26.6.2013(pathology)49 Flashcards

0
Q

Most common form of localised amyloidosis

A

A beta

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1
Q

Highly sensitive biopsy site for amyloidosis

A

Abdominal fat pad

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2
Q

Most common form of systemic amyloidosis

A

AL

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3
Q

Most common form of familial amyloidosis

A

Mutated TTR

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4
Q

Pathognomonic of ATTR amyloidosis

A

Vitreous opacities

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5
Q

The only type of systemic amyloidosis that occurs in children

A

AA

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6
Q

Commonest organ involved in AA amyloidosis

A

Kidney

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7
Q

Organs spared in AL amyloidosis

A

CNS

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8
Q

Rx of CCF in amyloidosis

A

Diuretics

Digitalis,CCB,Betablockers are contraindicated

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9
Q

The ratio of lambda to kappa

A

Normal persons and multiple myeloma 1:2

In AL amyloidosis it’s 2:1

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10
Q

Second most common organ involved in amyloidosis

A

Heart

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11
Q

Common cause of death in AL amyloidosis

A

Cardiac failure

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12
Q

Clotting factor deficient in amyloidosis

A

X

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13
Q

Type of cardiomyopathy in amyloidosis

A

Restrictive

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14
Q

Causes of hypertrophic cardiomyopathy

A

Friederich ataxia
Storage disorders
Infants of diabetic mother

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15
Q

Causes of dilated cardiomyopathy

A
Alcohol
Peripartum
Hemochromatosis 
Sarcoidosis
Myocarditis
Anemia
Doxorubicin
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16
Q

Rx of FMF

A

Colchicine

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17
Q

Amyloidosis with predominant peripheral neuropathy

A

A apoA1

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18
Q

Amyloid associated with lattice corneal dystrophy

A

A gel(gelsolin)

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19
Q

AIAPP(amylin,islet amyloid polypeptide) is deposited in

A

Insulinoma

DM2

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20
Q

AIns amyloid is deposited in

A

Site of insulin injection

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21
Q

Amyloid to be named (atbn) is deposited in

A

Pindborg tumors

Odontogenic tumors

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22
Q

Lactoferrin amyloid deposition is seen in

A

Corneal amyloid deposited in trichinosis

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23
Q

Macroglossia is seen in which amyloidosis

A

AL

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24
Q

Carpal tunnel syndrome seen in which amyloidosis

A

ATTR
beta2 amyloid
AL

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25
Q

Pupil change seen in hereditary amyloid syndrome

A

B/L scalloped pupil

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26
Q

Rx of choice for systemic ATTR

A

Liver transplantation

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27
Q

Most effective Rx for AL amyloid

A

Stem cell transplantation

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28
Q

Latest Rx for AL amyloidosis

A

Iododoxorubicin

29
Q

Latest Rx of AA amyloidosis

A

Eprodisate

30
Q

Latest Rx of ATTR

A

Diflunisal

31
Q

Amyloid protein deposited in cerebral amyloid angiopathy

A

Cystatin C

32
Q

Berry aneurysm is due to

A

Degeneration of tunica media

33
Q

Mitochondrial disorders associated with retinitis pigmentosa

A

NARP
Kearns sayre
MELAS

34
Q

Cardiac defect associated with LHON

A

Pre-excitation syndrome

Kearns sayre is associated with heart block

35
Q

B/L deafness is seen with which mitochondrial diseases

A

MELAS
MERRF
KSS

36
Q

Multiple lipoma is seen in which mitochondrial disease

A

MERRF

37
Q

Mitochondrial disease associated with hypertrophic cardiomyopathy

A

MELAS

38
Q

Basal ganglia lucencies are seen in which mitochondrial diseases

A

Leigh

NARP

39
Q

Leigh syndrome

A

Subacute relapsing encephalopathy

Cerebellar and brainstem signs

40
Q

Markers for Ewing sarcoma

A

CD99

MIC 2

41
Q

Markers of plasma cells

A
CD19
CD38
CD45
CD56
Cyto K and L
42
Q

Markers of mature B cells

A
CD19
CD20
CD21
CD22
CD23
CD79a
Surface Ig
43
Q

Markers of immature B cells

A
CD10
CD19
CD20
CD34
CD38
CD45
K,L
Tdt
CD13
44
Q

Translocations in burkitt lymphoma

A

t(8;14) heavy chain
t(2;8) kappa
t(8;22) lambda

45
Q

Burkitt lymphoma markers

A

CD10
BCL6
Surface Ig
All mature B cell markers

46
Q

CK7+/CK20+

A

Transitional cell Ca
Mucinous ovarian Ca
Ca pancreas

47
Q

CK7+/CK20-

A
Breast 
Lung 
thyroid
Endometrium
Serous ovarian Ca
48
Q

CK7-/CK20+

A

Colorectal
Stomach
Merckel cell

49
Q

CK7-/CK20-

A

Liver
Clear cell Ca of kidney
Prostate

50
Q

Marker for malignant fibrous histiocytoma

A

CD68

HAM56

51
Q

Neruoendocrine tumor markers

A

Leu7
NSE
synaptophysin
Chromogranin

52
Q

Markers for osteosarcoma

A

Osteopontin
Osteocalcin
Osteonectin
Bone GIA protein

53
Q

Fillipin is used to stain

A

Cholesterol

54
Q

Cholesterol esters are stained by

A

Schultz

55
Q

Nile blue sulphate stains

A

Phosphatidyl choline

Phosphatidyl serine

56
Q

HLA class 3 codes for

A

Complements C2 ,C4 ,properdin
TNF A,TNF B
Heat shock proteins

57
Q

BCL in diffuse large B cell lymphoma

A

BCL2

BCL6

58
Q

CD5 and CD23

A

Both positive in CLL
CD5 alone positive in mantle
Both negative in follicular lymphoma

59
Q

BCL2 positive in

A

Follicular

BCL6 positive in burkitt

60
Q

Causes of increased serum haptoglobin

A
Acute phase reactant
Billiary obstruction
Acute rheumatic disease and collagen diseases
Neoplasia,lymphomas
Tissue destruction
61
Q

Markers of langerhans cell histiocytosis

A

HLA DR
S100
CD1a

62
Q

Onset of letterer siewe

A

Before 2 yrs

63
Q

Langerhans cell histiocytosis subtypes

A
Multifocal multi system(letterer siewe)
Unifocal unisystem(Eosinophilic granuloma)
Multifocal unisystem(hand schuller Christian triad)
64
Q

Pulmonary langerhans cell histiocytosis is common in

A

Adult smokers

65
Q

Diff btw normal langerhans cells and neoplastic cells

A

Normal cells express CCR6

Neoplastic cells express both CCR6 and CCR7

66
Q

Manifestations of letterer siewe disease

A
Skin lesions resembling seborrheic dermatitis
Hepatosplenomegaly
Lymphadenopathy
Pulmonary lesions
Destructive osteolytic lesions
Anemia,thrombocytopenia
Fever
67
Q

Ligand for CCR6

A

CCL20 found in skin and bone

68
Q

Receptor for CCR7

A

CCL19 and 21 found in lymphoid organ

69
Q

Tumors asssociated with neurofibroma

A
Optic nerve glioma
Wilms
Pheochromocytoma 
Juvenile xanthogranuloma 
Rhabdomyosarcoma
Myeloid leukemia
70
Q

HPE in HIV infection of CNS

A

Microglial nodules
Perivascular giant cell
Vacuolations in posterior and lateral column
Foamy macrophages