Myeloma and Lymphomas Flashcards

1
Q

What is the origin of most lymphomas?

A

B cell

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2
Q

What do B cells produce?

A

Immunoglobulin antibodies

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3
Q

What do T cells produce?

A

Cytokines

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4
Q

Which types of NHL are typically more aggressive but sometimes curable?

A

Larger cells

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5
Q

Which types of NHL are typically less aggressive but generally incurable?

A

Smaller cells

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6
Q

How does lymphadenopathy progress in NHL?

A

Spreads non-contiguously from peripheral LN groups inward toward central LN groups

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7
Q

How does lymphadenopathy progress in Hodgkin’s lymphoma?

A

Spreads contiguously from central LN groups outward

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8
Q

Describe the diagnostic lymph node in NHL

A

Most of the cells are malignant

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9
Q

Describe the diagnostic lymph node in Hodgkin’s lymphoma

A
  • Only a minority of the cells are malignant (Reed-Sternberg)
  • Majority of cells are inflammatory and benign
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10
Q

What are Reed-Sternberg cells?

A
  • Malignant cells in a lymph node of Hodgkin’s lymphoma
  • “Owl eyed” appearance
  • CD 30 positive
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11
Q

MC adult leukemia?

A

CLL/SLL

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12
Q

Describe CLL/SLL

A

Identical - differ only in how they are initially diagnosed (SLL if found in a lymph node)

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13
Q

Typical lab findings of CLL

A
  • Absolute lymphocyte count over 5000 consisting of small mature B cells
  • CD 19 and 20 positive
  • CD 5 positive
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14
Q

Treatment of CLL/SLL

A
  • Not curable with chemo

- May be cured with allogeneic SCT (rarely used d/t morbidity)

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15
Q

What are CLL-like lymphoid leukemias?

A
  • Hairy cell leukemia

- Splenic marginal zone lymphoma/leukemia

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16
Q

Classic lab finding of hairy cell leukemia

A
  • Small lymphocytes w/hairy projections

- CD 19, 20, 11c, 103 positive

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17
Q

Treatment of hairy cell leukemia

A

Purine analogs (80% CR, 60% long term CR)

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18
Q

Gastric MALTs are frequently caused by what?

A

H pylori infections

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19
Q

Describe MALT lymphomas

A

Low grade lymphomas usually of lymphoid tissue a/w exocrine glandular tissue

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20
Q

What can cure some early stage MALT lymphomas?

A

Treatment of H pylori infections

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21
Q

What are follicular lymphomas characterized by?

A

Lymphocytes and CD 10, 20

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22
Q

MC adult lymphoma?

A

DLBCL

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23
Q

Describe DLBCL

A
  • MC adult lymphoma
  • Aggressive, frequently involves extra-nodal sites
  • CD 19 and 20 positive
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24
Q

Standard treatment of DLBCL?

A

R-CHOP

Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone

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25
Q

Describe Burkitt’s lymphoma

A
  • Highly aggressive
  • Endemic in sub-Saharan Africa
  • A/w EBV infection
  • Presents usually as a jaw mass (in Africa) or intra-abdominal mass (in US)
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26
Q

Prognosis of Burkitt’s lymphoma

A

90% CR

75% cure (long term CR)

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27
Q

Describe mantle cell lymphoma

A

Histologically low grade, but aggressive and incurable

28
Q

How does mantle cell lymphoma typically present?

A

Bowel obstruction or intra-abdominal mass

29
Q

Typical cytological findings of mantle cell lymphoma

A
  • CD20 and 5 positive

- Cyclin D1 overexpressed

30
Q

Median survival of mantle cell lymphoma

A

3.5 to 5 years

31
Q

What do T cell lymphomas typically involve?

A

Skin and subcutaneous tissue

32
Q

MC T cell lymphoma?

A

PTCL NOS

Peripheral T cell lymphoma not otherwise specified

33
Q

How do PTCL NOS cases present?

A
  • Nodal and skin involvement
  • Stage IV
  • CD4 and 5 positive
34
Q

Treatment of PTCL NOS

A

CHOP (but only 56% CR, 36% 5 yr overall survival)

35
Q

What is Mycosis Fungoides?

A
  • Cutaneous T cell lymphoma

- Develops from Sezary’s syndrome (once disease forms plaques, nodules, or lymphadenopathy it is called MF)

36
Q

What is Sezary’s syndrome?

A
  • Cutaneous T cell lymphoma
  • Most present w/ill defined diffuse red rash (“parapsoriasis”)
  • Called MF once disease forms plaques, nodules, or lymphadenopathy
37
Q

Treatment of Sezary’s/MF

A

Similar to psoriasis - light therapy (UVB) with or w/o psoralens or topical corticosteroids

38
Q

What type of lymphoma does NOT follow the rule that big cells in lymphomas are bad?

A

Anaplastic large cell lymphoma

39
Q

Describe Alk positive Anaplastic Large Cell lymphoma

A
  • Usually disease of 40 or younger

- Responds to CHOP with 75% 5 yr DFS

40
Q

Describe Alk negative Anaplastic Large Cell lymphoma

A
  • Disease of elderly
  • Skin only involvement
  • Usually indolent, but incurable
  • Poorly responsive to CHOP and poor prognosis
41
Q

Which type of anaplastic large cell lymphoma has a worse prognosis?

A

Alk negative

42
Q

Describe nasal NK T cell lymphoma

A
  • Asians
  • A/w EBV
  • Usually presents w/ulcerative lesion of nasal septum or sinuses
  • Chemo and RT therapy
43
Q

How does Hodgkin’s lymphoma present?

A
  • Central lymphadenopathy
  • Fever, wt loss, sweats
  • Spreads from one nodal group to adjacent groups
44
Q

Epidemiology of Hodgkin’s lymphoma

A
  • 50% cases a/w EBV

- Bimodal frequency (peak at 30 yo and 70 yo)

45
Q

Types of Hodgkin’s lymphoma

A

Classical and nodular

46
Q

Subtypes of classical Hodgkin’s lymphoma

A
  • Lymphocyte rich
  • Nodular sclerosis (MC)
  • Mix cellularity
  • Lymphocyte depleted
47
Q

MC type of classical Hodgkin’s lymphoma?

A

Nodular sclerosing

48
Q

Describe nodular sclerosing Hodgkin’s lymphoma

A
  • MC subtype of classical HL

- Mediastinum involvement of young adults

49
Q

Most aggressive variety of HL?

A

Lymphocyte depleted subtype of classical HL

50
Q

Which type of HL is MC in HIV pts?

A

Lymphocyte depleted subtype of classical HL

51
Q

Standard treatment for HL?

A

ABVD given every 2 weeks IV for 8-12 cycles

Adriamycin, Bleomycin, Vinblastine, DTIC

52
Q

Prognosis of HL?

A

Very curable

53
Q

Which malignancy has a high cure rate so that survivorship is a huge issue to consider?

A

Hodgkin’s lymphoma

54
Q

Myeloma and Waldenstrom’s are diseases of what?

A

Mature functioning B cells

55
Q

Myeloma and Waldenstrom’s are characterized by what?

A

Overproduction of immunoglobulins (from mature functioning B cells)

56
Q

Which IG is overproduced in Waldenstrom’s?

A

IgM

57
Q

Which IG is overproduced in myeloma?

A

IgG

can be IgA or rarely IgD

58
Q

Epidemiology of myeloma

A
  • Median age at onset is 65 yo

- 2x MC in AAs

59
Q

Prognosis of myeloma

A

Incurable but remission duration is improving

60
Q

Diagnosis of myeloma

A
  • CRAB (hypercalcemia, renal failure, anemia, bone lesions)
  • 10% or higher plasma cells in BM
  • 3 g/dl or more serum IgG/IgA
61
Q

Define MGUS

A

Monoclonal gammopathy of unclear significance (some progress to myeloma, most never do)

62
Q

Define smoldering myeloma

A

Disease between true myeloma and MGUS (50% develop myeloma within 5 yrs)

63
Q

Treatment of MGUS or smoldering myeloma

A

No standard tx - close observation

64
Q

Treatment of myeloma

A
  • Minimize end organ damage
  • Consider HSCT (if yes, avoid alkylating agents)
  • If not, alkylating agent plus biological response modifier
65
Q

Describe Waldenstrom’s

A
  • Usually 65 yo or older
  • IgM secretion by malignant cells
  • CD 19 and 20 positive
66
Q

Clinical presentation of Waldenstrom’s

A

Main symptoms are d/t the hyperviscosity caused by elevated IgM - lethargy, confusion, anemia, peripheral neuropathy

67
Q

Treatment of Waldenstrom’s

A
  • Plasmapharesis to control levels of IgM until chemo can be effective
  • Rituximab plus Cyclophosphamide