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Block 4 Final Flashcards

1
Q

Describe the common findings in Wiskott Aldrich syndrome (labs)

A

Low IgM
High IgA & IgE
Normal IgG
Low T cell levels

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2
Q

Low IgM
High IgA & IgE
Normal IgG
Low T cell levels

labs indicate which condition?

A

Wiskott Aldrich syndrome

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3
Q

Describe the clinical signs of Wiskott-Aldridge syndrome

A

Thrombocytopenia
Eczema
Recurrent infections
Atopic dermatitis (Extensor side)

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4
Q

ThrombocytopenIa
Eczema
Recurrent infections

What’s the condition?

A

Wiskott-Aldridge syndrome

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5
Q

What causes Wiskott-Aldridge syndrome?

A

Mutation of the WASP protein (Xp11.23)

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6
Q

Mutation of the WASP protein (Xp11.23) causes which condition?

A

Wiskott-Aldridge syndrome

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7
Q

Describe the common findings in Isolated IgA deficiency

A

1) Very low serum & secretory IgA
2) High risk of respiratory tract allergies & autoimmune disease
3) Defective BAFF (Bcell activating cytokine)

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8
Q

1) Very low serum & secretory IgA
2) High risk of respiratory tract allergies & autoimmune disease
3) Defective BAFF (Bcell activating cytokine)

Are all common findings of which condition?

A

Isolated IgA deficiency

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9
Q

Describe the clinical features of Isolated IgA Deficiency

A

Recurrent sino-pulmonary infections & diarrhea

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10
Q

Recurrent sino-pulmonary infections & diarrhea & low IgA indicate which conditions

A

Isolated IgA Deficiency

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11
Q

What is a complication of Isolated IgA Deficiency

A

Type II severe anaphylaxis when given a blood transfusion

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12
Q

Describe the common findings of DiGeorge syndrome

A

1) Thymic hypo/anaplasia (no Tcell immunity)
2) Hypocalcemia (no parathyroid)

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13
Q

1) Thymic hypo/anaplasia (no Tcell immunity)
2) Hypocalcemia (no parathyroid)

are all common findings of which condition?

A

DiGeorge

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14
Q

What is the cause of DiGeorge?

A

Deleted TBX1 region on 22q11 (causes 3rd & 4th pharyngeal pouches to not develop)

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15
Q

Deleted TBX1 region on 22q11 (causes 3rd & 4th pharyngeal pouches to not develop)

A

DiGeorge

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16
Q

Describe the clinical features of DiGeorge

A

1) Recurrent infections
2) Tetany
3) Congenital heart defects
4) Flat nasal bridge
5) Widened epicanthal fold
6) Low set/back rotated ears
7) small philtrum & chin

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17
Q

1) Recurrent infections
2) Tetany
3) Congenital heart defects
4) Flat nasal bridge
5) Widened epicanthal fold
6) Low set/back rotated ears
7) small philtrum & chin

Are features of which condition?

A

DiGeorge

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18
Q

What are the causes of X-linked Agammaglobinulemia?

A

Mutated Burton tyrosine kinase (Btk) on Xq21.22 (no Bcell development)

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19
Q

Mutated Burton tyrosine kinase (Btk) on Xq21.22 (no Bcell development)

cause which condition?

A

causes of X-linked Agammaglobinulemia

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20
Q

Describe the common findings (labs) in X-linked Agammaglobinulemia

A

1) None/Low B cell #
2) Low Igs (all of them)
3) CD19 +/ IgM - Bcells
4) Normal Tcells
5) Underdeveloped germinal centers in LN

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21
Q

1) None/Low B cell #
2) Low Igs (all of them)
3) CD19 +/ IgM - Bcells
4) Normal Tcells
5) Underdeveloped germinal centers in LN

Are all common findings in which condition?

A

X-linked Agammaglobinulemia

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22
Q

Describe the clinical features of X-linked Agammaglobinulemia

A

1) Recurrent respiratory infections
- Acute/chronic pharyngitis
- Sinusitis
- Bronchitis
- Osteitis media
- Pneumonia
2) Giardia lamblia (if IgA is low)
3) Recurrent diarrhea & GERD

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23
Q

1) Recurrent respiratory infections
- Acute/chronic pharyngitis
- Sinusitis
- Bronchitis
- Osteitis media
- Pneumonia
2) Giardia lamblia (if IgA is low)
3) Recurrent diarrhea & GERD

Are features of which condition?

A

clinical features of X-linked Agammaglobinulemia

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24
Q

Describe the etiology of X-linked Agammaglobinulemia

A

More common in males & it doesn’t become apparent until 6 months

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25
Q

What are the pathogens that pose the most serious threat to persons with X-linked Agammaglobinulemia?

A

H. influenza
S. pneumoniae
S. aureus
Enteroviruses (Echo/polio/coxsackie)

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26
Q

H. influenza
S. pneumoniae
S. aureus
Enteroviruses (Echo/polio/coxsackie)

Pose the most risk to persons with which autoimmune disorder?

A

X-linked Agammaglobinulemia

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27
Q

What is a consideration when vaccinating children with X-linked Agammaglobinulemia?

A

you can’t give them live vaccines because they don’t have B-cells to fight it!!!!

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28
Q

Describe the clinical features of SCID

A

1) Oral thrush
2) Extensive diaper rash
3) Failure to thrive
4) Morbilliform rash & GVHD (shortly after birth)
5) Recurrent infections
6) Chronic watery diarrhea (viral)

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29
Q

1) Oral thrush
2) Extensive diaper rash
3) Failure to thrive
4) Morbilliform rash & GVHD (shortly after birth)
5) Recurrent infections
6) Chronic watery diarrhea (viral)

Are all features of which condition?

A

SCID

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30
Q

Which pathogens pose the most risk to a person with SCID?

A

1) Candida albicans
2) Pneumonitis jiroveci (sand paper lung)
3) Pseudomonas
4) CMV
5) Vericella

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31
Q

1) Candida albicans
2) Pneumonitis jiroveci (sand paper lung)
3) Pseudomonas
4) CMV
5) Vericella

Are pathogens that pose the most risk to a person with which autoimmune condition?

A

SCID

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32
Q

Describe the cause of X-linked SCID (subtype)

A

Mutation in a common y-chain that messes with interleukin production especially IL-7 (lymphoid progenitors) & IL15 (NK proliferation)

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33
Q

Mutation in a common y-chain that messes with interleukin production especially IL-7 (lymphoid progenitors) & IL15 (NK proliferation)

IS THE CAUSE OF which AUTOIMMUNE disease?

A

cause of X-linked SCID (subtype

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34
Q

Describe the common findings in X-linked SCID

A

1) Normal neutrophil #
2) Black thymic shadow on X-ray
3) Low Igs
4) Low Tcell #

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35
Q

1) Normal neutrophil #
2) Black thymic shadow on X-ray
3) Low Igs
4) Low Tcell #

Are all common findings of which condition?

A

X-linked SCID

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36
Q

Describe the etiology of X-linked SCID

A

More common in young boys

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37
Q

Describe the cause of AUTO-REC SCID

A

Deficient ADA enzyme (Adenosine deaminase) causing an accumulation of deoxy-ATP which is toxic to lymphocytes
&
Mutated RAG gene (blocks B & T cell development)

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38
Q

Deficient ADA enzyme (Adenosine deaminase) causing an accumulation of deoxy-ATP which is toxic to lymphocytes

&

Mutated RAG gene (blocks B & T cell development)

The cause of which condition?

A

AUTO-REC SCID

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39
Q

Describe the etiology of Sjogren syndrome

A

Older females (50-60yrs) typically with thyroid issues

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40
Q

Describe the clinical features of Sjogren syndrome

A

1) Keratoconjunctivitis sicca (dry/burning/itchy eyes & blurred vision)
2. Xerostomia (dry mouth)
- diff swallowing solids
- tongue fissures
- Parotid gland enlargement
3. Extra glandular disease
- Synovitis
- Diffuse pulmonary fibrosis
- Peripheral neuropathy

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41
Q

1) Keratoconjunctivitis sicca (dry/burning/itchy eyes & blurred vision)
2. Xerostomia (dry mouth)
- diff swallowing solids
- tongue fissures
- Parotid gland enlargement
3. Extra glandular disease
- Synovitis
- Diffuse pulmonary fibrosis
- Peripheral neuropathy

All features of which condition?

A

Sjogren syndrome

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42
Q

Describe the immuno-markers of Sjogren syndrome?

A

1) Anti-CCLP ab **
2) IgM vs IgG
3) RA factor
4) SS-A (Ro)
5) SS-B (La)

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43
Q

In sjogren syndrome presenting with SS-A (Ro) is indicative of what prognosis?

A

poor prognosis (systemic spread of disease shows extra glandular symptoms)

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44
Q

What is the first form of Sjogren syndrome?

A

1) Sicca syndrome (primary) associated with defective:
- HLA-B8
- HLA-DR3
- DRW52
- HLA-DQA1
- HLA-DQB1

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45
Q

An autoimmune disorder associated with defective:
- HLA-B8
- HLA-DR3
- DRW52
- HLA-DQA1
- HLA-DQB1

A

Sicca syndrome (primary)

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46
Q

What is the second form of Sjogren syndrome?

A

2) legit when Sjogrens is associated with any other autoimmune disorder the most common being Rheumatoid arthritis

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47
Q

Describe the etiology of Systemic sclerosis (scleroderma)

A

Females 50-60yrs old usually

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48
Q

Describe the common findings in Systemic sclerosis (scleroderma)

A

Widespread damage of small blood vessels & progressive fibrosis in the interstitial & perivascular tissues of the skin, Gi, kidneys, heart, muscles, & lungs

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49
Q

Widespread damage of small blood vessels & progressive fibrosis in the interstitial & perivascular tissues of the skin, Gi, kidneys, heart, muscles, & lungs

Indicates which condition?

A

Systemic sclerosis (scleroderma)

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50
Q

Describe the clinical features of Limited Systemic Sclerosis

A

“CREST”
Calcification
Raynaud’s
Esophageal dysmotility (rubber hose)
Sclerodactyly (claw hands)
Telangiectasia

Typically skin symptoms are limited to the forearms, face & fingers

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51
Q

“CREST”
Calcification
Raynaud’s
Esophageal dysmotility (rubber hose)
Sclerodactyly (claw hands)
Telangiectasia

Typically skin symptoms are limited to the forearms, face & fingers

Are features of which condition?

A

Limited Systemic Sclerosis

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52
Q

Describe the cause of Limited Systemic Scleroderma

A

The body makes anti-SCL-70 (Targets DNA topoisomerase)

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53
Q

The body makes anti-SCL-70 (Targets DNA topoisomerase)

the cause of which condition?

A

Limited Systemic Sclerosis

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54
Q

In a persons with systemic scleroderma anti-centromere production indicates which prognosis?

A

It indicates CREST (aka limited SS) a good prognosis

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55
Q

In a persons with systemic scleroderma anti-RNA pol III production indicates which prognosis?

A

It indicates diffuse SS which has a poorer prognosis

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56
Q

Describe the common findings in Diffuse scleroderma

A

Widespread diffuse atrophy of the skin that spreads to the viscera of organs

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57
Q

Widespread diffuse atrophy of the skin that spreads to the viscera of organs

Indicated which condition?

A

Diffuse scleroderma

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58
Q

Describe the histological findings typical of Diffuse Scleroderma?

A

1) edema
2) Perivascular infiltration
3) Thickened basal lamina

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59
Q

1) edema
2) Perivascular infiltration
3) Thickened basal lamina

Are common histological findings in which condition?

A

Diffuse Scleroderma

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60
Q

Describe the clinical features of Diffuse scleroderma

A

1) Diffuse sclerotic atrophy of the skin
2) autoamputation (fingers)
3) Progressive atrophy and fibrosis of Gi & Esophagus (Esophageal dysmotility & Barrets)
4) Fibrosis of intralobular kidney arteries
5) Pulmonary hypertension/vasospasms
6) Interstitial fibrosis
7) Pericarditis with effusion
8) Myocardial fibrosis

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61
Q

1) Diffuse sclerotic atrophy of the skin
2) autoamputation (fingers)
3) Progressive atrophy and fibrosis of Gi & Esophagus (Esophageal dysmotility & Barrets)
4) Fibrosis of intralobular kidney arteries
5) Pulmonary hypertension/vasospasms
6) Interstitial fibrosis
7) Pericarditis with effusion
8) Myocardial fibrosis

Are all features of which condition?

A

Diffuse scleroderma

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62
Q

Describe the etiology of Acute myeloid leukemia (AML)

A

Happens at all ages but peaks at 60yrs

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63
Q

Describe the causes of Acute myeloid leukemia (AML)

A

AML gene disruptions due to:
1) t(8;21) translocation(disrupts RUNX1)
2) INV(16) (disrupts CBFB)
3) t(15;17) translocation(acute promyelocytic leukemia)
4) Benzene (leather industry)

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64
Q

AML gene disruptions due to:
1) t(8;21) translocation(disrupts RUNX1)
2) INV(16) (disrupts CBFB)
3) t(15;17) translocation(acute promyelocytic leukemia)
4) Benzene (leather industry)

Are all contributing factors to developing which condition?

A

Acute myeloid leukemia (AML)

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65
Q

Describe the common findings of Acute myeloid leukemia

A

1) There (20%) myeloblasts in the bone marrow
2) Neoplastic cells have delicate chromatin & azurophilic granules

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66
Q

1) There (20%) myeloblasts in the bone marrow
2) Neoplastic cells have delicate chromatin & azurophilic granules

What’s the condition?

A

Acute myeloid leukemia

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67
Q

1) Auer rods
2) AML t(15;17) translocation

What’s the condition?

A

Acute Promyelocytic leukemia

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68
Q

Describe the features of Acute myeloid leukemia

A

1) Recurrent infections’
2) Anemia
3) Thrombocytopenia (increased PT & PTT)
4) Neutropenia

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69
Q

1) Recurrent infections’
2) Anemia
3) Thrombocytopenia (increased PT & PTT)
4) Neutropenia

Are all features of which condition?

A

Acute myeloid leukemia

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70
Q

1) Cell differentiation
2) Fibrosis (increased Fibrogenic cytokines) (Downs syndrome)
3) Pseudopod formation***

What’s the condition

A

AML Megakaryoblastic type

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71
Q

1) Myeloperoxidase +
2) CD33/34+

What’s the condition

A

Acute Myeloblastic leukemia

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72
Q

1) Non-specific esterase +
2) Blueberry rash

What’s the condition

A

Monoblastic

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73
Q

1) Auer rods
2) AML t (15;17)

What’s the condition?

A

Acute promyelocytic leukemia

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74
Q

Describe the etiology of Lymphocytic predominance non-classical Hodgkin Lymphoma

A

Rare, Usually in males under 35yrs

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75
Q

Describe the common findings of Lymphocytic predominance non-classical Hodgkin Lymphoma

A

A mix of lymphocytes & histocyte variants (aka Popcorn cells)

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76
Q

Describe the immuno-markers for Lymphocytic predominance non-classical Hodgkin Lymphoma

A

1) CD20+ & BCL-6 +
2) CD15 - & CD30 -
3) EBV -

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77
Q

1) CD20+ & BCL-6 +
2) CD15 - & CD30 -
3) EBV -

Indicate which condition?

A

Lymphocytic predominance non-classical Hodgkin Lymphoma

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78
Q

Describe the etiology of Lymphocyte depletion-type Hodgkin Lymphoma

A

The rarest, usually older men who are HIV + & from 3rd world countries

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79
Q

Describe the common findings of Lymphocyte depletion-type Hodgkin Lymphoma

A

1) Low lymphocyte #
2) High Reed-Sternberg cell #
3) High association with EBV
4) Poorest prognosis

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80
Q

1) Low lymphocyte #
2) High Reed-Sternberg cell #
3) High association with EBV
4) Poorest prognosis

Are indicating of which condition?

A

Lymphocyte depletion-type Hodgkin Lymphoma

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81
Q

Describe the immuno-markers of Lymphocyte depletion-type Hodgkin Lymphoma

A

1) CD15+ & CD30+
2) EBV +

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82
Q

1) CD15+ & CD30+
2) EBV +

Are indicative of which condition?

A

Lymphocyte depletion-type Hodgkin Lymphoma

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83
Q

Describe the etiology of Lymphocyte-rich Hodgkin Lymphoma

A

Older men

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84
Q

Describe the common findings of Lymphocyte-rich Hodgkin Lymphoma

A

1) Reed-Sternberg cells
2) Mononuclear variants (halo cells)
3) More reactive lymphocytes

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85
Q

1) Reed-Sternberg cells
2) Mononuclear variants (halo cells)
3) More reactive lymphocytes

Are common findings of which condition?

A

Lymphocyte-rich Hodgkin Lymphoma

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86
Q

Describe the immuno-markers of Lymphocyte-rich Hodgkin Lymphoma

A

1) CD15+ & CD30+
2) EBV -

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87
Q

1) CD15+ & CD30+
2) EBV -

Are indicative of which condition?

A

Lymphocyte-rich Hodgkin Lymphoma

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88
Q

Describe the etiology of Mixed cellular type Hodgkin Lymphoma

A

Usually males, it has to peaks of incidence
- Young adulthood
- Over 55yrs old

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89
Q

Describe the common findings of Mixed cellular type Hodgkin Lymphoma

A

A heterogenous mix of cells:
- T cells
- Eosinophils
- Benign macrophages
- Reed-Sternberg cells
- Lacunar cells
- Mononuclear lacunar cells

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90
Q

A heterogenous mix of cells:
- T cells
- Eosinophils
- Benign macrophages
- Reed-Sternberg cells
- Lacunar cells
- Mononuclear lacunar cells

Are common findings of which condition?

A

Mixed cellular type Hodgkin Lymphoma

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91
Q

Describe the immunomarkers of Mixed cellular type Hodgkin Lymphoma

A

1) CD15+ & CD30+
2) EBV+
3) Heterogenous mix of cells

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92
Q

1) CD15+ & CD30+
2) EBV+
3) Heterogenous mix of cells

Indicate which condition?

A

Mixed cellular type Hodgkin Lymphoma

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93
Q

Describe the clinical features of Mixed cellular type Hodgkin Lymphoma

A

1) Painless lymphadenopathy
2) Fever
3) Night sweats
4) Weight loss

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94
Q

1) Painless lymphadenopathy
2) Fever
3) Night sweats
4) Weight loss

In a condition with heterogenous mix of cells indicates….

A

Mixed cellular type Hodgkin Lymphoma

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95
Q

Describe the etiology of Nodular sclerotic type Hodgkin Lymphoma

A

Usually young women

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96
Q

Describe the common findings of Nodular sclerotic type Hodgkin Lymphoma

A

1) Lacunar variant Reed-Sternberg cells
2) Fibrous bands & nodular architecture
3) Good prognosis usually

97
Q

1) Lacunar variant Reed-Sternberg cells
2) Fibrous bands & nodular architecture
3) Good prognosis usually

Are common findings of which condition?

A

Nodular sclerotic type Hodgkin Lymphoma

98
Q

Describe the immuno-markers of Nodular sclerotic type Hodgkin Lymphoma

A

1) CD15+ & CD30+
2) EBV -
3) Common in young women

99
Q

1) CD15+ & CD30+
2) EBV -
3) Common in young women

Indicate which condition?

A

Nodular sclerotic type Hodgkin Lymphoma

100
Q

Describe the clinical features of Nodular sclerotic type Hodgkin Lymphoma

A

Mediastinal mass & supraclavicular involvement

101
Q

Describe the etiology of Follicular (Bcell) Lymphoma

A

Usually middle aged men/women

102
Q

Describe the common findings of Follicular (Bcell) Lymphoma

A

1) t(14;18) translocation on IgH (heavy) & Bcl-2
2) Overexpression of Bcl-2
3) No mantle zone
4) No interfollicular space
5) Centrocytes (small/cleaved cells)
6) Centro blasts (large cells)

103
Q

1) t(14;18) translocation on IgH (heavy) & Bcl-2
2) Overexpression of Bcl-
3) No mantle zone
4) No interfollicular space
5) Centrocytes (small/cleaved cells)
6) Centro blasts (large cells)

Are all common findings of which condition?

A

Follicular (Bcell) Lymphoma

104
Q

3) No mantle zone
4) No interfollicular space
5) Centrocytes (small/cleaved cells)
6) Centro blasts (large cells)

Indicate which condition?

A

Follicular (Bcell) Lymphoma

105
Q

Describe the immuno-markers of Follicular (Bcell) Lymphoma

A

1) CD5- & CD23-
2) Bcl-2-
3) CD19+, CD20+, & CD10+

106
Q

1) CD5- & CD23-
2) Bcl-2-
3) CD19+, CD20+, & CD10+

Indicate which condition?

A

Follicular (Bcell) Lymphoma

107
Q

Describe the clinical feature of Follicular (Bcell) Lymphoma

A

Painless “waxing & wanning” lymphadenopathy

108
Q

Describe the etiology of Burkitt’s lymphoma

A

usually children & young adults

109
Q

Describe the causes of Burkitt’s lymphoma

A

All three types are associated with heavy Ig t(8;14) translocations & MYC-C translocations which increase the overall expression of myc proteins

110
Q

All three types are associated with heavy Ig t(8;14) translocations & MYC-C translocations which increase the overall expression of myc proteins

The cause for which condition?

A

Burkitt’s lymphoma

111
Q

Describe the histological findings in Burkitt’s lymphoma

A

Medium sized lymphoma with diffuse infiltrating cells that have round/oval nuclei, coarse chromatin, & several nucleoli

112
Q

What’s the condition?

A

Burkitt’s lymphoma

113
Q

Describe the common findings of Burkitt’s lymphoma

A

1) medium sized lymphoma with diffuse infiltrates
2) Tumor has a high mitotic index
3) Tumor has a “starry-sky” appearance (macrophages)

114
Q

1) medium sized lymphoma with diffuse infiltrates
2) Tumor has a high mitotic index
3) Tumor has a “starry-sky” appearance (macrophages)

Are all common findings of which condition?

A

Burkitt’s lymphoma

115
Q

Describe the immuno-markers of Burkitt’s lymphoma

A

1) IgM
2) CD19+ & CD20+
3) CD10+
4) Bcl-6 +
5) Usually Bcl-2 -

116
Q

1) IgM
2) CD19+ & CD20+
3) CD10+
4) Bcl-6 +
5) Usually Bcl-2 -

Indicate which condition?

A

Burkitt’s lymphoma

117
Q

Describe the African/Endemic subtype Burkitt’s lymphoma

A

1) Has a STRONG ass with EBV (+)
2) Presents as a jaw mass
3) Involves abdominal viscera (kidneys, ovaries, & adrenals)
4) Involves t(8;14) of heavy Ig & myc-c

118
Q

1) Has a STRONG ass with EBV (+)
2) Presents as a jaw mass
3) Involves abdominal viscera (kidneys, ovaries, & adrenals)
4) Involves t(8;14) of heavy Ig & myc-c

Indicates which subtype of Burkitt’s lymphoma

A

African/Endemic subtype Burkitt’s lymphoma

119
Q

Describe the Western/Sporadic subtype of Burkitt’s lymphoma

A

1) Not ass with EBV (-)
2) Manifests as a mass in the ileocecal valve, right iliac fossa, or peritoneum
3) Biopsy shows a starry sky app
4) Patient usually has Gi distress

120
Q

1) Not ass with EBV (-)
2) Manifests as a mass in the ileocecal valve, right iliac fossa, or peritoneum
3) Biopsy shows a starry sky app
4) Patient usually has Gi distress

Indicates which subtype of Burkitt’s lymphoma

A

Western/Sporadic subtype of Burkitt’s lymphoma

121
Q

Describe the Immunodeficiency subtype of Burkitt’s lymphoma

A

presents in the lymph nodes of immunocompromised patients

122
Q

presents in the lymph nodes of immunocompromised patients

Is indicative of which subtype of Burkitt’s lymphoma

A

Immunodeficiency subtype of Burkitt’s lymphoma

123
Q

Describe the etiology of Hodgkin lymphoma (Bcell lymphoma)

A

Typically young adults & adolescents (average age is 32yrs @ diagnosis)

124
Q

Describe the common findings of Hodgkin lymphoma (Bcell lymphoma)

A

1) Reed-Sternberg cells (present in all classic HL)
2) It can be associated with or without EBV infection (target Reed-Sternberg cells)

125
Q

1) Reed-Sternberg cells (present in all classic HL)
2) It can be associated with or without EBV infection (target Reed-Sternberg cells)

Are common findings of which condition?

A

Hodgkin lymphoma (Bcell lymphoma)

126
Q

Describe the features of Reed-Sternberg cells

A

1) Have an OWL’s eyes app
2) CD15+ & CD30+
3) CD45-
4) Release IL-5, IL-10, M-CSF, & Eotaxin (chemokine) to increase reactive leukocytes, macrophages & granulocytes in the tumor
5) Use the PD ligand to evade the immune system (by inhibiting CD8-Tcell apoptosis)

127
Q

1) Have an OWL’s eyes app
2) CD15+ & CD30+
3) CD45-
4) Release IL-5, IL-10, M-CSF, & Eotaxin (chemokine) to increase reactive leukocytes, macrophages & granulocytes in the tumor
5) Use the PD ligand to evade the immune system (by inhibiting CD8-Tcell apoptosis)

Are all features of which cell

A

Reed-Sternberg cells

128
Q

Describe the 3 main causes of Hodgkin lymphoma (Bcell lymphoma)

A

1) EBV targets cells that express CD21 (EBV receptor) & invade them via LMP to infect B-cells and increase NF-kB & proliferation)

2) A mutated REL protooncogene increases NF-kB production (increases Bcell prolif)

3) EBV(-) cells in the LN of patients with Mono can use EBV to turn B cells into Reed-Sternberg cells)

129
Q

1) EBV targets cells that express CD21 (EBV receptor) & invade them via LMP to infect B-cells and increase NF-kB & proliferation)

2) A mutated REL protooncogene increases NF-kB production (increases Bcell prolif)

3) EBV(-) cells in the LN of patients with Mono can use EBV to turn B cells into Reed-Sternberg cells)

Describes the 3 main causes of which condition?

A

Hodgkin lymphoma (Bcell lymphoma)

130
Q

NF-kB activates _______ lineages & JAK+ STAT activate __________ lineages

A

NF-kB activates lymphoid while
JAK/STAT activate myeloid

131
Q

Describe the clinical features of Hodgkin lymphoma (Bcell lymphoma)

A

1) Pruritis
2) Fever
3) Diaphoresis
4) Leukocytosis

It mimics acute infection but it’s not!

132
Q

1) Pruritis
2) Fever
3) Diaphoresis
4) Leukocytosis

It mimics acute infection but it’s not!

Are all clinical features of which condition?

A

Hodgkin lymphoma (Bcell lymphoma)

133
Q

Describe the cause of tumor lysis syndrome

A

triggered 1-5 days after chemotherapy

134
Q

Describe the common findings of tumor lysis syndrome

A

1) Hypocalcemia
2) Hyperuricemia
3) Hyperkalemia
4) Hyperphosphatemia

135
Q

1) Hypocalcemia
2) Hyperuricemia
3) Hyperkalemia
4) Hyperphosphatemia

Are all common findings of which condition?

A

Tumor Lysis Syndrome

136
Q

Describe the clinical features of Tumor Lysis Syndrome

A

1) Hypocalcemia & Hyperphosphatemia lead to:
- Neuromuscular irritability & Tetany
2) Hyperuricemia leads to:
- Renal failure, lactic acidosis, & Dehydration
3) Hyperkalemia leads to:
- Ventricular arrythmias & Sudden death

137
Q

1) Hypocalcemia & Hyperphosphatemia lead to:
- Neuromuscular irritability & Tetany
2) Hyperuricemia leads to:
- Renal failure, lactic acidosis, & Dehydration
3) Hyperkalemia leads to:
- Ventricular arrythmias & Sudden death

Are all clinical features of which condition?

A

Tumor lysis syndrome

138
Q

Describe the etiology of Adult T-cell Leukemia/Lymphoma (ATL)

A

50-60 yr olds, there’s a higher risk in African Americans

139
Q

Describe the 2 causes of Adult T-cell Leukemia/Lymphoma (ATL)

A

1) An HTLV-1 infection
2) IV drug abuse

140
Q

Describe the common findings in Adult T-cell Leukemia/Lymphoma (ATL)

A

1) Production of Tax protein (lets HTLV-1 infiltrate cells & activate NF-kB to up Tcell proliferation)
2) Clover/Flower cells (cells with HTLV-1 inside)

141
Q

1) Production of Tax protein (lets HTLV-1 infiltrate cells & activate NF-kB to up Tcell proliferation)
2) Clover/Flower cells (cells with HTLV-1 inside)

Are common findings of which condition?

A

Adult T-cell Leukemia/Lymphoma (ATL)

142
Q

Describe the clinical features of Adult T-cell Leukemia/Lymphoma (ATL)

A

1) Skin lesions
2) Generalized lymphadenopathy
3) Hepatosplenomegaly
4) Peripheral blood lymphocytosis
5) Hypercalcemia

143
Q

1) Skin lesions
2) Generalized lymphadenopathy
3) Hepatosplenomegaly
4) Peripheral blood lymphocytosis
5) Hypercalcemia

Are all clinical features of which of the following condition?

A

Adult T-cell Leukemia/Lymphoma (ATL)

144
Q

Describe the etiology of Diffuse large Bcell lymphoma

A

It can happen in children & young adults but it’s more common in older persons (~60yrs)

145
Q

Describe the causes of Diffuse large Bcell lymphoma

A

1) Dysregulation of Bcl-6 (DNA-binding zinc finger transcription repressor) causing unregulated proliferation

2) t(8;14) translocation causing overexpression of Bcl-2 (inhibits apoptosis)

146
Q

1) Dysregulation of Bcl-6 (DNA-binding zinc finger transcription repressor) causing unregulated proliferation

2) t(8;14) translocation causing overexpression of Bcl-2 (inhibits apoptosis)

Causes of which condition?

A

Diffuse large Bcell lymphoma

147
Q

Describe the clinical features of Diffuse large Bcell lymphoma

A

1) Presents as a large extra nodal mass
2) Has very large cells with a diffuse growth pattern

148
Q

1) Presents as a large extra nodal mass
2) Has very large cells with a diffuse growth pattern

Indicates which condition?

A

Diffuse large Bcell lymphoma

149
Q

Describe the etiology of mantle cell lymphoma

A

Usually older men aged 50-60/+ yrs

150
Q

Describe the common findings in mantle cell lymphoma

A

t(8;14) translocation of heavy IgH to increase expression of Cyclin D1

151
Q

t(8;14) translocation of heavy IgH to increase expression of Cyclin D1

Findings indicate which condition?

A

Mantle cell lymphoma

152
Q

Describe the clinical features of Mantle cell lymphoma

A

1) Painless Generalized lymphadenopathy
2) Spleen or peyer’s patch involvement
3) Incurable (3-4yr survival time)

153
Q

1) Painless Generalized lymphadenopathy
2) Spleen or peyer’s patch involvement
3) Incurable (3-4yr survival time)

Clinical features indicate which condition?

A

Mantle cell lymphoma

154
Q

Describe the immunomarkers of Mantle cell lymphoma

A

1) Very high cyclin D1
2) CD5+
3) CD23-
4) CD19+ & CD20+

155
Q

1) Very high cyclin D1
2) CD5+
3) CD23-
4) CD19+ & CD20+

indicate which condition?

A

Mantle cell lymphoma

156
Q

Describe the etiology of small cell lymphocytic lymphoma

A

usually 60/+ yr old men

157
Q

Describe the common findings of small cell lymphocytic lymphoma

A

1) Elimination of nodal architecture
2) Increase in prolymphocytes
3) Effects lymph nodes

158
Q

1) Elimination of nodal architecture
2) Increase in prolymphocytes
3) Effects lymph nodes

Are common findings of which condition?

A

small cell lymphocytic lymphoma

159
Q

Describe the clinical features of small cell lymphocytic lymphoma

A

1) Asymptomatic at diagnosis
2) Enlargement of LN
3) Weight-loss/anorexia
4) Fatigue
5) Generalized lymphadenopathy
6) Hepatosplenomegaly

160
Q

1) Asymptomatic at diagnosis
2) Enlargement of LN
3) Weight-loss/anorexia
4) Fatigue
5) Generalized lymphadenopathy
6) Hepatosplenomegaly

Are all clinical findings of which condition?

A

small cell lymphocytic lymphoma

161
Q

Describe the etiology of Chronic lymphocytic leukemia

A

usually older men 60yrs/+

162
Q

Describe the common findings of Chronic lymphocytic leukemia

A

1) An absolute lymphocyte # >5000 ***
2) Smudge cells
3) Proliferation centers

163
Q

1) An absolute lymphocyte # >5000 ***
2) Smudge cells
3) Proliferation centers

Are common findings of which condition?

A

Chronic lymphocytic leukemia

164
Q

Describe the causes of Chronic lymphocytic leukemia

A

Deletions of the following:
- 13q14.3
- 11q1
- 17p
- XXX12q

165
Q

Deletions of the following:
- 13q14.3
- 11q1
- 17p
- XXX12q

Causes indicate which condition?

A

Chronic lymphocytic leukemia

166
Q

Describe the immunomarker of Chronic lymphocytic leukemia

A

1) Pan Bcell (CD19+ & CD20+)
2) CD5+ & CD23+
3) CD10-

167
Q

1) Pan Bcell (CD19+ & CD20+)
2) CD5+ & CD23+
3) CD10-

Indicate which condition?

A

Chronic lymphocytic leukemia

168
Q

In Chronic lymphocytic leukemia which gene deletion is associated with the poorest prognosis?

169
Q

Describe clinical features of Chronic lymphocytic leukemia

A

1) Asymptomatic at diagnosis
2) Non-specific symptoms (weight-loss, anorexia, & fatigue)
3) Generalized lymphadenopathy
4) Hepatosplenomegaly

170
Q

1) Asymptomatic at diagnosis
2) Non-specific symptoms (weight-loss, anorexia, & fatigue)
3) Generalized lymphadenopathy
4) Hepatosplenomegaly

Are clinical features of which condition?

A

Chronic lymphocytic leukemia

171
Q

Describe the 3 main complications of Chronic lymphocytic leukemia

A

1) Warm (IgG) or cold (IgM) Ab-autoimmune hemolytic anemia
2) Hypogammaglobulinemia (high bacterial infection risk)
3) It can progress to aggressive diffuse large B cell lymphoma (Richter syndrome)

172
Q

Describe the etiology of plasma cell myeloma (multiple myeloma)

A

Older adult men, usually 65-70yrs olds, being African American is a bigger risk

173
Q

Describe the causes of plasma cell myeloma (multiple myeloma)

A

1) Rearrangements of IgH (x6p21) with cyclin D3
2) Deletions of 17p (p53 gene)

Both lead to unregulated proliferation of neoplastic plasma cells that make too much M protein (IgG or IgA or kappa or lambda light chain variants)

174
Q

1) Rearrangements of IgH (x6p21) with cyclin D3
2) Deletions of 17p (p53 gene)

Both lead to unregulated proliferation of neoplastic plasma cells that make too much M protein (IgG or IgA or kappa or lambda light chain variants)

Causes indicate which condition?

A

plasma cell myeloma (multiple myeloma)

175
Q

Describe the common findings of plasma cell myeloma (multiple myeloma)

A

1) High IL-6 (Growth factor for plasma cells)
2) High IL-7 (Osteoclast activator)
3) High monoclonal IgG spike (Kappa) or IgA
4) High levels of Bence-Jones protein (casts in kidneys)
5) Hyperglobulinemia (high serum globins & proteins)
6) High ESR
7) Rouleaux formation of RBCs (poker chip)

176
Q

1) High IL-6 (Growth factor for plasma cells)
2) High IL-7 (Osteoclast activator)
3) High monoclonal IgG spike (Kappa) or IgA
4) High levels of Bence-Jones protein (casts in kidneys)
5) Hyperglobulinemia (high serum globins & proteins)
6) High ESR
7) Rouleaux formation of RBCs (poker chip)

Are all common findings of which condition?

A

plasma cell myeloma (multiple myeloma)

177
Q

Describe the clinical features of plasma cell myeloma (multiple myeloma)

A

1) “CRAB”
Calcification
hypeRcalcemia
Anemia
“punched out” Bone lytic lesions (Severe bone pain)

2) Osteopenia (diffuse mineralization)
Tumors in axial skeleton
3) Leukopenia
4) Thrombocytopenia
5) Renal insufficiency with azokmia
6) Primary amyloidosis (Puffy face, confusion, periorbital edema)

178
Q

1) “CRAB”
Calcification
hypeRcalcemia
Anemia
“punched out” Bone lytic lesions (Severe bone pain)

2) Osteopenia (diffuse mineralization)
Tumors in axial skeleton
3) Leukopenia
4) Thrombocytopenia
5) Renal insufficiency with azokmia
6) Primary amyloidosis (Puffy face, confusion, periorbital edema)

Are all clinical findings of which condition?

A

plasma cell myeloma (multiple myeloma)

179
Q

Describe the immunomarkers of plasma cell myeloma (multiple myeloma)

A

1) CD138 (syndecan)
2) CD56+

180
Q

1) CD138 (syndecan)
2) CD56+

Are indicative of what condition?

A

plasma cell myeloma (multiple myeloma)

181
Q

In plasma cell myeloma (multiple myeloma) Rouleaux formations of RBCs are due to high levels of ____

182
Q

In plasma cell myeloma (multiple myeloma) what indicates a poor prognosis?

A

High levels of IL-6

183
Q

How is plasma cell myeloma (multiple myeloma) able to persist in the host?

A

The tumor increases IL-6 levels, which trigger RANKLE to activate osteoclasts (IL7)

The osteoclasts lyse the bone matrix causing an increase in
-MIP-1alpha (tumors on axial skeleton)
-FRP-2 (calcification
- WNT (punched out lesions

184
Q

Which bones are at risk of being affected by plasma cell myeloma (multiple myeloma)

A
  1. Spine
  2. Ribs
  3. Skull
  4. Pelvis
  5. Femur
  6. Clavicle
  7. Scapulae
185
Q

Describe the 3 stages of progression of mycosis fungiodes (cutaneous Tcell lymphoma)

A
  1. Premycotic (erythematous, eczematoid, &/or psoriasiform rashes)
  2. Plaques (raised)
  3. Tumors
186
Q
  1. Premycotic (erythematous, eczematoid, &/or psoriasiform rashes)
  2. Plaques (raised)
  3. Tumors
A

the 3 stages of progression of mycosis fungiodes (cutaneous Tcell lymphoma)

187
Q

Describe the common findings of mycosis fungiodes (cutaneous Tcell lymphoma)

A

1) High CLA (Cutaneous leukocyte antigen)
2) High CCR4 & CCR10 (Chemokines)
3) Neoplastic T cells with cerebriform nucleus
4) Halo cells & spongiosis (Pautier misprocesses

188
Q

1) High CLA (Cutaneous leukocyte antigen)
2) High CCR4 & CCR10 (Chemokines)
3) Neoplastic T cells with cerebriform nucleus
4) Halo cells & spongiosis (Pautier misprocesses

Common findings indicate which condition?

A

mycosis fungiodes (cutaneous Tcell lymphoma)

189
Q

Describe what Sezary syndrome is

A

A Cutaneous T cell variant that manifests as a generalized exfoliative erythroderma which can progress to an aggressive T cell lymphoma

190
Q

A Cutaneous T cell variant that manifests as a generalized exfoliative erythroderma which can progress to an aggressive T cell lymphoma

Describes which condition?

A

Sezary syndrome

191
Q

What are the common findings with Sezary syndrome?

A

1) High serum IgE
2) Sezary cell
3) Redness

192
Q

Describe the etiology of Langerhans cell histiocytosis (Histiocytosis X)

A

Usually male children

193
Q

Describe the common findings of Langerhans cell histiocytosis (Histiocytosis X)

A

1) Dendritic/Langerhans cells in the skin that express S-100 & CD1A
2) Birbeck granules (tennis rackets)

194
Q

1) Dendritic/Langerhans cells in the skin that express S-100 & CD1A
2) Birbeck granules (tennis rackets)

Indicate which condition?

A

Langerhans cell histiocytosis (Histiocytosis X)

195
Q

Describe the Eosinophilic granule subtype of Langerhans cell histiocytosis (Histiocytosis X)

A

A unifocal condition that affects the medullary cavities of bones (skull, ribs, & femur)

Patient (young 10-12yrs) experiences severe bone pain

196
Q

A unifocal condition that affects the medullary cavities of bones (skull, ribs, & femur)

Patient (young 10-12yrs) experiences severe bone pain

Describes which subtype of Langerhans cell histiocytosis (Histiocytosis X)?

A

Eosinophilic granule

197
Q

Describe Hand-Schuller Christian triad subtype of Langerhans cell histiocytosis (Histiocytosis X)

A

A multifocal condition usually affecting young (6-10yrs) that causes:
-Multiple boney masses
- Diabetes insipidus (post pituitary)
- Exophthalmos (post pituitary)
- Headaches
- Bloody vision
- Lytic bone lesions

198
Q

A multifocal condition usually affecting young (6-10yrs) that causes:
-Multiple boney masses
- Diabetes insipidus (post pituitary)
- Exophthalmos (post pituitary)
- Headaches
- Bloody vision
- Lytic bone lesions

Describes which subtype of Langerhans cell histiocytosis (Histiocytosis X)?

A

Hand-Schuller Christian triad subtype

199
Q

Describe the Letterer-Siwe disease subtype of Langerhans cell histiocytosis (Histiocytosis X)

A

A multifocal condition that appears before 2yrs old & causes:
- Cutaneous lesions (Seborrheic eruptions on trunk & scalp)
- Hepatosplenomegaly
- Lymphadenopathy
- Pulmonary lesions
- Destructive osteolytic bone lesions
- Recurrent infections (Otitis media & mastoiditis)

200
Q

A multifocal condition that appears before 2yrs old & causes:
- Cutaneous lesions (Seborrheic eruptions on trunk & scalp)
- Hepatosplenomegaly
- Lymphadenopathy
- Pulmonary lesions
- Destructive osteolytic bone lesions
- Recurrent infections (Otitis media & mastoiditis)

Describes which subtype of Langerhans cell histiocytosis (Histiocytosis X)?

A

Letterer-Siwe disease subtype

201
Q

Describe the etiology of Chronic Myelogenous leukemia

A

A broad age range but it peaks in the 45-85yrs gap (64 being the median)

202
Q

Describe the causes of Chronic Myelogenous leukemia

A

BCR-ABL combination (due to a t(9;22) translocation (Philadelphia)) it activates BCR-ABL tyrosine kinase

203
Q

BCR-ABL combination (due to a t(9;22) translocation (Philadelphia)) it activates BCR-ABL tyrosine kinase

Cause indicates which condition?

A

Chronic Myelogenous leukemia

204
Q

Describe the common findings of Chronic Myelogenous leukemia

A

1) Band cells
2) Blast cells
3) LOW LAP
4) Hypercellular bone marrow
5) High eosinophils, basophils, megakaryocytes, & neutrophils
6) Scattered macrophages (Abundant Sea blue histocytes)
7) Leukocytosis of 100,000

205
Q

1) Band cells
2) Blast cells
3) LOW LAP
4) Hypercellular bone marrow
5) High eosinophils, basophils, megakaryocytes, & neutrophils
6) Scattered macrophages (Abundant Sea blue histocytes)
7) Leukocytosis of 100,000

Are all common findings of which condition?

A

Chronic Myelogenous leukemia

206
Q

Describe the etiology of T cell Acute lymphoblastic Leukemia/Lymphoma

A

Usually male children & adolescents ages 8-14yrs old

207
Q

Describe the Causes of T cell Acute lymphoblastic Leukemia/Lymphoma

A

A gain of function mutation in NOTCH1 (needed for Tcell development)

208
Q

A gain of function mutation in NOTCH1 (needed for Tcell development)

Cause is indicative of which condition?

A

T cell Acute lymphoblastic Leukemia/Lymphoma

209
Q

Describe the features of T cell Acute lymphoblastic Leukemia/Lymphoma

A

1) Mediastinal thymic masses
2) Lymphadenopathy
3) Splenomegaly

210
Q

1) Mediastinal thymic masses
2) Lymphadenopathy
3) Splenomegaly

Describes which condition?

A

T cell Acute lymphoblastic Leukemia/Lymphoma

211
Q

Describe the immunomarkers of T cell Acute lymphoblastic Leukemia/Lymphoma

A

1) TdT +
2) CD1+, CD2+, CD5+, & CD7+

212
Q

1) TdT +
2) CD1+, CD2+, CD5+, & CD7+

Indicate which condition?

A

T cell Acute lymphoblastic Leukemia/Lymphoma

213
Q

What are the 2 complications of T cell Acute lymphoblastic Leukemia/Lymphoma

A

1) Superior vena cava syndrome (facial edema in morning & engorged neck neins)
2) Pancoast tumor (bronchial tumor that presses against Rec. laryngeal nerv)

214
Q

Describe the etiology of Hairy cell Leukemia (B-cell)

A

Usually middle aged men (whites are the highest risk)

215
Q

Describe the causes of Hairy cell Leukemia (B-cell)

A

A point mutation in Serine/threonine kinase BRAF gene

216
Q

A point mutation in Serine/threonine kinase BRAF gene

Indicates which condition?

A

Hairy cell Leukemia (B-cell)

217
Q

Describe the common findings of Hairy cell Leukemia (B-cell)

A

1) Leukemic cells with fine hair-like filamentous projections (cytoskeleton)
2) Dry tap is needed (due to extensive Bone marrow fibrosis)
3) Tear drop RBCs

218
Q

1) Leukemic cells with fine hair-like filamentous projections (cytoskeleton)
2) Dry tap is needed (due to extensive Bone marrow fibrosis)
3) Tear drop RBC

Common findings indicate which condition?

A

Hairy cell Leukemia (B-cell)

219
Q

Describe the immunomarkers of Hairy cell Leukemia (B-cell)

A

1) CD11C+
2) CD25+
3) CD103+
4) TRAP+ (tartrate-resistant-acid-phosphate)
5) BRAF V(valine) 600F(Glutamate)

220
Q

1) CD11C+
2) CD25+
3) CD103+
4) TRAP+ (tartrate-resistant-acid-phosphate)
5) BRAF V(valine) 600F(Glutamate)

Indicate which condition?

A

Hairy cell Leukemia (B-cell)

221
Q

Describe the clinical features of Hairy cell Leukemia (B-cell)

A

1) MASSIVE HEPATOSPLENOMEGALY
2) Rare lymphadenopathy
3) Pancytopenia

222
Q

1) MASSIVE HEPATOSPLENOMEGALY
2) Rare lymphadenopathy
3) Pancytopenia

Are features of which condition

A

Hairy cell Leukemia (B-cell)

223
Q

Describe the etiology of Acute lymphoblastic leukemia (ALL)

A

Most common in children under 15yrs it peaks at 3yrs, & Hispanics & most responsive to therapy

224
Q

Describe the immunomarkers Acute lymphoblastic leukemia (ALL)

A

1) TdT +
2) CD10+, CD19+, & CD22+
3) PAX5+

225
Q

1) TdT +
2) CD10+, CD19+, & CD22+
3) PAX5+

Indicate which condition

A

Acute lymphoblastic leukemia (ALL)

226
Q

Describe the common findings of Acute lymphoblastic leukemia (ALL)

A

1) Increase lymphoblasts
2) Decreased Hematopoiesis
3) Starry-sky appearance

227
Q

1) Increase lymphoblasts
2) Decreased Hematopoiesis
3) Starry-sky appearance

are all common findings of which condition?

A

Acute lymphoblastic leukemia (ALL)

228
Q

Describe the common features of Acute lymphoblastic leukemia (ALL)

A

1) Anemia + fatigue
2) Thrombocytopenia
3) Recurrent infections
4) Bone pain
5) Generalized lymphadenopathy
6) Hepatosplenomegaly
7) Testicular enlargement
8) Headaches
9) Vomiting
10) nerve palsies

229
Q

1) Anemia + fatigue
2) Thrombocytopenia
3) Recurrent infections
4) Bone pain
5) Generalized lymphadenopathy
6) Hepatosplenomegaly
7) Testicular enlargement
8) Headaches
9) Vomiting
10) nerve palsies

Are indicative of which condition?

A

Acute lymphoblastic leukemia (ALL)

230
Q

What are considered good prognosis factors for Acute lymphoblastic leukemia (ALL)

A

1) Age 2-10yrs
2) low WBC#
3) t(12;21) rearrangement
4) Hyperdiploidy

231
Q

1) Age 2-10yrs
2) low WBC#
3) t(12;21) rearrangement
4) Hyperdiploidy

are all indicators of a ___ prognosis ofAcute lymphoblastic leukemia (ALL)

232
Q

What are considered poor prognosis factors for Acute lymphoblastic leukemia (ALL)

A

1) Younger than 2ys
2) Presents in adolescences or young adulthood
3) Peripheral blood blast count of over 100,000

233
Q

1) Younger than 2ys
2) Presents in adolescences or young adulthood
3) Peripheral blood blast count of over 100,000

are all indicators of a ___ prognosis of Acute lymphoblastic leukemia (ALL)

234
Q

Bcell neoplasms immunophenotype

A

CD19/20+

mature TdT -
immature TdT +

235
Q

Tcell neoplasms immunophenotype

A

CD3/7+

Mature Tdt -
Immature TdT+

236
Q

Myeloperoxidase immunophenotype

237
Q

Sepsis

A

Neutropenia #60,000 & Dohle bodies

238
Q

Leukemoid reaction

A

WBC >50,000
High LAP
no bands
CD13/15+
CD33/34-

Path SJSM (26 decks)

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