Test Code #

17

CPT Code(s) #

88346

Test Name

Paraneoplastic Pemphigus Antibody Titer

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 hours from the time of specimen receipt

Specimen Requirements:

Collect 5-10 ml of blood in a red top or serum separator tube. If possible, separate serum from clot and place into red capped tube provided with Beutner Laboratories collection kits. If separation facilities are not available, the blood can be sent in the tube used for collection.

Sample Stability:

Stable at ambient temperature during shipment. If sample is stored prior to shipment, it is stable refrigerated (2-8ºC) up to five days and frozen (-20ºC or lower) up to one 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%.

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