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Pathology > Diseases of the Immune System > Flashcards

Diseases of the Immune System Flashcards

1
Q

Caused by activation of TH2 CD4 helper T cells by environmental antigens —> PRODUCTION OF IgE ANTIBODIES which become attached to mast cells

anaphylaxis
bronchial asthma
allergic rhinitis
sinusitis (hay fever)
food allergies

A

Type I (immediate, IgE-mediated)

vascular dilation
edema
smooth muscle contraction
mucus production
tissue injury and inflammation

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2
Q

caused by ANTIBODIES that bind to fixed tissues and cell antigens and promote phagocytosis and destruction of the coated cells or trigger pathologic inflammation in tissues

AIHA
Immune Thrombocytopenic Purpura
Pemphigus Vulgaris
ANCA-vasculitis
Goodpasture syndrome
Acute Rheumatic Fever
Myasthenia Graves
Insulin Resistant Diabetes
Pernicious anemia

A

Type II (Antibody-mediated)

phagocytosis and lysis of cells
inflammation
functional derangements w/o evidence of cell or tissue injury

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3
Q

Caused by ANTIBODIES BINDING ANTIGENS TO FORM COMPLEXES that circulate and may deposit in vascular beds and stimulate inflammation secondary to complement activation

SLE
PSAGN
Polyarteritis Nodosa
Reactive arthritis
Serum sickness
Arthus reaction

A

Type III (Immune Complex Mediated)

inflammation
necrotizing vasculitis (fibrinoid necrosis)

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4
Q

Cell mediated responses in which T LYMPHOCYTES CAUSE TISSUE INJURY either by producing cytokines that induce inflammation and activate macrophages or by directly killing cells

Rheumatoid arthritis
Multiple sclerosis
Type I DM
IBD
Psoriasis
Contact sensitivity
PPD

A

Type IV (T cell mediated/delayed)

perivascular cellular infiltrates
edema
granuloma formation
cell destruction

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5
Q

32/F w/ DOB, bilateral elbow joint pains and rash after sun exposure. CBC showed low hemoglobin and reticulocytosis. (+) ANA titer. Diagnosis

A

Systemic Lupus Erythematosus (SLE)

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6
Q

Mechanisms of organ damage in SLE

A

Type III
Type II (opsonization and phagocytosis, hematologic manifestations)

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7
Q

MC autoantibody in SLE

A

ANA

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8
Q

MOST SPECIFIC autoantibodies for SLE

A

anti-Sm (Smith)
anti-dsDNA - correlates w/ disease activity

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9
Q

Histopathologic findings in SLE w/ skin involvement

A

LM: liquefactive degeneration of basal layer
edema of DEJ
mononuclear infiltrates around blood vessels and skin apppendages

IF: deposition of IG and complement at DEJ

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10
Q

Type of LE associated w/ HYDRALAZINE, INH, POCAINAMIDE and D-PENICILLAMINE intake, rarely associated w/ anti-dsDNA

Associated w/ ANTI-HISTONE antibodies

A

Drug-Induced Lupus

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11
Q

34/F, w/ DRY EYES and DRY MOUTH. Lip biopsy shows ACINAR ATROPHY, FIBROSIS and HYALINIZATION of the minor salivary glands

Diagnosis

A

Sjogren syndrome (late)

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12
Q

MC and most important autoantibody detected in Sjogren syndrome

A

Anti-Ro (SS-A)
Anti-La (SS-B)

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13
Q

45/F with CHRONIC GERD, hx of PROGRESSIVE DYSPNEA and CKD with noted THICKENING OF THE SKIN

Diagnosis

A

Systemic Sclerosis(Scleroderma)

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14
Q

MC autoantibody associated w/ DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

A

anti-DNA topoisomerase I (anti-Scl 70)

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15
Q

Common autoantibody associated w/ LIMITED SCLERODERMA (limited cutaneous systemic sclerosis)

A

Anti-centromere antibodies

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16
Q

48/M, s/p kidney transplant suddenly developed BLOODY URINE few hrs after the procedure. Nephrectomy revealed a CYANOTIC, MOTTLED and FLACCID KIDNEY w/ NECROTIC CORTEX. Biopsy shows THROMBOTIC OCCLUSION OF THE CAPILLARIES and FIBRINOID NECROSIS OF ARTERIAL WALLS.
Diagnosis

A

Hyperacute Rejection

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17
Q

45/F w/ signs of RENAL FAILURE 2 mos AFTER her RENAL TRANSLANT. Biopsy shows EXTENSIVE INTERSTITIAL MONONCULEAR INFILTRATE w/ edema and mild interstitial hemorrhage.

(+) CD3, CD4 and CD8

A

Acute cellular (T cell mediated) rejection, Tubuloinerstitial type (tubulitis)

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18
Q

30/M, developed OLIGURIA and SUBSEQUENT RENAL FAILURE 3 WEEKS AFTER an un uneventful kidney transplant. Biopsy shows INFLAMMATION OF GLOMERULI and PERITUBULAR CAPILLARIESw/ FOCAL THROMBOSIS OF SMALL VESSELS.

Diagnosis

A

Acute Antibody-mediated Rejection

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19
Q

24/F, diagnosed w/ renal failure underwent renal transplant. 4 years later, INCREASING LEVEL OF CREATININE was noted. Biopsy. shows INTERSTITIAL FIBROSIS and TUBULAR ATROPHY.

Diagnosis

A

Chronic Rejection

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20
Q

66/M, known case of MULTIPLE MYELOMA underwent HSC transplantation presented w/ BLOODY DIARRHEA and GENERALIZED MORBILIFORM RASH FEW WEAKS after the procedure.

Diagnosis

A

Acute graft VS host disease (GVHD)

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21
Q

Few years after HSC transplantation developd cutaneous sclerosis, jaundice and dysphagia. Barium swallow showed ESOPHAGEAL STRICTURES.

Diagnosis

A

Chronic GVHD

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22
Q

6 mos/M, w/ MORBILIFORM RASH, RECURRENT ORAL THRUSH, DIAPER RASH and FAILURE TO THRIVE.
PE: (-) CERVICAL LYMPHADENOPATHY
CXR: (-) thymic shadow

Diagnosis

A

Severe Combined Immunodeficiency (SCID)

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23
Q

7 mos/M, w/ UNREMARKABLE 1st 6 MOS of LIFE, RECURRENT PNEUMONIA and STEATORRHEIC DIARRHEA.
Sputum Culture: H. influenzae
Fecalysis: Giardia lamblia trophozoites
PE: (-) cervical lymphadenopathy
LOW serum Igs

A

X-Linked Agammaglobulinemia ( Bruton disease)

24
Q

4 mos/ M, CYANOSIS since BIRTH associated w/ COARSE FACIAL FEATURES and CLEFT PALATE presented w/ RECURRENT VIRAL INFECTIONS.
PE: +3 reflexes
CXR: boot shaped heart (-) thymic shadow
FISH: (+) deletion of Ch22q11.2 locus

Diagnosis

A

DiGeorge syndrome

25
Q

8 mos/M, history of ECZEMATOUS DERMATITIS and RECURRENT INFECTIONS.
CBC: low platelet

Diagnosis

A

Wiskott-Aldrich Syndrome (x-liked recessive)

26
Q

16/F, with HISTORY OF VIRAL URTI 3 weeks prior, developed RECURRENT UTI and AGE.
Low serum IgA

Diagnosis

A

Isolated IgA deficiency (acquired)

27
Q

MC secondary immunodeficiency caused by HIV infection
PROFOUND IMMUNE DEFICIENCY - HALLMARK
Opportunistic infections, secondary neoplasms and neurologic manifesations
CD4 T cells

A

Acquired Immune Deficiency Syndrome (AIDS)

28
Q

MC fungal infection in patients w/ AIDS

A

Candidiasis

29
Q

MC type of lymphoma associated w/ HIV infection

A

B-cell lymphoma

30
Q

AIDS-DEFINING SARCOMA caused by Human Herpesvirus 8 (HHV8)
Typified by appearance of PURPLE PATCHES, PLAQUES or NODULES on the SKIN

A

Kaposi Sarcoma

31
Q

Common Autoimmune Diseases and HLA types involved

A

HLA-B27 - ankylosing spondylitis and RA

HLA-DR,DQ - type I DM and multiple sclerosis

HLA-DQ2, DQ8 - Celiac disease

HLA-DR4 - RA

HLA-DQ - SLE

32
Q

TRIAD of Sjogren Syndrome

A

dry eyes (keratoconjunctivitis sicca)
dry mouth (xerostomia)
autoimmune mediated destruction of lacrimal gland and salivary glands

33
Q

MC associated autoimmune disease in Sjogren syndrome

A

Rheumatoid Arthritis

34
Q

TRIAD of Systemic Sclerosis (Scleroderma)

A

chronic inflammation (autoimmunity)
widespread damage to small blood vessels
progressive interstitial and perivascular fibrosis in the skin and multiple organs

35
Q

CREST SYNDROME

A

Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

36
Q

Autoantibodies in scleroderma associated with HIGHER risk for pulmonary fibrosis and peripheral vascular disease

A

Anti-Scl 70 (anti-DNA topoisomerase I)

37
Q

Defect in migration and chemotaxis of phagocytes

LATE SEPARATION OF UMBILICAL CORD

recurrent skin and mucosal bacterial infection
ABSENT PUS

A

Leukocyte Adhesion Deficiency (LAD)

AR

defect expression of INTEGRIN
CD18

37
Q

Defect in migration and chemotaxis of phagocytes

LATE SEPARATION OF UMBILICAL CORD

recurrent skin and mucosal bacterial infection
ABSENT PUS

A

Leukocyte Adhesion Deficiency (LAD)

AR

defect expression of INTEGRIN
CD18

38
Q

Defect in LYST gene – problem in PHAGOLYSOSOME FUNCTIONING

ALBINISM
neurodegeneration
recurrent pyogenic infections

A

CHEDIAK HIGASHI SYNDROME

AR

39
Q

Defect in MICROBICIDAL activity

Deficiency of NADPH OXIDASE

Severe, persistent and chronic pyogenic infections caused by catalase POSITIVE bacteria

A

Chronic Granulomatous Disease

XR

40
Q

Primary Immunodeficiency (Genetic Defects)

INNATE IMMUNITY (Granulocytes)

A

Leukocyte Adhesion Deficiency (LAD)
CHEDIAK HIGASHI SYNDROME
Chronic Granulomatous Disease

41
Q

Primary Immunodeficiency (Genetic Defects)

ADAPTIVE IMMUNITY (Lymphocytes)

A

SCID
X-linked (Bruton) Agammaglobulinemia
Thymic aplasia (CATCH 22)

42
Q

Absent or markedly decreased B cells in circulation, depressed serum Ig levels (ALL CLASSES)

ABSENT PLASMA CELLS

RECURRENT BACTERIAL AND ENTEROVIRAL INFECTIONS AFTER 6 mos

A

X-linked (Bruton) Agammaglobulinemia

XR

underdeveloped germinal centers of lymph nodes, Peyer patches, appendix and tonsils

43
Q

Defective IL-2 receptor gamma chain

ADENOSINE DEAMINASE DEFICIENCY

SMALL THYMUS (-) lymphoid cells
HYPOPLASTIC lymphoid tissues

failure to thrive, chronic diarrhea, thrush

A

SEVERE COMBINED IMMUNODEFICIENCY (SCID)

44
Q

22q11 microdeletion
failure to develop 3rd and 4th pharyngeal pouches

ABSENT THYMUS (no thymic shadow on CXR)
ABSENT PARATHYROID GLANDS

LOW T cells in blood and lymphoid tissues

A

Thymic aplasia (CATCH 22)

CATCH

Cardiac defects (conotruncal abnormalities)
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia d.t. parathyroid aplasia

44
Q

Triad of Wiskott Aldrich Syndrome

WAS gene mutation –> combined B and T cell disorder

associated with SMALL PLATELETS

A

Thrombocytopenia
Infection
Eczema

45
Q

MC secondary immunodeficiency

A

AIDS

46
Q

Hallmark of AIDS

A

Profound immune deficiency

47
Q

Most abundant viral antigen and is widely used to diagnose HIV

A

protein p24

48
Q

ATTACHES to CD4 – conformation change – new recognition site of CXCR4 and CCR5 – gp120 binds to CXCR4 and CCR5

A

gp120

49
Q

FUSION PROTEIN – integrates itself into host cell membrane – facilitates fusion and delivery of viral genome

A

gp41

50
Q

Acute retroviral syndrome

A

flu-like illness
rash
cervical adenopathy
diarrhea
vomiting

51
Q

Phase of CLINICAL LATENCY - few or no clinical manifestations

Slow but steady decline in CD4+T cell counts

Increasing viral load

A

CHRONIC PHASE (AIDS)

7-10 yrs

52
Q

Dramatic increase in viral load

Opportunistic infections, secondary neoplasms, CNS disease

A

AIDS

typical phase length (from onset to death) - 2-3 years

53
Q

MC and most serious form of organ involvement in amyloidosis

A

KIDNEY

54
Q

Major organ involved in senile systemic amyloidosis

A

HEART

Pathology (24 decks)

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