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Winter 2016 > B&L Unit 3 > Flashcards

B&L Unit 3 Flashcards

1
Q

What is a primary immunodeficiency?

A

Mutations in genes required for normal development of parts of the immune system

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2
Q

What is secondary immunodeficiency?

A

It has an underlying cause

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3
Q

What is the defect in SCID-X1? (3)

A

Defect in gene for gamma chain that forms receptors for IL-2 and other cytokines necessary for lymphoid development or their signalling pathways

Can also result from adenosine deaminase deficiency, MHC class II deficiency

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4
Q

What happens in X-linked (Bruton) agammaglobulinemia?

A

Protein tyrosine kinase is defective -> no mature B cells

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5
Q

What types of infections to people with Bruton agammaglobulinema have?

A

Bacterial infections -> pneumonia, chronic diarrhea

Enteroviruses

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6
Q

What happens in X-linked hyper IgM syndrome?

A

Defect in IgM to IgG switch

CD40 (B cells) or CD40 ligand (T cells)

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7
Q

What happens in common variable immunodeficiency?

A

B cells are difficult to activate

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8
Q

What happens in DiGeorge syndrome?

A

Absent 3rd and 4th pharyngeal pouches -> no thymus (or parathyroid)

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9
Q

What type of pathogens are T cell deficiencies associated with? B cell deficiencies?

A

Intracellular pathogens, especially candida and pneumocystis jirovecii

Extracellular, pyogenic (high-grade) bacteria like staph, haemophilus, strep

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10
Q

What comorbidity is common in selective IgA deficiency?

A

Celiac disease

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11
Q

What happens in ataxia telegiectasia?

A

T and B cell deficiency (not absolute)
IgA depression
Sinus infections, pneumonia, ataxia, telangiectasia (dilated abnormal blood vessels), tumors

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12
Q

What happens in Wiskott-Aldrich syndrome?

A

Platelet and B cell deficiency
Eczema
Bacterial infections
X-linked

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13
Q

What is a lymphoma called that presents at a site that is not a lymph node?

A

Extranodal

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14
Q

What is the most common chromosomal abnormality in hematologic malignancies?

A

Balanced translocations

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15
Q

What are 3 viruses that can cause hematologic malignancies?

A

Epstein-Barr -> various B cell lymphomas
Human T cell leukemia virus-1 (HTLV-1)  adult T cell leukemia/lymphoma
Kaposi sarcoma herpesvirus/Human herpesvirus 8  primary effusion lymphoma

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16
Q

What 5 parameters does the WHO use to classify heme malignancies?

A

Microscopic appearance
Histologic growth patterns
Presence or absence of specific cytogenic/molecular findings
Relative amounts of cells in marrow and blood
Presence or absence of cell surface, cytoplasmic, and/or nuclear markers

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17
Q

What is myelodysplastic syndrome?

A

The marrow is overtaken by a neoplastic blood cell clone incapable of making normal blood cells

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18
Q

What is a myeloproliferative neoplasm?

A

The marrow is overtaken by a neoplastic blood cell clone that makes normal functioning myeloid cells

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19
Q

What is a non-hodgkin lymphoma?

A

Lymphomas of mature-appearing lymphocytes (that aren’t Hodgkin lymphoma or plasma cells)

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20
Q

CD________ marks B cells

A

20

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21
Q

CD________ marks T cells

A

3

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22
Q

What lymphocyte levels are found in CLL/SLL? (chronic lymphocytic leukemia/small lymphocytic lymphoma)

A

> =5 E9/L

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23
Q

What is the most common genetic alteration in follicular lymphoma?

A

Translocations of chromosomes 4 and 18, which places the BCL2 gene on chromosome 18 under influence of the IGH promoter on chromosome 14

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24
Q

What is mantle cell lymphoma?

A

A B-cell neoplasm. Characterized by BCL1 gene rearrangement  overexpression of cyclin D1

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25
Q

3 mechanisms of tissue damage in autoimmunity?

A

Neutrilization
Complement-mediated damage
Stimulatory hypersensitivity (autoantibodies can act as agonists)

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26
Q

Myasthenia gravis involves autoantibodies against __________

A

Acetylcholine receptor

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27
Q

Goodpasture syndrome involves autoantibodies against ____________

A

Lung and kidney basement membranes

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28
Q

Dressler syndrome involves autoantibodies against ____________

A

Heart

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29
Q

What is hybrid antigen formation n autoimmune disease?

A

B cell binds self + a foreign epitope
Foreign epitopes is presented to Th2 on class II MHC
B cell is activated and secretes antibody to self

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30
Q

Mnemonic for stuff that happens in DiGeorge syndrome

A

CATCH-22

Calcium – lack of parathyroids -> can’t regulate calcium. Presents first usually as neonatal convulsions

Appearance – wide set eyes, low-set ears, fish mouth

Thymus - missing

Clefts (palate)

Heart – many big defects in development of big veins

Chromosome 22 defect as a de novo mutation

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31
Q

What is IVIg used for besides immunodeficiency?

A

Anti-inflammatory

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32
Q

Why do we give irradiated RBCs to people with SCID?

A

RBCs have high adenosine deaminase

Irradiation kills lymphocytes

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33
Q

What is the innocent bystander process of loss of self-tolerance?

A

An antigen gets attached to normal tissue and then gets fucked up by the immune system

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34
Q

What is an indirect vs. a direct immunofluorescence test?

A

Direct - look for antibody attached to its target

Indirect - look for antibody in serum

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35
Q

What cell type is in the mantle zone of the lymph node?

A

B cells

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36
Q

What cell type is predominantly in the light zone of a germinal center? Dark zone?

A

Centrocytes
Centroblasts

These are both B cells

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37
Q

Cancer of the immature B cell

A

B-cell acute lymphoblastic leukemia/lymphoblastic lymphoma

B-ALL/LBL

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38
Q

Cancer of the mantle cell

A

Mantle cell lymphoma

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39
Q

3 cancers of the germinal center B cell

A

Follicular lymphoma
Burkitt lymphoma
Hodgkin lymphoma

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40
Q

Pre or post-germinal center B cell cancer

A

Chronic lymphocytic leukemia/small lymphocytic lymphoma

CLL/SLL

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41
Q

Plasma cell cancer

A

Plasma cell myeloma (multiple myeloma)

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42
Q

5 risk factors for acute leukemia

A 
Previous chemotherapy
Tobacco smoke
Ionizing radiation
Benzene exposure
Genetic stuff like Down syndrome, Bloom syndrome, Fanconi anemia, ataxia-telangiectasia
43
Q

What age group does acute lymphoblastic leukemia occur in?

A

Usually in children under 6 yo

44
Q

What 2 things do we look for to identify lymphoblasts?

A 
CD34
TdT expression (not expressed by myeloblasts or mature lymphocytes)
45
Q

What 3 things do B-lymphoblasts express? What do they not express?

A

CD19, 22, and/or 79a

CD20

46
Q

Which subgroup of B-acute lymphoblastic leukemia has the worst prognosis?

A

Ph+ ALL

The Philadelphia chromosome: t(9;22) -> BCR-ABL fusion protein

47
Q

Which subgroup of B-acute lymphoblastic leukemia has the best prognosis?

A

t(12;21)(p13;q22) ETV6-RUNX1

48
Q

Does T-ALL often come with a component of lymphoblastic lymphoma?

A

Yes. It often manifests as a large mediastinal mass.

49
Q

What CDs do T-lymphoblasts express?

A

CD2-8
CD99
CD1a

50
Q

How does number of chromosomes affect ALL prognosis?

A

Hiperdiploidy - good

Hypodiploidy - bad

51
Q

What distinguishes a myeloblast morphologically?

A

Auer rods

52
Q

What CDs indicate monocytic differentiation of myeloblasts?

A

CD64

CD14

53
Q

What CDs indicate megakaryocytic differentiation of myeloblasts?

A

CD41

CD61

54
Q

What does RUNX1 do?

A

Encodes the alpha unit of core binding factor

This is a transcription factor needed for differentiation

55
Q

What does CBFB do?

A

Encodes the beta unit of core binding factor

This is a transcription factor needed for differentiation

56
Q

What is the translocation for acute promyelocytic leukemia?

A

t(15;17)

57
Q

What leukemia can cause DIC?

A

Acute promyelocytic leukemia

58
Q

How can you treat a subset of acute promyelocytic leukemia?

A

Retinoic acid receptor-alpha becomes a fusion gene that works less well
Can give high doses of retinoic acid

59
Q

What are 3 causes of neutropenia?

A

Kostmann syndrome
Schwann-Diamond syndrome
Cyclic neutropenia

60
Q

What is sjogren syndrome?

A

An autoimmune reaction against exocrine glands, especially those that secrete tears and saliva

61
Q

What is a visible sign of vasculitis?

A

Palpable purpura

62
Q

What 2 things characterize myelodysplastic syndrome?

A

Ineffective hematopoiesis

Increased risk of transformation to acute myeloid leukemia

63
Q

What is the difference between low and high grade myelodysplastic syndrome classification?

A

Low - myeloblasts are

64
Q

What is the main genetic change in chronic myeloid leukemia (CML)?

A 
Fusion of  BCR-ABL1
From t(9;22)
This is the philadelphia chromosome
65
Q

What is the drug we use for CML (chronic myeloid leukemia)

A

imatinib (Gleevec)

It is a protein tyrosine kinase inhibitor

66
Q

What is the main genetic change in polycythemia vera?

A

Mutation of JAK2

67
Q

What is the ultimate result of polycythemia vera and primary myelofibrosis?

A

Fibrosis of the bone marrow so more blood cells cannot be produced

68
Q

What are the 2 leukemias with BCR-ABL fusions?

A

CML

B-ALL

69
Q

Which lymphoma makes a starry sky histological pattern?

A

Burkitt lymphoma

70
Q

What is the immunophenotype of Burkitt lymphoma?
5 positive
2 negative

A

B Cell markers: CD19, CD20
Germinal center B cell markers: CD10, BCL6
Myc

Negative: CD5, CD23

71
Q

What is the translocation for Burkitt lymphoma?

A

t(8;14)

Fuses the MYC gene, overexpression MYC, which is a transcription factor

72
Q

What is the most common type of non-Hodgkin’s lymphoma?

A

Diffuse large B-cell lymphoma

73
Q

Which hematologic malignancy results in lytic bone lesions?

A

Plasma cell myeloma (multiple myeloma)

74
Q

Where do tumor cells usually hang out in plasma cell myeloma?

A

In the bone marrow

75
Q

What is the most common monoclonal gammopathy (too many immunoglobulins)

A

MGUS - monoclonal gammopathy of undertermied significance

76
Q

What is a solitary plasmacytoma of bone?

A

An individual tumor of plasma cell myeloma. There is no evidence of other bone lesions.
So I think it’s when you have plasma cell myeloma and only have 1 bone tumor

77
Q

What is the immunophenotype of classic hodgkin’s lymphoma? 2 positive, 1 neative

A

CD30, CD15

No CD45, a common leukocyte antigen

78
Q

What are the 4 hodgkin’s lymphoma subtypes?

A

Nodular sclerosis
Mixed cellularity variant
Lymphocyte rich variant
Lymphocyte depleted variant

In order of frequency!

79
Q

What complement coponents attract neutrophils?

A

C3a

C5a

80
Q

Which antibody is helpful for helminth defense? Where are these antibodies?

A

IgE, which binds to FcER1 receptors on the surface of mast cells

81
Q

What things attract eosinophils?

A

Prostaglanding

Leukotrienes

82
Q

What do eosinophils release that kills helminths?

A

Major basic protein

83
Q

What signals the mast cell to degranulate?

A

IgEs on bound to the FcER1 receptors that are cross-linked by antigen

84
Q

Why don’t oral antigens affect the rest of the gut?

A

Because they are destroyed by stomach acid. They can cross the mucus membranes though and encounter mast cells in the oral cavity

85
Q

What are the two components of asthma?

A

Bronchoconstriction inflammation

86
Q

When an activated Th2 cell goes out into the body and locates a helmint, what 2 cells does it attract?

A

Eosinophils

M2 macrophages

87
Q

What is atopy?

A

A tendency to be “hyperallergic”. So they are more likely to have eczema, allergic rhinitis, or asthma.
It gets worse

88
Q

What are 2 common myeloid markers that are usually not on lymphoblasts?

A

CD117

Myeloperoxidase

89
Q

What is t-AML?

A

AML arising secondary to DNA damage from a prior therapy

Bad prognosis

90
Q

Myelodysplastic syndrome is a precursor to _____

A

AML

91
Q

What is dyshematopoiesis?

A

> 10% of cells in a lineage are dysplastic?

92
Q

IL-4 is chemotactic for _______ and _______

A

Eosinophils

M2 macrophages

93
Q

What does IL-5 do to eosinophils?

A

Upregulates production and release of eosinophils from bone marrow

94
Q

What 3 things are released in the late phase of an allergic reaction?

A

Prostaglandins
Leukotrienes
ECF-A

95
Q

What are the 3 phases of CML?

A

Chronic
Accellerated
BLast

96
Q

What is the translocation for Burkitt lymphoma?

A

MYC

t(8;14)

97
Q

What condition are auer rods in?

A

AML

98
Q

What are 4 myeloproliferative neoplasms?

A

CHronic myelogenous leukemia
Polycythemia vera
Primary myelofibrosis
Essential thrombocythemia

99
Q

What are the 2 stages of polycythemia vera?

A

Polycythemic phase

Spent phase / post-PV myelofibrosis

100
Q

What kills you in polycythemia vera?

A

Thrombosis

101
Q

Why does primary myelofibrosis cause hepatosplenomegaly?

A

Extramedullary hematopoiesis

102
Q

How does primary myelofibrosis differ from polycythemia vera?

A

No increased erythrocytes, though megakaryocytes and granulocytes are increased

103
Q

What mutation is common in essential thrombocythemia?

A

Jak2

Winter 2016 (8 decks)

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