AMYLOIDOSIS
EXTRACELLULAR DEPOSITION OF AMORPHOUS, EOSINOPHILIC, INSOLUBLE FIBRILLAR PROTIEN WITHIN VARIOUS BODY TISSUES
5TH -6TH DECADE
WHITE > OTHER RACES
MALES > FEMALES
DIVIDED INTO LOCALIZED (RARE)–GOOD Px AND SYSTEMIC (COMMON)–POOR Px
3 TYPES OF SYSTEMIC
PRIMARY (B-CELL OR PLASMA CELL NEOPLASMS–ALPHA IMMUNOGLOBULIN LIGHT CHAINS)—-15% OF PTS WITH MULTIPLE MYELOMA (BENCE JONES PROTIENS) AA, AL, P
SECONDARY (REACTIVE) CHRONIC ILLNESS, TB, RA
12-50% H&N INVOLVEMENT, PRIMARY SITE IS THE LARYNX, UP TO 15% LESIONS MULTIFOCAL
(PRIMARILY TONGUE)–DECREASED MOBILITY, DYSARTHRIA, DYSPHAGIA, DENTAL IMPRESSIONS, PAINFUL TO PALP
LARYNX— PRIMARILY AFFECTS TVC’S (HOARSENESS) IN DIFF Dx FOR SUBGLOTTIC STENOSIS—EYE, NP, SINUSES
CAN INVOLVE THE HEART—CARDIOMEGALLY, CARDIOMYOPATHY, CHF, GI TRACT, SKIN, NERVES, AND TONGUE—-ALSO KIDNEYS–CHRONIC RENAL FAILURE
Dx : BIOPSY—-APPEARS GROSSLY AS FIRM TAN-YELLOW–MUCOSAL COVERED SWELLING
STAIN + CONGO RED APPEARS SALMON PINK UNDER LIGHT MICROSCOPY
BIREFRINGENT APPLE- GREEN UNDER POLARIZED LIGHT MICROSCOPY—ADD POTASSIUM PERMANGANATE—GET REVERSIBILITY OF BIREFRINGENCE = SECONDARY AMYLOID
CRYSTAL VIOLET—METACHROMIC
+ THIOFLAVIN T STAIN
TO DETERMINE SYSTEMIC INVOLVEMENT–ASPIRATION OF ABD FAT AND Bx OF MINOR SALIVARY GLANDS OR RECTAL MUCOSA (80% SENSITIVE)
Rx : SUPPORTIVE (CHEMOTHERAPEUTICS), RT, COLCHICINE, MELPHALAN, PREDNISONE—NO BETTER SURVIVAL
LOCAL EXCISION MAY BE PALLIATIVE