26.6.2013(pathology)49 Flashcards
Most common form of localised amyloidosis
A beta
Highly sensitive biopsy site for amyloidosis
Abdominal fat pad
Most common form of systemic amyloidosis
AL
Most common form of familial amyloidosis
Mutated TTR
Pathognomonic of ATTR amyloidosis
Vitreous opacities
The only type of systemic amyloidosis that occurs in children
AA
Commonest organ involved in AA amyloidosis
Kidney
Organs spared in AL amyloidosis
CNS
Rx of CCF in amyloidosis
Diuretics
Digitalis,CCB,Betablockers are contraindicated
The ratio of lambda to kappa
Normal persons and multiple myeloma 1:2
In AL amyloidosis it’s 2:1
Second most common organ involved in amyloidosis
Heart
Common cause of death in AL amyloidosis
Cardiac failure
Clotting factor deficient in amyloidosis
X
Type of cardiomyopathy in amyloidosis
Restrictive
Causes of hypertrophic cardiomyopathy
Friederich ataxia
Storage disorders
Infants of diabetic mother
Causes of dilated cardiomyopathy
Alcohol Peripartum Hemochromatosis Sarcoidosis Myocarditis Anemia Doxorubicin
Rx of FMF
Colchicine
Amyloidosis with predominant peripheral neuropathy
A apoA1
Amyloid associated with lattice corneal dystrophy
A gel(gelsolin)
AIAPP(amylin,islet amyloid polypeptide) is deposited in
Insulinoma
DM2
AIns amyloid is deposited in
Site of insulin injection
Amyloid to be named (atbn) is deposited in
Pindborg tumors
Odontogenic tumors
Lactoferrin amyloid deposition is seen in
Corneal amyloid deposited in trichinosis
Macroglossia is seen in which amyloidosis
AL
Carpal tunnel syndrome seen in which amyloidosis
ATTR
beta2 amyloid
AL
Pupil change seen in hereditary amyloid syndrome
B/L scalloped pupil
Rx of choice for systemic ATTR
Liver transplantation
Most effective Rx for AL amyloid
Stem cell transplantation
Latest Rx for AL amyloidosis
Iododoxorubicin
Latest Rx of AA amyloidosis
Eprodisate
Latest Rx of ATTR
Diflunisal
Amyloid protein deposited in cerebral amyloid angiopathy
Cystatin C
Berry aneurysm is due to
Degeneration of tunica media
Mitochondrial disorders associated with retinitis pigmentosa
NARP
Kearns sayre
MELAS
Cardiac defect associated with LHON
Pre-excitation syndrome
Kearns sayre is associated with heart block
B/L deafness is seen with which mitochondrial diseases
MELAS
MERRF
KSS
Multiple lipoma is seen in which mitochondrial disease
MERRF
Mitochondrial disease associated with hypertrophic cardiomyopathy
MELAS
Basal ganglia lucencies are seen in which mitochondrial diseases
Leigh
NARP
Leigh syndrome
Subacute relapsing encephalopathy
Cerebellar and brainstem signs
Markers for Ewing sarcoma
CD99
MIC 2
Markers of plasma cells
CD19 CD38 CD45 CD56 Cyto K and L
Markers of mature B cells
CD19 CD20 CD21 CD22 CD23 CD79a Surface Ig
Markers of immature B cells
CD10 CD19 CD20 CD34 CD38 CD45 K,L Tdt CD13
Translocations in burkitt lymphoma
t(8;14) heavy chain
t(2;8) kappa
t(8;22) lambda
Burkitt lymphoma markers
CD10
BCL6
Surface Ig
All mature B cell markers
CK7+/CK20+
Transitional cell Ca
Mucinous ovarian Ca
Ca pancreas
CK7+/CK20-
Breast Lung thyroid Endometrium Serous ovarian Ca
CK7-/CK20+
Colorectal
Stomach
Merckel cell
CK7-/CK20-
Liver
Clear cell Ca of kidney
Prostate
Marker for malignant fibrous histiocytoma
CD68
HAM56
Neruoendocrine tumor markers
Leu7
NSE
synaptophysin
Chromogranin
Markers for osteosarcoma
Osteopontin
Osteocalcin
Osteonectin
Bone GIA protein
Fillipin is used to stain
Cholesterol
Cholesterol esters are stained by
Schultz
Nile blue sulphate stains
Phosphatidyl choline
Phosphatidyl serine
HLA class 3 codes for
Complements C2 ,C4 ,properdin
TNF A,TNF B
Heat shock proteins
BCL in diffuse large B cell lymphoma
BCL2
BCL6
CD5 and CD23
Both positive in CLL
CD5 alone positive in mantle
Both negative in follicular lymphoma
BCL2 positive in
Follicular
BCL6 positive in burkitt
Causes of increased serum haptoglobin
Acute phase reactant Billiary obstruction Acute rheumatic disease and collagen diseases Neoplasia,lymphomas Tissue destruction
Markers of langerhans cell histiocytosis
HLA DR
S100
CD1a
Onset of letterer siewe
Before 2 yrs
Langerhans cell histiocytosis subtypes
Multifocal multi system(letterer siewe) Unifocal unisystem(Eosinophilic granuloma) Multifocal unisystem(hand schuller Christian triad)
Pulmonary langerhans cell histiocytosis is common in
Adult smokers
Diff btw normal langerhans cells and neoplastic cells
Normal cells express CCR6
Neoplastic cells express both CCR6 and CCR7
Manifestations of letterer siewe disease
Skin lesions resembling seborrheic dermatitis Hepatosplenomegaly Lymphadenopathy Pulmonary lesions Destructive osteolytic lesions Anemia,thrombocytopenia Fever
Ligand for CCR6
CCL20 found in skin and bone
Receptor for CCR7
CCL19 and 21 found in lymphoid organ
Tumors asssociated with neurofibroma
Optic nerve glioma Wilms Pheochromocytoma Juvenile xanthogranuloma Rhabdomyosarcoma Myeloid leukemia
HPE in HIV infection of CNS
Microglial nodules
Perivascular giant cell
Vacuolations in posterior and lateral column
Foamy macrophages