Pathology 1 - Block 1 - Part 5

davidwurbel7's version from 2016-04-12 18:57


Question Answer
Pathologic proteinaceous substance, deposited in the extracellular space in various tissues and organs of the bodyAmyloidosis
The abnormal fibrils of amyloid are produced by aggregation of thisMisfolded Protein
With progressive accumulation it encroaches on adjacent cells and produces thisPressure Atrophy
Amyloid is stained with this stainCongo Red
This amyloid is derived from Ig light chains produced by plasma cells. Can be seen plasma cell tumors (contains complete Ig light chain: ʎ and ƙ)Amyloid Light Chain (AL)
From an unique non-Ig protein synthesized by the liver. Seen in inflammation (SAA, acute phase reactants). It is derived from proteolysis from a bigger molecule called SAAAmyloid Associated (AA)
Produced by the β-amyloid precursor protein (Alzheimer disease CNS)Aβ Amyloid
A mutant form is deposited in a group of genetic diseases: familial amyloid polyneuropathiesTransthyretin (ATTR)
Component of MHC class 1 molecules and a normal serum protein (Aβ2m), which is seen to complicate the course of patients on long term hemodialysisβ2-Microglobulin
Misfolded ‘prion’ protein clump in extracellular space. Prion diseasePrion Protein
Plasma cell disordersImmune Dyscrasias
Referred to as “primary amyloidosis”. AL type amyloid with systemic distributionSystemic Amyloidosis
Plasma cell malignancy. produces abnormally large quantity of ‘M’ protein. Both Ig molecules and light chains are seenMultiple Myeloma
Light chains that may escape in urine because of their small molecular weight are frequently excreted thru’ urine is referred to asBence-Jones Protein
Also referred to as “secondary amyloidosis”. AA type, systemic distribution. Often seen in chronic inflammation, in which SAA is converted in to AA amyloidReactive Systemic Amyloidosis
Deposition of β2-microglobulin seen in patients on long term hemodialysis. β2-microglobulin present in serum of patients with renal failure is not filtered through dialysis membrane. Therefore remain in circulation and gets deposited in tissues. Patients present with ‘carpal tunnel syndrome’Hemodialysis-Associated Amyloidosis
Patients present with ‘carpal tunnel syndrome’ that is also on long term hemodialysis may haveHemodialysis-Associated Amyloidosis
Autosomal recessive, very rare. Abnormally high production of IL-1, in response to inflammatory stimuli. The gene encodes for: pyrin. There is widespread amyloidosis (AA type)Familial Mediterranean Fever
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Question Answer
This type of amyloid is seen in medullary carcinoma of thyroid - MEN syndrome and in islet Langerhans in patients with type II diabetes mellitusEndocrine Amyloid
Amyloid appears pink with this stainH & E Stain
Amyloid appears highlighted usingCongo Red Stain
Amyloid appears this way on polarizing microscopyApple Green Birefringence
Deposits limited to splenic follicles, with tapioca like granulesTapioca Spleen
Deposits involve wall of the splenic sinuses, and connective tissue framework of red pulpLardaceous Spleen
Amyloid deposits in the tongueMacroglossia