Lymphoproliferative Disorders (Exam III) Flashcards

(55 cards)

1
Q

Normal adult peripheral blood

A
  • 60-80% T lymphocytes
  • up to 20% B lymphocytes (have surgace Ig)
  • Up to 22.3% NK cells
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2
Q

chronic lymphoproliferative disorders

A

clonal proliferations of morphogically and immunophenotypically mature B or T cell

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3
Q

Classification

A
  • morphology
  • immunophenotype
  • molecular genetics
  • cytogenetics
  • clinical features
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4
Q

B cell leukemia

A

Chronic lymphocytic leukemia

Hairy Cell Leukemia

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5
Q

T cell leukemia

A

Sezary syndrome

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6
Q

Lymphomas

A

Hodgkin’s Lymphoma

Non Hodgkins lymphoma

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7
Q

Plasma cell disorders

A

Multiple Myeloma
- Leukemia Variant Plasma Cell leukemia

Waldenstroms Macroglobulinemia

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8
Q

CLL (B cell) show

A

failed apoptosis - high levels of antipoptotic protein BCL2
- long lived and immunologically dysfunctional lymphocytes in PB and BM
- Gradual accumulation of small mature B lymphocytes

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9
Q

CLL replace normal BM elements

A

Anemia, thrombocytopenia, neutropenia

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10
Q

Extramedullary hematopoiesis CLL

A

Splenomegaly

hypersplenism - Anemia, Thrombocytopenia, neutropenia

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11
Q

CLL impaited immunologic activity

A

Hypogammaglobulinemia
- increased infection

Autoimmunity
- Autoantibodies against RBCs, PLT, or both

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12
Q

CLL is more prevalent in

A

2x in males

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13
Q

90% of CLL cases

A

> 50%

Rare in young adults

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14
Q

Signs and symptoms CLL

A

symptoms develop gradually.

accidently discovered during doctor visit
- Unexplained lymphocytosis
- Lamphadenopathy
- Splenomegaly

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15
Q

CLL chronic fatige

A

anemia

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16
Q

infections CLL

A

neutropenia / hypogammaglobulinemia

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17
Q

easy bruising CLL

A

thrombocytopenia

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18
Q

Dermatologic manifestations

A

clinical feature of CLL

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19
Q

CLL - Early phase

A

tumor cells are predominately small in size

low proliferation rate

cells are living longer
- prolonged survival

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20
Q

CLL - Transformation phase

A

increase in immature cells

Frequent occurence of extramedullar proliferation

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21
Q

Lab findings CLL

A

Decreased Hgb and Hct
- anemia
- normocytic, normochromic RBCs

Decreased PLT count
- thrombocytopenoa

Variable WBC
- Neutropenia
- lymphocytosis

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22
Q

CLL lymphocytes

A

Hypercondensed “soccer ball” nuclear chromatin pattern

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23
Q

smudge cells CLL

A

Fragile lymphocytes “smudged” in smear process
- remedy: add albumin

do NOT count in differential

Bare nuclei are common

24
Q

CLL additional morphology

A

Increased Prolymphocytes

Activated Lymphocytes

Increased Lymphoplasmacytoid cells
- Lymphocytes that look similar to plasma cells

25
BM infiltration
Non diffuse - nodular pattern - interstitial pattern diffuse pattern
26
Nodular pattern
distinct randomly distributed aggregates of small lymphocytes *focal nodular*
27
Interstital pattern
Lymphocytes infiltrate the interstitum without displacing fat cells
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Diffuse pattern
entire BM space between bone trabeculae is replaced by small lymphocytes
29
Immunophenotype CLL
Positive: CD19, CD20, CD21, CD23 Negative: CD10 Positive: CD5 (usually T cell marker)
30
PB lymphocytosis (diagnosis)
PB lymphocytosis - absolute lymphocyte count >5x10^9/L - normal is 1.3-3.4x10^9/L) - monoclonal (identical) - sustained for 3 months or longer
31
BM Lymphocytosis (diagnosis)
- 30% lymphocytic cells appearing morphologically mature - previously required, still often performed
32
Prognosis/Treatment CLL
many live 10 years post diagnosis Nodular or Interstitial BM patterns have longer life expectancy than diffuse paterns - still able to preserve normal hematopoiesis Most patients die with resiual leukemia and usually succmb to infection or CLL unrelated cause
33
CD23 indicates
mature state
34
common treatments CLL
- Fludarabine - Radiation
35
Fludarabine
chemotherapy that induces apoptosis
36
radiation
enlarged spleen and lymph node masses
37
Morphology ALL vs CLL
ALL - smoother nuclear chromatin pattern pf lymphoblasts CLL - heavy condensation of nuclear chromatin
38
Cytochemistry ALL vs CLL
ALL - Lymphoblasts are TdT**+** CLL - CLL lymphocytes are TdT**-**
39
Hairy cell leukemia (HCL)
- Rare B lymphocyte derived chronic leukemia - Fine hairlike irregular cytoplasmic projections - BM - "fried egg" appearance where lymphocytic infiltrates have clear cytoplasm seperating one from another - increase B lymphocytes in 90% of cases
40
Cytochemistry HCL
strong acid phosphatase and TRAP reaction
41
Cell markers HCL
CD19, CD20, CD22, CD11c, CD25, CD103
42
TRAP
Acid phosphatase stain repeated with addition of tartrate - abolishes all acid phosphatase activity, except isoenzyme 5 - hairy cells contain isoenzyme 5 Positive staining = orange-red precipitate
43
TRAP is used to differentiate
Hairy cell luekemia (+) from other acid phosphatase (+) staining
44
Sezary Syndrome
Leukemia phase of T cell lymphoma Cutaneous manifestation Abnormal circulation lymphocytes (Sezary Cells) 1. small lymph nuclear folding and groovin (cerebriform) - whorl like 2. looks monocyte-like but - NSE dot pattern - CD2,CD3, CD4, CD5
45
cutaneous manifestation
mycosis fungoides
46
lymphoma definition
heterogenous group of diseases that arise from cells of the lymphoid tissue
47
lymphomas 2 categories
- hodgkin disease (hodgkin's lymphoma) - Lymphocytic lymphoma (aka non-hodgkins lymphoma)
48
symptoms of lymphoma
- enlarged lymph nodes, usually painless - weight loss - night sweats - chills - fatigue - frequent infections
49
Hodgkin's lymphoma
group of neoplasms characterized by the presence of a Reed-Sternberg cell 2 groups: - Nodular Lymphocyte Predominant Hodgkin's Lymphoma (NLPHL) - Classical Hodgkin's Lymphoma (CHL)
50
Hodgkin's lymphoma - Etiology
Viral Cofactor - **Epstein Barr Virus** on exam - Cytomegalovirus - Herpesvirus 6
51
Reed Sternberg cell (Hodgkins)
Malignant (clonal) prolferation of Reed-Sternberh cells and its variants - giant cell (up to 45 um) - abundant acidophilic cytoplasm j - Multinucleated or polyoblated nucleaus - "owl eyes"
52
**L & H, or popcorn cell** (RS cell variants)
- variable amount og pale staining cytoplasm - convoluted nucleaus - popcorn - indistinct nucleolus - Found in Nodular Lymphocytic Predominant Hodgkin's Lymphoma (NLPHL)
53
Lacunar Cell (RS cell variant)
RS cell where cytoplasm retracts during formalin fixation step Empty space surroinds it
54
Hodgkins lymphoma Epidemiology
- 15% of lymphoma cases Bimodial Distribution - 15-35% - over 50 years old
55
Lymphocytic lymphomas (**Non** Hodgkins lymphoma)
malignant (clonal) proliferation of lymphocytic **cells that are not Reed Sternberg cells** - makes up the majority of lymphoma cases - Poorer prognosis than Hodgkin Lymphoma - Several classsification schemes have been identified using 1. grown pattern (nodular vs. diffuse) 2. Cytologic features 3. Immunophenotype 4. Genotype