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AIIMS > 26.6.2013(pathology)49 > Flashcards

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
Carpal tunnel syndrome seen in which amyloidosis
ATTR beta2 amyloid AL
25
Pupil change seen in hereditary amyloid syndrome
B/L scalloped pupil
26
Rx of choice for systemic ATTR
Liver transplantation
27
Most effective Rx for AL amyloid
Stem cell transplantation
28
Latest Rx for AL amyloidosis
Iododoxorubicin
29
Latest Rx of AA amyloidosis
Eprodisate
30
Latest Rx of ATTR
Diflunisal
31
Amyloid protein deposited in cerebral amyloid angiopathy
Cystatin C
32
Berry aneurysm is due to
Degeneration of tunica media
33
Mitochondrial disorders associated with retinitis pigmentosa
NARP Kearns sayre MELAS
34
Cardiac defect associated with LHON
Pre-excitation syndrome Kearns sayre is associated with heart block
35
B/L deafness is seen with which mitochondrial diseases
MELAS MERRF KSS
36
Multiple lipoma is seen in which mitochondrial disease
MERRF
37
Mitochondrial disease associated with hypertrophic cardiomyopathy
MELAS
38
Basal ganglia lucencies are seen in which mitochondrial diseases
Leigh | NARP
39
Leigh syndrome
Subacute relapsing encephalopathy | Cerebellar and brainstem signs
40
Markers for Ewing sarcoma
CD99 | MIC 2
41
Markers of plasma cells
``` CD19 CD38 CD45 CD56 Cyto K and L ```
42
Markers of mature B cells
``` CD19 CD20 CD21 CD22 CD23 CD79a Surface Ig ```
43
Markers of immature B cells
``` CD10 CD19 CD20 CD34 CD38 CD45 K,L Tdt CD13 ```
44
Translocations in burkitt lymphoma
t(8;14) heavy chain t(2;8) kappa t(8;22) lambda
45
Burkitt lymphoma markers
CD10 BCL6 Surface Ig All mature B cell markers
46
CK7+/CK20+
Transitional cell Ca Mucinous ovarian Ca Ca pancreas
47
CK7+/CK20-
``` Breast Lung thyroid Endometrium Serous ovarian Ca ```
48
CK7-/CK20+
Colorectal Stomach Merckel cell
49
CK7-/CK20-
Liver Clear cell Ca of kidney Prostate
50
Marker for malignant fibrous histiocytoma
CD68 | HAM56
51
Neruoendocrine tumor markers
Leu7 NSE synaptophysin Chromogranin
52
Markers for osteosarcoma
Osteopontin Osteocalcin Osteonectin Bone GIA protein
53
Fillipin is used to stain
Cholesterol
54
Cholesterol esters are stained by
Schultz
55
Nile blue sulphate stains
Phosphatidyl choline | Phosphatidyl serine
56
HLA class 3 codes for
Complements C2 ,C4 ,properdin TNF A,TNF B Heat shock proteins
57
BCL in diffuse large B cell lymphoma
BCL2 | BCL6
58
CD5 and CD23
Both positive in CLL CD5 alone positive in mantle Both negative in follicular lymphoma
59
BCL2 positive in
Follicular BCL6 positive in burkitt
60
Causes of increased serum haptoglobin
``` Acute phase reactant Billiary obstruction Acute rheumatic disease and collagen diseases Neoplasia,lymphomas Tissue destruction ```
61
Markers of langerhans cell histiocytosis
HLA DR S100 CD1a
62
Onset of letterer siewe
Before 2 yrs
63
Langerhans cell histiocytosis subtypes
``` Multifocal multi system(letterer siewe) Unifocal unisystem(Eosinophilic granuloma) Multifocal unisystem(hand schuller Christian triad) ```
64
Pulmonary langerhans cell histiocytosis is common in
Adult smokers
65
Diff btw normal langerhans cells and neoplastic cells
Normal cells express CCR6 | Neoplastic cells express both CCR6 and CCR7
66
Manifestations of letterer siewe disease
``` Skin lesions resembling seborrheic dermatitis Hepatosplenomegaly Lymphadenopathy Pulmonary lesions Destructive osteolytic lesions Anemia,thrombocytopenia Fever ```
67
Ligand for CCR6
CCL20 found in skin and bone
68
Receptor for CCR7
CCL19 and 21 found in lymphoid organ
69
Tumors asssociated with neurofibroma
``` Optic nerve glioma Wilms Pheochromocytoma Juvenile xanthogranuloma Rhabdomyosarcoma Myeloid leukemia ```
70
HPE in HIV infection of CNS
Microglial nodules Perivascular giant cell Vacuolations in posterior and lateral column Foamy macrophages

AIIMS (13 decks)

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