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Hematology > Lymphoproliferative Disorders (Exam III) > Flashcards

Lymphoproliferative Disorders (Exam III) Flashcards

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
Q

BM infiltration

A

Non diffuse
- nodular pattern
- interstitial pattern

diffuse pattern

26
Q

Nodular pattern

A

distinct randomly distributed aggregates of small lymphocytes

focal nodular

27
Q

Interstital pattern

A

Lymphocytes infiltrate the interstitum without displacing fat cells

28
Q

Diffuse pattern

A

entire BM space between bone trabeculae is replaced by small lymphocytes

29
Q

Immunophenotype CLL

A

Positive: CD19, CD20, CD21, CD23

Negative: CD10

Positive: CD5 (usually T cell marker)

30
Q

PB lymphocytosis (diagnosis)

A

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
Q

BM Lymphocytosis (diagnosis)

A
  • 30% lymphocytic cells appearing morphologically mature
  • previously required, still often performed
32
Q

Prognosis/Treatment CLL

A

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
Q

CD23 indicates

A

mature state

34
Q

common treatments CLL

A
  • Fludarabine
  • Radiation
35
Q

Fludarabine

A

chemotherapy that induces apoptosis

36
Q

radiation

A

enlarged spleen and lymph node masses

37
Q

Morphology ALL vs CLL

A

ALL - smoother nuclear chromatin pattern pf lymphoblasts

CLL - heavy condensation of nuclear chromatin

38
Q

Cytochemistry ALL vs CLL

A

ALL - Lymphoblasts are TdT+

CLL - CLL lymphocytes are TdT-

39
Q

Hairy cell leukemia (HCL)

A
  • 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
Q

Cytochemistry HCL

A

strong acid phosphatase and TRAP reaction

41
Q

Cell markers HCL

A

CD19, CD20, CD22, CD11c, CD25, CD103

42
Q

TRAP

A

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
Q

TRAP is used to differentiate

A

Hairy cell luekemia (+) from other acid phosphatase (+) staining

44
Q

Sezary Syndrome

A

Leukemia phase of T cell lymphoma

Cutaneous manifestation

Abnormal circulation lymphocytes (Sezary Cells)
1. small lymph nuclear folding and groovin (cerebriform)
- whorl like

  1. looks monocyte-like but
    - NSE dot pattern
    - CD2,CD3, CD4, CD5
45
Q

cutaneous manifestation

A

mycosis fungoides

46
Q

lymphoma definition

A

heterogenous group of diseases that arise from cells of the lymphoid tissue

47
Q

lymphomas 2 categories

A
  • hodgkin disease (hodgkin’s lymphoma)
  • Lymphocytic lymphoma (aka non-hodgkins lymphoma)
48
Q

symptoms of lymphoma

A
  • enlarged lymph nodes, usually painless
  • weight loss
  • night sweats
  • chills
  • fatigue
  • frequent infections
49
Q

Hodgkin’s lymphoma

A

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
Q

Hodgkin’s lymphoma - Etiology

A

Viral Cofactor
- Epstein Barr Virus on exam
- Cytomegalovirus
- Herpesvirus 6

51
Q

Reed Sternberg cell (Hodgkins)

A

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
Q

L & H, or popcorn cell

(RS cell variants)

A
  • variable amount og pale staining cytoplasm
  • convoluted nucleaus - popcorn
  • indistinct nucleolus
  • Found in Nodular Lymphocytic Predominant Hodgkin’s Lymphoma (NLPHL)
53
Q

Lacunar Cell
(RS cell variant)

A

RS cell where cytoplasm retracts during formalin fixation step

Empty space surroinds it

54
Q

Hodgkins lymphoma Epidemiology

A
  • 15% of lymphoma cases

Bimodial Distribution
- 15-35%
- over 50 years old

55
Q

Lymphocytic lymphomas

(Non Hodgkins lymphoma)

A

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

Hematology (36 decks)

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