Hematology Week 3: Mature Lymphoid Neoplasms Flashcards

1
Q

Patient Presentation

A
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2
Q

Question 1

A

A Mature Lymphocytes

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3
Q

Most common mature lymphoid leukemia

A

Chronic Lymphocytic Leukemia (CLL)

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4
Q

CLL neoplasm of what kind of cells?

A

CLL is a neoplasm of mature monoclonal B-cells

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5
Q

What kind of cells?

A

Normal polyclonal B cells

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6
Q

What Kind of Cells?

A

Monoclonal B cells can choose only Kappa or Lambda, in this case this clonal expansion expressed only lambda

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7
Q

Clonal vs non-clonal

A

clonicity is a valuable diagnostic tool

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8
Q

Abnormal immunophenotype for CLL

A

CD5 and CD23 on B cells

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9
Q

CD5 is usually found on?

A

T cells

if found on B cells is indicative of CLL

or

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10
Q

CLL Peripheral blood smear characteristic features

5 listed

A
  • scant cytoplasm
  • dark nuclear chromatin
  • small
  • uniform size
  • “Smudge cells”
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11
Q

CLL in the lymph node

A

referred to as small lymphocytic lymphoma

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12
Q

CLL and SLL

A

are the same entity

same genetics

same treatment

the difference is if they are found in the blood or tissue vs BM

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13
Q

CLL vs SLL

A
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14
Q

Epidemiology of CLL/SLL

A
  • Most common leukemia of adults in the US
  • Patients typically elderly
  • often asymptomatic at diagnosis (incidental)
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15
Q

Most common leukemia in adults

A

CLL/SLL

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16
Q

CLL/SLL Characteristic Cell findings

A

monoclonal small B-cells with aberrant co-expression of CD5 and CD23

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17
Q

CLL/SLL Cytopenias from?

A

BM is filled up by CLL cells and is replaced by neoplastic cells

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18
Q

CLL/SLL immune disruption

2 listed

A
  • Hypogammaglobulinemia -> infections
  • Autoimmune phenomena -> anemia, thrombocytopenia
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19
Q

CLL/SLL clinical course

A

Typically indolent but variable

CLL follows the rule of 3rds

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20
Q

Staging in CLL

A

Rai System

&

Binet System

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21
Q

Lab studies for CLL/SLL

A
  • CBC+Diff
  • FISH (lesion at 17p is worst)
  • CD49d expression (when not expressed is better prognosis)
  • immunoglobulin heavy chain variable region somatic mutation (mutation is a good thing because that is what it is supposed to do)
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22
Q

When are CLL/SLL patients treated?

5 listed

A
  • treat symptoms
  • threatened end-organ function
  • progressive bulky disease
  • progressive anemia/thrombocytopenia
  • not curable so treat for symptom relief if the benefit is worth the cost of toxic treatment
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23
Q

Symptoms treated for in CLL/SLL

4 listed

A
  • Fever without infection
  • Weight loss
  • Night Sweats
  • Severe fatigue
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24
Q

the most common reason for treating CLL/SLL

A

Progressive anemia/thrombocytopenia

25
Q

How to treat CLL/SLL drug classes

4 listed

A
  • Monoclonal Antibody
  • Chemotherapy
  • Biologic agents
  • Combinations of them
26
Q

Rituximab

A
  • The prototype monoclonal antibody
  • Anti-CD20
  • Usually used in combo with chemo
27
Q

Fludarabine

A
  • Purine analog
  • specific toxicities
  • severe marrow toxicity
  • autoimmune phenomena
  • autoimmune hemolytic anemia
  • ITP
28
Q

Bendamustine and Chlorambucil

A
  • Nitrogen mustards/alkylating agents
  • Marrow suppressive
29
Q

Ibrutinib

A
  • Bruton tyrosine kinase inhibitor
  • Oraol
  • Extremely variable toxicity
  • GI upset (diarrhea)
  • bleeding
  • a-fib
  • immunosuppression
  • joint pain
  • nothing
30
Q

Idelalisib

A

PI3 Kinase Inhibitor

Significant toxicity - infections (PJP pneumonia, CMV infection), liver toxicity

31
Q

Hairy Cell Leukemia morphology

A

shaggy cytoplasm “hairy cells”

32
Q

Hairy Cell Leukemia Immunophenotype

A
  • Positive for TRAP (Tartrate-resistant acid phosphatase)
  • Negative for CD5
33
Q

BM of Hairy Cell Leukemia

A

difficult to get BM aspirate because cells are so tightly packed but can get a core biopsy

34
Q

Treatment of Hairy Cell Leukemia

2 listed

A

Responds very well to purine analogs

  • Cladribine
  • Pentostatin
35
Q

M Protein AKA

A

monoclonal paraprotein

36
Q

M protein interpretation

A

aka m spike

can be IgM, IgG, IgA or whichever

37
Q

Multiple Myeloma pathophysiology

4 listed

A
  • neoplasm of clonal bone marrow plasma cells
  • typically IgG or IgA
  • Most cases have CRAB
  • Hypercalcemia, Renal insufficiency, Anemia, Bone lytic lesions
  • Produce large amounts of M-protein
38
Q

Multiple Myeloma histological features

A
  • very eccentric nucleus
  • prominent “hof” perinuclear clearing (pale area by nucleus which is very prominent Golgi apparatus)
  • bone marrow taken over by plasma cells
39
Q

CRAB

A
  • Hypercalcemia
  • Renal insufficiency
  • anemia
  • bone lytic lesions

In Multpile Myeloma

40
Q

Clues to Myeloma

5 listed

A
  • Hypercalcemia - from bone resorption
  • Renal Failure - Filtering light chains is toxic to the renal tubules
  • Anemia - normal marrow is replaced by the plasma cells
  • Pathologic bone fractures - bone remodeling leavs out bony lesions
  • Rouleaux formation -Stacks of RBCs die to increased immunoglobulin
41
Q

Monoclonal gammopathy of undetermined significance

A

clonal expansion of plasma cells but …

lower amount of M-protein

only few clonal plasma cells in bone marrow

NO CRAB

42
Q

MGUS AKA

A

Monoclonal gammopathy of undetermined significance

43
Q

MGUS Treatment

A

do not treat at this stage

44
Q

MGUS evolution

A

this condition progresses to overt myeloma at the rate of 1-2% per year

45
Q

Lymphoplasmacytic Lymphoma

A

Monoclonal plasma cells and monoclonal lymphocytes mixed together

46
Q

What type of immunoglobulin in lymphoplasmacytic lymphoma?

A

IgM

47
Q

Hyperviscosity of the blood along with IgM M protein

A

Waldenstrom Macroglobulinemia

48
Q

M protein think about these diagnoses

3 listed

A
  • Multiple Myeloma
  • MGUS
  • Lymphoplasmacytic lymphoma
49
Q

LPL AKA

A

Lymphoplasmacytic lymphoma

50
Q

A characteristic expression of LPL

A
  • Hyperviscosity of the blood along with IgM M-protein
  • Waldenstrom Macroglobulinemia
51
Q

Treatment of Multiple Myeloma

A
52
Q

Treatment of Multiple Myeloma is only indicated if?

A
  • CRAB is present
  • don’t need to have all of them but can have some of them
  • not curable so only treat if it can benefit the patient
53
Q

Treatment of Multiple Myeloma drug classes

4 listed

A
  • Immunomodulators (i.e. Thalidomide)
  • Proteasome inhibitors (Bortezomib)
  • Corticosteroids
  • Chemotherapy
54
Q

Thalidomide Toxicities

A

Marrow suppression

peripheral neuropathy

highly regulated

teratogen (highly regulated)

55
Q

Bortezomib Toxicities

A

Toxic Neuropathy

56
Q

Chemotherapic agents in Multiple Myeloma

3 listed

A
  • Cyclophosphamide
  • Liposomal doxorubicin
  • Melphalan
57
Q

Autologous Stem Cell Transplant in Multiple Myeloma

A

can collect cells before over-exposure to marrow toxic drugs

58
Q

Bone lesions in multiple myeloma

A
  • Bisphosphanate therapy - same drugs used to treat osteoporosis
  • bone lytic lesions can be on skull which is very dangerous
59
Q

Clinical Overview

A