and eosinophilic cytoplasm. Usually with two mirror-image acidophilic nuclear lobes manifesting in an “owl-eye” appearance.
  2. Arises in a single lymph node or chain, spreads anatomically.
46
Q

Characteristics of Hodgkin’s Lymphoma morphology? (3)

A
  1. Neoplasm arises from germinal B center
  2. EBV is present in 70% of cases (mostly in classical [mixed-cellularity subtype]). EBV contributes to tumor development.
  3. Non-neoplastic inflammatory cell infiltrate is generated by a number of cytokines
47
Q

B cell markers for Hodgkin’s Lymphoma?

A

Classic (first 4 types): CD15, CD30, but NOT CD45 (LCA) or B+T cell antigen
Lymphocyte Predominant Type (Non-classic): CD20, but NOT CD15 or CD30

48
Q

Clinical features of Hodgkin’s Lymphoma? (6)

A
  1. Painless lymphadenopathy
  2. Younger patients with earlier stages (I and II) are free of B symptoms
  3. Older adults typically have advanced stages (III and IV) and exhibit B symptoms (fever, weight loss, night sweats) + pruritus and anemia
  4. Very good prognosis
  5. Patients treated with radiotherapy have an increased risk of malignancy in the lung, breast, and CVD.
  6. Good response to Anti-PD1 in patients with relapsed disease
49
Q

What are the 5 types of Hodgkin’s Lymphoma?

A
  1. Nodular sclerosis HL
  2. Mixed cellularity HL
  3. Lymphocyte rich HL
  4. Lymphocyte depletion HL
  5. Lymphocyte predominant HL
50
Q

Characteristics of Nodular Sclerosis HL morphology? (2)

A
  1. Lacunar cells: multiple, small, multi-lobed cells with pale cytoplasm. Cytoplasm is torn away, leaving “empty-looking” cells.
  2. Collagen bands: Divide lymphoid tissue into nodules. The fibrosis can be scant or abundant. Cellular infiltrate contains lymphocytes, eosinophils, histiocytes, and lacunar cells.
51
Q

Clinical features of Nodular Sclerosis HL? (4)

A
  1. Most common form of HL
  2. Affects men and women equally
  3. Predominantly affects adolescents and young adults
  4. Involves the lower cervical, supraclavicular, and mediastinal lymph nodes.
52
Q

Characteristics of Mixed-cellularity HL morphology?

A

Many RS cells with a mix of small lymphocytes, eosinophils, plasma cells, and macrophages

53
Q

Clinical features of Mixed-cellularity HL? (4)

A
  1. Most common in older patients
  2. Affects men more than women
  3. 25% of total HL cases overall
  4. Associated with systemic manifestations (B symptoms)
54
Q

Characteristics of Lymphocyte Rich HL morphology? (2)

A
  1. Reactive lymphocytes make up majority of cellular infiltrate
  2. Presence of RS cells with classic profile
55
Q

Clinical features of Lymphocyte Rich HL?

A

Uncommon form

56
Q

Characteristics of Lymphocyte Depletion HL morphology? (2)

A
  1. Scarcity of lymphocytes

2. Large amount of RS cells

57
Q

Clinical features of Lymphocyte Depletion HL?

A

Least common. Less than 5% of cases.

58
Q

Characteristics of Lymphocyte Predominant HL morphology?

A

Variant of RS cells with presence of lymphohistiocytic (L&H) RS cells: multi-lobed puffy nucleus resembling “popcorn”. Usually found within large nodules containing small B cells and macrophages.

59
Q

Clinical features of Lymphocyte Predominant HL?

A
  1. 5% of cases. Non-classic.
  2. Cervical or axillary lymphadenopathy
  3. Excellent prognosis

BIIC Cancers and Pharmo (10 decks)

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