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*MHD Exam 6 > Chronic Leukemias and Lymphomas > Flashcards

Chronic Leukemias and Lymphomas Flashcards

1
Q

What are the 3 main types of chronic leukemias?

A
  1. Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)
  2. Hairy cell leukemia
  3. Adult T-cell leukemia/lymphoma
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2
Q

What is the most common leukemia of adults in the Western world?

A

CLL

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3
Q

CLL is a B-cell neoplasm that typically expresses which cell markers?

A

CD20, CD23, CD5 (pan T-cell)

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4
Q

Most CLL patients have co-existing:

A

immune dysfunction and hypogammaglobulinemia

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5
Q

Some CLL patients may transform to a higher grade process (prolymphocytic leukemia or diffuse large cell lymphoma), which is known as:

A

Richter syndrome/transformation

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6
Q

What is CLL prognosis based on?

A

presence of mutations in the Ig heavy chain gene (IGH) - those WITH the mutation have a favorable outcome

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7
Q

“soccer ball” appearance on peripheral blood smear

A

CLL/SLL

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8
Q

What are the 2 types of cells seen in CLL/SLL on blood smear?

A
  • small round lymphocytes (condensed chromatin, scant cytoplasm)
  • disrupted tumor cells (smudge cells)
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9
Q

Describe lymph node histology of CLL/SLL.

A

The lymph node is monotonous-appearing with numerous small cells.

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10
Q

Why is CLL also called SLL?

A

CLL is a systemic disease, and organ involvement outside of the marrow (spleen and liver) is known as small lymphocytic leukemia (SLL).

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11
Q

What is the key histologic feature of hairy cell leukemia?

A

cytoplasmic, hair-like projections

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12
Q

How does hairy cell leukemia classically present?

A

pancytopenia, monocytopenia, splenomegaly (most common), hepatomegaly, infections

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13
Q

In which age group does hairy cell leukemia typically present?

A

middle aged men

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14
Q

Which lymphoid organs are involved in hairy cell leukemia?

A

always the bone marrow; spleen involvement is primarily red-pulp infiltration

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15
Q

Cytochemical staining shows what in hairy cell leukemia?

A

tartrate resistant acid phosphatase (TRAP)

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16
Q

What is the prognosis for hairy cell leukemia?

A

excellent prognosis, as it is slow-growing, with good response to gentle chemo

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17
Q

In which part of the world is adult T-cell leukemia/lymphoma prevalent?

A

southern Japan, West Africa, Caribbean (NOT very often in the U.S.)

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18
Q

What are the clinical features of adult T-cell leukemia/lymphoma?

A

skin lesions, hepatosplenomegaly, lymphocytosis, hypercalcemia

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19
Q

What is the prognosis of adult T-cell leukemia/lymphoma?

A

usually fatal; rarely, the tumor will involve only the skin and follow an indolent course

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20
Q

What is seen on peripheral blood smear with adult T-cell leukemia?

A

floret-like lymphocytes (CD4+)

21
Q

What is a predisposing factor to development of adult T-cell leukemia?

A

infection with HTLV-1

22
Q

What are the types of non-Hodgkin lymphomas?

A
  • B-cell (Follicular, Mantle Cell, Burkitt, Diffuse Large B-cell)
  • T-cell
23
Q

What are the classic cell markers used to diagnose lymphoma?

A
  • CD19, CD20 (B-cell)
  • CD3 (T-cell)
  • Myeloperoxidase (myeloid)
24
Q

What are the immune deficiencies implicated in lymphoma etiology?

A

AIDS, post-transplant, RA, SLE, Sjogren’s

25
Q

What are the viruses and bacteria implicated in lymphoma etiology?

A
  • Viruses: HTLV-I, EBV, HHV-8, Hep C

- Bacteria: H. pylori in gastric MALT

26
Q

What are the major categories of lymphoma etiologies?

A
  • Chromosomal abnormalities
  • Immune deficiencies
  • Viruses
  • Bacteria
  • Iatrogenic (radiation)
27
Q

What is needed to make a diagnosis of lymphoma?

A

lymph node or tissue biopsy

28
Q

What is the predominant phenotype of lymphoma?

A

B-cell (85%)

29
Q

True or false: Lymphomas derive from multiple different cells (polyclonal).

A

False! All lymphomas derive from a single cell and are therefore monoclonal.

30
Q

What are the 2 types of cells seen with follicular lymphoma?

A
  • Small cells with cleaved or irregular nuclei (centrocytes)
  • Larger cells with open nuclear chromatin and several nucleoli (centroblasts)
31
Q

What are the subtypes of diffuse large B-cell lymphoma?

A
  • Germinal center B-cell (GCB)

- Activated B-cell (ABC)

32
Q

Most DLBCLs express which cell marker?

A

CD20 (B-cell marker)

33
Q

Which cytogenetic abnormality is implicated in 30% of patients with DLBCL?

A

t(14;18) or rearrangements of BCL6 locus on chromosome 3q27

34
Q

What are “double hit” DLBCLs that have a particularly aggressive clinical course?

A

cases in which there are MYC translocations along with t(14;18) or BCL6

35
Q

Describe the clinical presentation of peripheral T-cell lymphoma (PTCL).

A

lymphadenopathy, eosinophilia, pruritus, fever, weight loss

36
Q

How is PTCL diagnosed?

A

It lacks a specific histologic feature, and the most important ways to make a diagnosis are immunophenotypic (CD3+ with loss of other pan T-cell marker) and genotypic analysis (PCR)

37
Q

Hodgkin lymphoma has a ________ age involvement.

A

bimodal (young adults/adolescents and older adults)

38
Q

Hodgkin lymphoma is characterized by which type of cells?

A

Reed-Sternberg

39
Q

What are the 4 subtypes of Hodgkin lymphoma?

A
  • Lymphocyte rich
  • Mixed cellularity
  • Lymphocyte depleted
  • Nodular sclerosis
40
Q

Reactive cells (non-neoplastic) in Hodgkin lymphoma are most likely due to what?

A

cytokines secreted by RS cells (IL5, IL6, IL13, TNFa, GM-CSF)

41
Q

What is the most common subtype of HL?

A

nodular sclerosis

42
Q

What is the difference between classical HL and nodular lymphocyte predominant HL?

A
  • Classical: CD15 and CD30 expression

- Nodular lymphocyte predominant: CD20 and CD45 expression

43
Q

Classical HL usually shows which type of cells, in addition to RS, RS variants, and reactive lymphocytes?

A

eosinophils

44
Q

Describe the histology of the nodular sclerosis subtype of HL.

A

fibrous nodular pattern with lacunar cells

45
Q

Describe the histology of the lymphocyte depleted subtype of HL.

A

paucity of lymphocytes and relative abundance of RS cells

46
Q

Describe the histology of the mixed cellularity subtype of HL.

A

RS cells + variants on a cellular background of eosinophils, plasma cells, T-lymphocytes, and histiocytes

47
Q

Describe the histology of the lymphocyte rich subtype of HL.

A

relative small lymphocytes predominate with few mononuclear or classic RS cells

48
Q

Describe the histology of the nodular lymphocyte predominant subtype of HL.

A

nodularity with predominance of mature lymphocytes and popcorn cell or L&H variants of RS cells

*MHD Exam 6 (20 decks)

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