Autoimmune Disease

Front 1

X linked (Bruton) agammaglobinemia

Back 1

1- Defect in BTK gene - tyrosine kinase gene (X linked recessive) - No B cell maturation

2- Recurrent bacterial and enterioviral infection after 6 months ( decrease maternal IgG)

3- Absent B cell in peripheral blood ( no Ig of any class)

4- Absent/scant lymph nodes and tonsil

5- Live attenuated vaccine contraindicated

Front 2

Selective IgA deficiency

Back 2

1- Most common primary immunodeficiency

2- Mostly asymptomatic

3- Can see Airway and GI infections

4- Autoimmune disease

5- Atopy and anaphylaxis with IgA containing products

6- Increase susceptible to giardiasis

7- Cause false positive BHCG

Front 3

Common variable immunodeficiency

Back 3

1- Defect in B cell differentiation

2- Develop during childhood but diagnose at puberty

3- Autoimmune disease, bronchiectasis, lymphoma and sinopulmonary disease

4- Decrease plasma cells and immunoglobulins

Front 4

Autoimmune disease of B cells

Back 4

1- X linked (Bruton) agammaglobinemia

2- Selective IgA deficiency

3- Common variable immunodeficiency

Front 5

Autoimmune disease of T cells

Back 5

1- Thymic aplasia

2- IL-12 receptor deficiency

3- Autosomal dominant hyper Ig-E syndrome

4- Chronic mucocutanous Candidiasis

Front 6

Thymic aplasi

Back 6

1- 22q11 micro-deletion, failure of development of 3th and 4th pharyngeal pouches - thymus and parathyroid

2- DiGeorge syndrome- thymus, parathyroid and cardiac defect

3- Velocardiofacial syndrome - palate, facies and cardiac defect

4- CATCH 22- 1- cardiac abnormality

2- Abnormal facies

3- Thymic hypoplasia

4- Cleft palate

5- Hypoparathyroids - decrease Ca tetany

5- Decrease T cell, PTH and Ca

6- Absent Thymic shadow on CXR

Front 7

IL-12 receptor deficiency

Back 7

1- Decrease Th1 cell response (autosomal recessive)

2-Disseminated Mycobacteria and fungal infections after administration of BCG

3- Decrease IFN- y

4- most common cause of Medelian susceptibility to mycobacterial disease (MSMD)

Front 8

Autosomal dominant hyper IgE syndrome job disease

Back 8

1- Defect in Th17 cell due to mutation of STAT 3 - impaired recruitment of neutrophils to the site of infection

2- Features 1- Cold

2- Staphylococcus Abscess

3- Retained baby teeth

4- Course facies

5- Dermatological abnormalities (eczema)

6- Increase IgE

7- Increase Bone Fracture after minor trauma

Front 9

Chronic mucocutanous candidiasis

Back 9

1- T cell dysfunction against Candida

2- Defect of AIRE

3- Persistent non-invasive Candida infection of skin and mucus membrane

4- Absent in vitro T cell proliferation in response to Candida antigen

5- Absent cutaneous response to Candida antigen

Front 10

Autoimmune disease of B and T cells

Back 10

1- Sever combined immunodeficiency

2- Ataxia telangiectasia

3- Hyper IgM syndrome

4- Wiskott- Aldrich syndrome

Front 11

Severe combined immunodeficiency syndrome

Back 11

1- Defect of IL-2R gamma chain (x linked recessive)

Adenosine deaminase deficiency (autosomal recessive)

RAG mutation- VDJ recombination chain

2- Failure to thrive, developmental delay, chronic diarrhea, thrush, recurrent bacterial viral fungal and protozoan infections

3- Decrease T cell receptor excision circle (TREC)

4- Absent Thymic shadow (CXR), germinal center (lymph node biopsy) and T cell (flow cytometry)

5- Live vaccine contraindicated

Front 12

Ataxia telangiectasia

Back 12

1- Defect in ATM gene- Failure to detect DNA damage

2- Failure to halt the progression of the cell cycle - mutation accumulate

3- Triad 1- Cerebellar defect (Ataxia)

2- Spider agioma (telangiectasia)

3- Ig A deficiency

Increase sensitivity to radiation

4- Increase AFP

5- Decrease IgG, A and E

6- lymphopenia and cerebral atrophy

7- Increase risk of lymphoma and leukemia

Front 13

Ataxia telangiectasia

Back 13

1- Defect in ATM gene- Failure to detect DNA damage

2- Failure to halt the progression of the cell cycle - mutation accumulate

3- Triad 1- Cerebellar defect (Ataxia)

2- Spider agioma (telangiectasia)

3- Ig A deficiency

Increase sensitivity to radiation

4- Increase AFP

5- Decrease IgG, A and E

6- lymphopenia and cerebral atrophy

7- Increase risk of lymphoma and leukemia

Front 14

Hyper Ig M syndrome

Back 14

1- CD40 L defect on Th cell (x linked recessive) class switching defect

2- Severe pyogenic and opportunistic infection early in life

3- Normal or increase IgM

Decrease Ig G, A and E

Failure to make germinal center

Front 15

Ataxia telangiectasia

Back 15

1- Defect in ATM gene- Failure to detect DNA damage

2- Failure to halt the progression of the cell cycle - mutation accumulate

3- Triad 1- Cerebellar defect (Ataxia)

2- Spider agioma (telangiectasia)

3- Ig A deficiency

Increase sensitivity to radiation

4- Increase AFP

5- Decrease IgG, A and E

6- lymphopenia and cerebral atrophy

7- Increase risk of lymphoma and leukemia

Front 16

Hyper Ig M syndrome

Back 16

1- CD40 L defect on Th cell (x linked recessive) class switching defect

2- Severe pyogenic and opportunistic infection early in life

3- Normal or increase IgM

Decrease Ig G, A and E

Failure to make germinal center

Front 17

Wishkott- Aldrich syndrome

Back 17

1- Defect in WAS gene- leukocytes and platelets unable to reorganize actin in cytoskeleton

2- Wishkott- Aldrich

2- Thrombocytopenia

3- Eczema

4- Recurrent pyogenic infections

3- Increase risk of autoimmune disease and malignancy

4- normal or decrease IgG and IgM increase Ig A and E

Front 18

Phagocyte dysfunction

Back 18

1- Phagocyte dysfunction (leukocyte adhesion deficiency type 1)

2- Chediak- Higashi syndrome

3- Chronic granulomatous disease

Front 19

Leukocyte adhesive deficiency type 1

Back 19

1- Defect in LEA- 1 interferon (CD18) protein on phagocytes- defect in migration and chemotaxis (autosomal recessive)

2- 1- Late separation of umbilical cord (> 30 days)

2- Absent pus

3- Dysfunctional neutrophils

3- Increase neutrophils in blood , absent neutrophils at infection site - poor wound healing

Front 20

Chediak- Higashi syndrome

Back 20

1- Defect in lysosomal trafficking regulator gene (LYST)

2- Microtubles defect in phagoso-lysosome fusion

3- 1-Progressive neurodegeneration and Pancytopenia

2- Lymphohistocytosis

3- Albinism

4-Infection recurrent pyogenic

5- Peripheral neuropathy

4- Giant granules in granulocytes

Front 21

Immunodeficiency that are X linked

Back 21

1- Bruton X linked agammaglobinemia

2- Severe combine immunodeficiency syndrome

3- Hyper IgM syndrome

4- Wishkott- Aldrich

5- Chronic granulomatous disease

Front 22

Immunodeficiency that cause false negative celiac disease results

Back 22

Selective IgA deficiency

Front 23

Autosomal recessive immunodeficiency

Back 23

IL-12 receptor deficiency

Adenosine deaminase deficiency in SCID

Ataxia telangiectasia

Leukocyte adhesion deficiency type 1

Chediak Higashi syndrome

Front 24

What cardiac defect are typically associated with DeGeorge syndrome and velocardiofacial syndrome

Back 24

Conotruncal abnormality such as tetralogy of Fallot and truncus arteriosus

Front 25

Diagnosis

Back 25

Giant granules in granulocytes seen in Chediak- Higashi syndrome

Front 26

Diagnosis and what precaution must be taken for the patient

Back 26

Ataxia telangiectasia

Limit X Ray

Sensitivity to radiation

Front 27

Name 2 immunodeficiency disorder where the Thymic shadow is absent

Back 27

Thymic aplasia

SCID

Front 28

Level of which molecule is used as a newborn screening for severe combined immunodeficiency

Back 28

Decrease T cell receptor excision circles

Front 29

Cyclosporine

Back 29

1- Calcineurin inhibitor

2- Binds to Cyclophilin

3- Blocks T cell activation by preventing IL 2 transcription

Front 30

Use of cyclosporine

Back 30

Rheumatoid arthritis

Psoriasis

Front 31

Toxicity of cyclosporine

Back 31

1- Nephrotoxicity

2- Neurotoxicity

3- Gingival hyperplasia

4- Hypertension

5- Hyperlipidemia

6- Hirsutism

Front 32

Tacrolimus (FK 506)

Back 32

1- Calcineurin inhibitor

2- Binds to FK 506 binding protein (FKBP)

3- Block T cell activation by preventing IL 2 transcription

Front 33

Use of tacrolimus

Back 33

1- Rheumatoid arthritis

2- Psoriasis

Front 34

Use of tacrolimus

Back 34

1- Rheumatoid arthritis

2- Psoriasis

Front 35

Toxicity of Tacrolimus

Back 35

1- Nephrotoxicity

2- Increase risk of diabetes mellitous and neurotoxicity

3- No gingival hyperplasia or hirsutism

4- Hypertension

5- Hyperlipidemia

Front 36

Sircolimus (rompamycin)

Back 36

1- mTor inhibitor

2- Bind to FKBP

3- Block T cell activation and B cell differentiation by preventing response to IL2

Front 37

Use of Sircolimus

Back 37

1- Renal transplant rejection prophylaxis specifically Sir Basil’s binding transplant

Front 38

Toxicity of sircolimus

Back 38

1- Pancytopenia

2- Insulin resistance

3- Hyperlipidemia

Front 39

Basiliximab

Back 39

1- Monoclonal antibody

2- Block IL 2R

Front 40

Toxicity of basiliximab

Back 40

1- Hypertension

2- Edema

3- Tremor

Front 41

Azathioprine

Back 41

1- Antimetabolite of 6 mercaptopurine

2- Inhibit lymphocyte proliferation by blocking nucleotide synthesis

Front 42

Use of azathioprine

Back 42

1- Rheumatoid arthritis

2- Crohn’s disease

3- Glomeraleronephropathy

4- Autoimmune condition

Front 43

Toxicity of azathioprine

Back 43

Pancytopenia

Front 44

Mycophenolate (mofetil)

Back 44

1- IMP dehydrogenase inhibitor reversible

2- Prevent purine synthesis of B and T cell

3- Associated with invasive CMV infection

Front 45

Use of mycophenolate

Back 45

Lupus nephritis

Front 46

Toxicity of mycophenolate

Back 46

1- Pancytopenia

2- GI upset

3- Hypertension

4- Hyperglycemia

Front 47

Glucocorticoid

Back 47

1- NF-BK inhibitor

2- Suppress B cell and T cell function by decreasing transcription of many cytokines

3- Induce T cell apoptosis

4- Demigration of WBC causes atypical leukocytosis

5- Adrenal insufficiency develop if the drug is stoped abruptly after chronic use

Front 48

Use of glucocorticoids

Back 48

1- Autoimmune and inflammatory condition

2- Adrenal insufficiency

3- Asthma

4- CLL

5- Non- Hodgkin lymphoma

Front 49

What are the side effects of prednisone on MSK

Back 49

Osteoporosis

Avascular necrosis of the head of the femur

Myopathy

Front 50

Which immunosuppressant is often found in drug-eluting stent

Back 50

Sircolimus (rapamycin)

Front 51

Which immunosuppressant medication is synergistic with cyclosporine

Back 51

Sirolimus - no Nephrotoxicity

Front 52

Long term use of immunosuppressant increase the risk of which 2 condition

Back 52

Cancer

Infections

Front 53

What 2 factors can amplify the nephrotoxicity of Calcineurin inhibitor

Back 53

Higher doses

Poor renal function

Front 54

Which messenger, directly downstream from the T cell receptor is activated to promote inflammatory cytokines (eg IL-2) gene transcription

Back 54

NFAT, which is generated from NFAT-P by Calcineurin mediated dephosphorylation

Front 55

mTOR is responsible for diving the transcription of what type of gene

Back 55

Proliferation gene

Front 56

What are the 2 monoclonal antibodies that bind to and inhibit the IL-2 receptor

Back 56

Declizumab

Basiliximab

Front 57

6 mercaptopurine inhibits what specific protein involved in nucleotide synthes

Back 57

PRPP aminotransferase

Front 58

What downstream messenger is activated by IL-2R signaling

Back 58

mTOR

Front 59

Erythropoietin

Back 59

1- Epoetin alpha (EPO analog)

2- Anemia (especially renal failure)

Front 60

Erythropoietin

Back 60

1- Epoetin alpha (EPO analog)

2- Anemia (especially renal failure)

Front 61

Colony stimulating factor

Back 61

1- Filgrastim (G-CDF)

Sargramostim (GM-CSF)

2- leukopenia (recover granulocytes and monocytes ****)

Front 62

Thrombopoietin

Back 62

1- Romiplostim (TPO analog)

Eltrombopag (TPO receptor agonist)

2- Autoimmune thrombocytopenia (platelet stimulator)

Front 63

Thrombopoietin

Back 63

1- Romiplostim (TPO analog)

Eltrombopag (TPO receptor agonist)

2- Autoimmune thrombocytopenia (platelet stimulator)

Front 64

Bone marrow stimulating cytokine

Back 64

Erythropoietin

Clingy stimulating factor

Thrombopoietin

Front 65

Thrombopoietin

Back 65

1- Romiplostim (TPO analog)

Eltrombopag (TPO receptor agonist)

2- Autoimmune thrombocytopenia (platelet stimulator)

Front 66

Bone marrow stimulating cytokine

Back 66

Erythropoietin

Clingy stimulating factor

Thrombopoietin

Front 67

Immunotherapy recombinant cytokines

Back 67

IL 2

IFN alpha beta and gamma

Front 68

Thrombopoietin

Back 68

1- Romiplostim (TPO analog)

Eltrombopag (TPO receptor agonist)

2- Autoimmune thrombocytopenia (platelet stimulator)

Front 69

Bone marrow stimulating cytokine

Back 69

Erythropoietin

Clingy stimulating factor

Thrombopoietin

Front 70

Immunotherapy recombinant cytokines

Back 70

IL 2

IFN alpha beta and gamma

Front 71

IL 2 recombinant cytokines

Back 71

1- Aldesleukin

2- Renal cell carcinoma

Metastatic melanoma

Front 72

Thrombopoietin

Back 72

1- Romiplostim (TPO analog)

Eltrombopag (TPO receptor agonist)

2- Autoimmune thrombocytopenia (platelet stimulator)

Front 73

Bone marrow stimulating cytokine

Back 73

Erythropoietin

Clingy stimulating factor

Thrombopoietin

Front 74

Immunotherapy recombinant cytokines

Back 74

IL 2

IFN alpha beta and gamma

Front 75

IL 2 recombinant cytokines

Back 75

1- Aldesleukin

2- Renal cell carcinoma

Metastatic melanoma

Front 76

Interferon alpha beta and gamma cytokines

Back 76

Alpha 1- HCV, HBV

2- Renal cell carcinoma

Beta 1- multiple sclerosis

Gamma 1- chronic granulomatous disease

Front 77

Alemtuzumab

Back 77

1- CD 52

2- CLL

Multiple sclerosis

Front 78

Bevacizumab

Back 78

1- VEGF

2- 1- Colorectal cancer

2- Non small cell lung cancer

3- Renal cell cancer

4- Neurovascular age related macular degeneration

5- Proliferative diabetic retinopathy

6- Macular edema

Front 79

Rituximab

Back 79

1- CD 20

2- 1- CLL

2- Multiple sclerosis

3- B cell non- Hodgkin lymphoma

4- ITP

5- Rheumatoid arthritis

3- Risk of PML in patients with JC virus

Front 80

Trastuzumab

Back 80

1- HER2

2- 1- Breast cancer

2- Gastric cancer

Front 81

Adalimumab inflixamab

Back 81

1- Soluble TNF alpha

2- 1- Rheumatoid arthritis

2- Psoriasis

3- IBD

4- Ankylosis spondylitis

Front 82

Eculizumab

Back 82

1- Complement protein C5

2- Paroxysmal nocturnal hemoglobinuria

Front 83

Ixekizumab Secukinumab

Back 83

1- IL 17A

2- 1- Psoriasis

2- Psoriatic arthritis

Front 84

Natalizumab

Back 84

1- Alpha 4 integrin

2- 1- Crohn’s disease

2- Multiple sclerosis

3- Risk of PML Progressive multi focal leukoencephalopathy in patients with JC virus

Front 85

Ustekinumab

Back 85

1- IL 12/ IL 23

2- 1- Psoriasis

2- Psoriatic arthritis

Front 86

Abciximab

Back 86

1- Platelet glycoproteins IIb/IIIa

2- Anti-platelet agent that prevent ischemic complications in patients undergoing percutaneous coronary intervention

Front 87

Denosumab

Back 87

1- RANKL

2- Osteoporosis (inhibit osteoclasts maturation) mimic osteoprotegesis)

Front 88

Omalizumab

Back 88

1- IgE

2- Refractory allergic asthma (prevent IgE binding to FcERI)

Front 89

Palivizumab

Back 89

1- RSV F protein

2- RSV prophylaxis in high risk infants

Front 90

Therapeutic antibodies for cancer therapy

Back 90

1- Alemtuzumab

2- Bevacizumab

3- Rituximab

4- Trastuzumab

Front 91

Therapeutic antibodies for cancer therapy

Back 91

1- Alemtuzumab

2- Bevacizumab

3- Rituximab

4- Trastuzumab

Front 92

Therapeutic agents for autoimmune disease

Back 92

1- Adalimumab/inflizamab

2- Eculizamab

3- Ixekizumab/ secukinmab

4- Natalizumab

5- Ustekinumab

Front 93

Other therapeutic agents

Back 93

1- Abciximab

2- Denosumab

3- Omalizumab

4- Palivizumab

Front 94

What drug is a decoy tumor necrosis factor alpha receptor

Back 94

Etanercept (not a monoclonal antibody)

Front 95

Screening of what infection is necessary when beginning treatment with adalimumab or infliximabb

Back 95

Tuberculosis due to risk of reactivation

Front 96

Which therapeutic antibody mimic factor VIII in the treatment of hemophilia A

Back 96

Emicixumab

Front 97

Emicizumab

Back 97

1- Factor IXa and X

2- Hemophilia A