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MHD VI: Pulm > 11.17: Hemo II > Flashcards

11.17: Hemo II Flashcards

1
Q

What do chronic hematopoietic neoplasms involve?

A

Mature cells

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2
Q

3 categories of chronic lymphoid disorders?

A
  1. Chronic leukemias
  2. Lymphomas
  3. Plasma cell disorders
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3
Q

What are some of the chronic leukemias?

A
  1. CLL: Chronic lymphocytic leukemia
  2. SLL: Small lymphocytic lymphoma
  3. Hairy cell leukemia
  4. Adult T cell leukemia / Lymphoma
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4
Q

How are CLL and SLL different?

A

They are the same disease

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5
Q

What is most common cause of leukemia in adults?

A

CLL/SLL

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6
Q

What cell is usually involved in CLL/SLL?

A
  • B cell neoplasm with the following markers:
    1. CD20
    2. CD5
    3. CD 23
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7
Q

Clinical features of CLL/SLL?

A
  1. Seen in older ptns. with immune issue and hypogammaglobulinemia
    - Tons of B cells but they do not function
  2. Some have autoimmune hemolytic anemia
  3. Richter transformation
  4. Mutated IgH gene shows better prognosis
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8
Q

What can CLL/SLL turn into?

A

“Richter syndrome/transformation”

  1. Prolymphocytic leukemia
  2. Diffuse large cell lymphoma
    * *Prognosis is poor when this occurs
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9
Q

Cell appearance in CLL/SLL?

A
  • Small round lymphocytes w/ condensed chromatin and scant cytoplasm
    “Smudge cells” in peripheral smear
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10
Q

When are smudge cells seen?

A

CLL/SLL

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11
Q

Characteristics of hairy cell leukemia?

A

“B Cell neoplasm”

  1. Hair like cytoplasmic projections in cells
  2. Usually in middle aged men with splenomegaly
  3. Pancytopenia
  4. Bone marrow involvement
  5. Responds well to gentle chemotherapry
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12
Q

What is tartrate resistant acid phosphatase?

A

“TRAP”

- Seen in chemical staining of Hairy cell leukemia

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13
Q

Where is adult T cell leukemia / lymphoma often seen?

A
  1. Southern Japan
  2. West Africa
  3. Caribbean
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14
Q

Presentation of Adult T cell?

A
  1. Skin lesions
  2. Hepatosplenomegaly
  3. Lymphocytosis
  4. HYPERcalcemia
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15
Q

What is thought to cause adult T cell?

A
  • HTLV 1 retrovirus
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16
Q

What are floret like lymphocytes characteristic of?

A

Adult T cell leukemia / lymphoma

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17
Q

How do diagnose Adult T cell leukemia / lymphoma?

A
  • Floret like CD4 lymphocytes in peripheral blood
18
Q

Main categories of chronic lymphomas?

A
  1. Non Hodgkin: B and T cell

2. Hodgkin

19
Q

What are the B cell Non hodgkin lymphomas?

A
  1. Follicular lymphoma
  2. Mantle cell lymphoma
  3. Burkitt lymphoma
  4. Diffuse large B cell
20
Q

Where is burkitt lymphoma often seen?

A

Africa

21
Q

Where is malt lymphoma often seen?

A

Mediterranean

22
Q

How are lymphomas diagnosed?

A
  1. Biopsy of lymph node is necessary
  2. B cell markers 19 and 20
  3. T cell markers CD3
  4. Myeloperoxidase
23
Q

What are the following markers indicative of?

  • B cell markers 19 and 20
  • T cell markers CD3
  • Myeloperoxidase
A

Lymphoma

24
Q

Characteristics of follicular lymphoma?

A
  1. 40% of adult NHLs
  2. Painless lymphadenopathy
  3. Not many symptoms otherwise
  4. 7 - 9 year survival
  5. Can become diffuse large cell lymphoma
25
Q

Cells seen in follicular lymphoma?

A

Centrocytes: small cells with cleaved / irregular nuclei
Centroblasts: large cells with open nuclear chromatin and several nucleoli
**B Cells expressing CD 19/20/10

26
Q

What is most common genotypic finding in follicular lymphoma?

A
  • 14/18 translocation with BCL2/IGH fusion

- Causes over amplification of BLC2 which is antiapoptotic

27
Q

Why is BLC2 overexpressed in lymphomas?

A
  • It is fused to IGH gene
  • Follicular lymphoma is B cell tumor so lots of IgH being expressed
  • This leads to over expression of BLC2 which is antiapoptotic which is not good
28
Q

What are the round structures beneath the capsule of the lymph node?

A
  • Germinal centers / secondary follicles

- Appear a bit paler and only found next to capsule

29
Q

What happens in center of Follicle?

A
  • B cells are selected for producing high affinity antibodies
30
Q

Where are B cells selected for producing high affinity antibodies?

A
  • Germinal centers

- Ineffective B cells undergo apoptosis and are eaten by Tingible body macrophages

31
Q

What happens to follicles in follicular lymphoma?

A
  • Many more follicles present and arranged back to back

- And not just next to capsule

32
Q

What is high BCLS characteristic of?

A

Follicular lymphoma

33
Q

What causes mantle cell lymphoma?

A
  • 11/14 translocation leading to CD1-IgH fusion
  • Cycle D1 is overexpressed leading to increased proliferation
  • IgH is normally overexpressed in B cells to CD1 will be as well due to translocation
34
Q

What is overexpression of Cycle D1 characteristic of?

A

Mantle cell lymphoma

35
Q

Characteristics of Burkitt lymphoma?

A

“B cell neoplasm “

  1. Aggressive in children and young adults
  2. Tumors in extranodal sites
    a. Endemic: jaw tumors in africa
    b. Non endemic: abdominal tumor
  3. Increase histiocytes surrounded by clear space: “Starry sky pattern”
36
Q

What is starry sky pattern indicative of?

A

Burkitt Lymphoma

37
Q

What can lead to Burkitt Lymphoma?

A
  1. HIV

2. EBV

38
Q

Genetic cause of Burkitt Lymphoma?

A
  • 8/14 translocation of MYC next to IgH
39
Q

Translocation seen in Burkitt lymphoma?

A
  • t(8;14) [ MYC/IGH fusion]

- Causing MYC over-amplification (increased proliferation)

40
Q

Translocation see in Follicular lymphoma?

A
  • t(14;18) [BCL2/IGH fusion]

- causing BCL2 over-amplification (anti-apoptotic)

41
Q

Translocation see in Mantle cell lymphoma?

A
  • t(11;14) [Cyclin D1/IGH fusion]
  • causing Cyclin D1 over-amplification (increased
    proliferation)

MHD VI: Pulm (20 decks)

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