017
Test #
Paraneoplastic Pemphigus Antibody
CPT Code(s) #
88346
If Profile, Includes Tests:
N/A
Disease Name:
Paraneoplastic Pemphigus
Type of Study:
Serum Studies
Methodology:
Indirect Immunofluorescence
Substrate:
Rat bladder
Reference Range:
Negative: <1:10
Units:
Titer
Schedule:
Assay performed daily Monday-Friday. Report availability is within 48-72 hours from the time of specimen receipt.
Specimen Requirements:
Collect 2-5 ml of blood in a red top Vacutainer or serum separator (SST) tube, allow blood to clot, and centrifuge. Label tube appropriately.
Sample Stability:
Room temperature = stable during shipment
2ºC to 8ºC = 5 days
-25ºC to -15ºC = 1 year
Clinical Relevance:
Paraneoplastic pemphigus, or PNP, is a rare but often fatal autoimmune blistering disease, with five-year mortality rates approaching 40%. PNP is most commonly associated diagnoses are hematologic malignancies and occasionally sarcomas and other solid-organ malignancies. In children, the most commonly associated cancer is Castleman disease. Indirect immunofluorescence using rat bladder epithelium substrate is a sensitive and specific test, and it is positive in about 80% of cases of PNP. Paraneoplastic pemphigus, or PNP, is a rare but often fatal autoimmune blistering disease, with five-year mortality rates approaching 40%.
