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Heme/Lymph Exam 1 (EH) > Chapter 13: Plasma Cell Neoplasms/Disorders and Uncommon Lymphoid Neoplasms > Flashcards

Chapter 13: Plasma Cell Neoplasms/Disorders and Uncommon Lymphoid Neoplasms Flashcards

1
Q

What is seen in Waldenstrom Macroglobulinemia?

A
  • High levels of IgM lead to sx’s of hyper-viscosity
  • Older adults, commonly in assoc. w/ lymphoplasmacytic lymphoma
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2
Q

Findings of multiple myeloma can be remembered with the mnemonic CRAB.

A
  • HyperCalcemia
  • Renal abnormalities
  • Anemia
  • Bone lytic lesions / Back pain
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3
Q

Multiple myeloma is more common in which sex, ethnicity, and what is peak age of incidence?

A
  • More common in men
  • African descent
  • Peak age = 60-70 y/o
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4
Q

Multiple myeloma is associated with diverse rearrangements of which gene and deletions of?

A

RearrangementsinvolvingIgH; deletions of13qandoverexpressionofD cyclins

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5
Q

High serum levels of what cytokine are seen in pt’s with active Multiple Myeloma and is associated with what?

A

IL-6 = important plasma cell GF; associted with poor prognosis

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6
Q

What finding in the peripheral blood of multiple myeloma is due to the high levels of M proteins; is it specific to MM?

A
  • Rouleaux formation = red cells sticking together in linear arrays
  • Characteristic finding, but NOT specific
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7
Q

Which cytologic variant seen in multiple myeloma is due to dysregulated synthesis and secretion of Ig leading to multiple grapelike cytoplasmic droplets within plasma cells of bone marrow?

A

Mott cells

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8
Q

Plasma cell tumors, like MM, are positive for what markers?

A

CD138 (aka syndecan-1) and often express CD56

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9
Q

What are the most significant complications associated with Multiple Myeloma?

A
  • Recurrent bacterial infections due to ↓ production of normal Igs = most common cause of death
  • Renal insufficiency from Bence-Jones proteinuria (light chains toxic to tubular epithelial cells)
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10
Q

What are the diagnostic lab findings for multiple myeloma; how is definitive diagnosis made?

A
  • Definitive dx requires BM exam; usually contains >30% plasma cells w/ considerable atypia
  • Typically >3 gm/dL of Ig (M protein) and/or >6 mg/dL of urinary Bence-Jones proteins
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11
Q

What is the most common monoclonal Ig (“M protein”) seen with Multiple Myeloma?

A

IgG

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12
Q

How are translocations involving cyclin D1, deletions of 13q or 17p, and the t(4;14) associated with prognosis in Multiple Myeloma?

A
  • Involvement of cyclin D1 = assoc. w/ good outcome
  • Deletions of 13q or 17p and the t(4;14) = more aggressive
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13
Q

What are sx’s of smoldering myeloma; findings in BM and serum?

A
  • Patients are asymptomatic; about 75% progress to MM over 15-year period
  • Plasma cells make up 10-30% of cells in marrow
  • Serum M protein is >3 gm/dL
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14
Q

What is the most common plasma cell dyscrasia?

A

Monoclonal Gammopathy of Uncertain Significance (MGUS)

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15
Q

How does solitary osseous plasmacytoma differ from extraosseous plasmacytomas in terms of progression?

A
  • Solitary osseous plasmacytoma almost invariably progresses to MM, over 10-20 years
  • Extraosseous plasmacytomas, particularly of upper respiratory tract can be cured by local resection
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16
Q

Characteristic sx’s and serum findings in Monoclonal Gammopathy of Uncertain Significance (MGUS)?

A
  • By definition, patients are asymptomatic!
  • Serum M protein levels is <3 gm/dL
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17
Q

Lymphoplasmacytic lymphoma is a neoplasm of which cells and most often arises in which age group?

A
  • B-cell neoplasm of older adults; usually 6th-7th decade
  • Superficial resemblence to CLL/SLL; but differs in that majority of tumor cells undergo terminal differentiation to plasma cells
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18
Q

In lymphoplasmacytic lymphoma, the plasma cell component most commonly secretes what and this leads to?

A

Secretes monoclonal IgM, often sufficient enough to cause hyperviscosity syndrome (aka Waldenstrom Macroglobulinemia)

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19
Q

How are the pathologic features of lymphoplasmacytic lymphoma different from MM?

A
  • Complications from secretion of free light chains (i.e., renal failure and amyloidosis) = rare
  • Bone destruction does NOT occur
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20
Q

Virtually all cases of lymphoplasmacytic lymphoma are associated with acquired mutations in what gene?

A

MYD88

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21
Q

What are dominant presenting complaints and common features seen in pt with lymphoplasmacytic lymphoma?

A
  • Usually non-specific and include weakness, fatigue, and weight loss
  • LAD, hepatomegaly, and splenomegaly in 50% pt’s
  • Anemia due to marrow infiltration is common
  • 10% pt’s have autoimmune hemolysis due to cold agglutinins
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22
Q

Lymphoplasmacytic lymphoma pt’s with higher levels of IgM (Waldenstorm Macroglobulinemia) are more likely to have what signs/sx’s?

A
  • Visual impairment
  • Neurologic problems; such as HA, dizziness, deafness, and stupor
  • Bleeding
  • Cryoglobulinemia; producing sx’s such as Raynaud’s and cold urticaria
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23
Q

Lymphoplasmacytic lymphoma is uncurable, but what can be done to alleviate the sx’s of a high IgM load?

A

Plasmapheresis

24
Q

Which disorder associated with monoclonal gammopathy is characterized by a demyelinating peripheral neuropathy?

A

POEMS syndrome(Polyneuropathy,Organomegaly,Endocrinopathy,Monoclonal gammopathy, andSkin changes)

25
Q

Virtually all mantle cell lymphomas have what translocation; which genes invovled?

A

t(11;14) creating cyclin D1-IgH fusion gene

26
Q

The t(11;14) cyclin D1-IgH fusion gene in mantle cell lymphoma contributes to the pathogenesis how?

A

Up-regulation of cyclin D1 promotes G1—> S-phase progression

27
Q

Mantle cell lymphoma most often presents when and in what sex?

A

5th-6th decased w/ male predominance

28
Q

Mucosal involvement of the small bowel or colon producing polyp-like lesions (lymphomatoid polyposis) is most often seen with what form of NHL?

A

Mantle Cell Lymphoma

29
Q

What is the most common presentation of Mantle Cell Lymphoma; which sites commonly involved?

A
  • Most common is painless LAD
  • Sx’s related to involvement of gut, spleen, liver, and BM are common
30
Q

Which makers expressed by Mantle Cell Lymphoma help to distinguish it from CLL/SLL?

A
  • CD5 (+) and CD23 (-)
  • Also express high levels of cyclin D1; most (+) for CD19 and CD20
31
Q

Prognosis of Mantle Cell Lymphoma?

A

Poor

32
Q

Which lymphoid neoplasm is associated with homogenous population of small lymphoid cells surrounding a small, atrophic germinal center?

A

Mantle Cell Lymphoma - t(11;14)

33
Q

Marginal Zone Lymphomas are considered to be of what origin?

A

Memory B-cell origin

34
Q

What are 3 exceptional characteristics of Marginal Zone Lymphomas which arise at extranodal sites?

A
  • Arise in tissue involved by chronic inflammatory disorders of autoimmune or infectious etiology (i.e., Sjogren, Hashimoto, H.pylori gastritis)
  • -* Remain localized for prolonged periods
  • May regress if inciting agent is eradicated
35
Q

Which 3 translocations may be seen in Marginal Zone Lymphomas?

A

t(11;18); t(14;18); or t(1;14) all upregulate BCL10 or MALT1

36
Q

Which type of transition is seen in development of Marginal Zone Lymphoma?

A

Polyclonal to monoclonal

37
Q

Hairy Cell Leukemia most often arises when and more often in which sex?

A

Middle-aged white males (median age = 55); 5x more common in men

38
Q

Hairy Cell Leukemia is associated with activating point mutations in what; what is the speicific AA substitution?

A

BRAF; specifically valine –> glutamate at residue 600

39
Q

Which markers are specific for Hairy Cell Leukemia?

A

CD11c, CD25, CD103 and annexin A1

40
Q

Most common presentation and lab findings for Hairy Cell Leukemia?

A
  • Massive splenomegaly, sometimes only finding
  • Pancytopenia due to infiltration of BM and spleen
41
Q

Patients with Hairy Cell Leukemia have increased susceptibility to what infections?

A

Atypical mycobacterial infections

42
Q

Behavior and prognosis of Hairy Cell Leukemia?

A
  • Generally indolent and exceptionally sensitive to “gentle” chemo
  • Prognosis = excellent
43
Q

How do pt’s with Peripheral T-Cell Lymphoma, Unspecified, generally present; diagnosis requires what?

A
  • Generalized LAD, sometimes w/ eosinophilia, fever, and weight loss

  • Diagnosis required immunophenotyping
44
Q

Anaplastic Large Cell Lymphoma is defined by the presence of what rearrangements?

A

ALK on chromosome 2p23

45
Q

Anaplastic Large Cell Lymphoma tend to occur in whom and present with what?

A
  • Occur in children or young adults; with soft tissue “masses”
  • Tumors tend to cluster about venules and infiltrate lymphoid sinuses, mimicking the appearance of metastatic carcinoma
46
Q

Prognosis of Anaplastic Large Cell Lymphoma w/ ALK rearrangements?

A

Very good

47
Q

What are the hallmark cells seen in Anaplastic Large Cell Lymphoma?

A

Large anaplastic cells w/ horseshoe-shaped nuclei and voluminous cytoplasma (so-called hallmark cells)

48
Q

Anaplastic Large Cell Lymphoma express which CD marker; which T cells are anaplastic?

A

CD30+; anaplastic CD8+ T cells

49
Q

Most common presentation of Adult T-Cell Leukemia/Lymphoma; behavior and prognosis?

A
  • Adults w/ skin lesions, generalized LAD, hepatosplenomegaly, peripheral bloodlymphocytosis, andHYPERcalcemia
  • Rapidly progressive disease, fatal in months to 1 year
50
Q

What is expressd by the tumor cells of Mycosis Fungoides?

A

Cutaneous leukocyte antigen (CLA); CCR4 and CCR10

51
Q

What are the salient clinical features/presentation of Mycosis Fungoides/Sezary Syndrome?

A
  • Adult pt’s w/ cutaneous patches, plaques, nodules or generalized erythema
  • Cells can spread to involve blood, producing Sezary syndrome; characteristic lymphocytes w/ cerebriform nuclei seen on blood smear
52
Q

Large granular lymphocytic leukemia has a T-cell and NK-cell variant and is associated with mutations in what?

A

STAT3

53
Q

Which CD markers are indicative of the T-cell vs. NK-cell variant of large granular lymphocytic leukemia; general behavior of each?

A
  • T-cell variant = CD3 (+) —> more indolent
  • NK-cell variant = CD3(-) and CD56(+) —> more aggressive
54
Q

Despite the presence of marrow involvement in large granular lymphocytic leukemia, what 2 findings dominate the clinical picture?

A

Anemia and neutropenia

55
Q

Some patients with what syndrome have large granular lymphocytic leukemia as the underlying cause?

A

Felty syndrome, which is triad of RA + Splenomegaly + Neutropenia

56
Q

Extranodal NK/T-cell lymphomas arise from what?

A

A single EBV-infected cell

Heme/Lymph Exam 1 (EH) (18 decks)

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