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HelpTest Code PC2TS Purkinje Cell Cytoplasmic Antibody Type 2 (PCA-2) Titer, Serum
Specimen Required
Only orderable as a reflex. For more information see:
-PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum
-DMS2 / Dementia, Autoimmune/Paraneoplastic Evaluation, Serum
-ENS2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
-EPS2 / Epilepsy, Autoimmune/Paraneoplastic Evaluation, Serum
-MDS2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum
-MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
-AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
-DYS2 / Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum
-GID2 / Gastrointestinal Dysmotility, Autoimmune/Paraneoplastic Evaluation, Serum
Secondary ID
43438Useful For
Evaluating patients who present with a subacute neurological disorder of undetermined etiology and have risk factors for lung cancer
Reporting an end titer result from serum specimens
Testing Algorithm
If the indirect immunofluorescence pattern suggests Purkinje cell cytoplasmic antibody type 2 (PCA-2), then this test will be performed at an additional charge.
Method Name
Only orderable as a reflex. For more information see:
-PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum
-DMS2 / Dementia, Autoimmune/Paraneoplastic Evaluation, Serum
-ENS2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
-EPS2 / Epilepsy, Autoimmune/Paraneoplastic Evaluation, Serum
-MDS2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum
-MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
-AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
-DYS2 / Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum
-GID2 / Gastrointestinal Dysmotility, Autoimmune/Paraneoplastic Evaluation, Serum
Indirect Immunofluorescence Assay (IFA)
Reporting Name
PCA-2 Titer, SSpecimen Type
SerumSpecimen Minimum Volume
0.6 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Refrigerated (preferred) | 28 days | |
| Frozen | 28 days | ||
| Ambient | 72 hours |
Clinical Information
Purkinje cell autoantibodies (PCA) are among the antineuronal autoantibodies (ANNA) recognized clinically as markers of a patient's immune response to specific cancers (paraneoplastic autoantibodies).
In 1976, a PCA, defined by indirect immunofluorescence, was described by Dr. John Trotter and colleagues as a serological accompaniment of cerebellar ataxia related to Hodgkin lymphoma. That autoantibody is now known as anti-Tr or PCA-Tr.
PCA-1 (or anti-Yo), first described in 1983, serves as a serological marker for a new or recurrent carcinoma of the ovary, other Mullerian tissue, or breast. PCA-1-positive patients are women in 99% of cases. They usually present with subacute cerebellar degeneration, but 10% have sensory or motor neuropathy.
In 2000, the Mayo Clinic Neuroimmunology Laboratory described and named PCA-2, a new IgG marker of an immune response to small-cell lung carcinoma (SCLC) in patients presenting with a subacute paraneoplastic neurologic disorder.
Other autoantibody markers of immune responses to SCLC include ANNA-1, ANNA-2, ANNA-3, amphiphysin, collapsin response-mediated protein-5 (CRMP-5)-IgG, anti-glial/neuronal nuclear antibody-type 1 (AGNA-1), neuronal calcium channel antibodies (N-type > P/Q-type), ganglionic acetylcholine receptor antibodies, muscle acetylcholine receptor antibodies, neuronal potassium channel antibodies, and striational antibodies.
Reference Values
Only orderable as a reflex. For more information see:
-PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum
-DMS2 / Dementia, Autoimmune/Paraneoplastic Evaluation, Serum
-ENS2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
-EPS2 / Epilepsy, Autoimmune/Paraneoplastic Evaluation, Serum
-MDS2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum
-MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
-AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
-DYS2 / Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum
-GID2 / Gastrointestinal Dysmotility, Autoimmune/Paraneoplastic Evaluation, Serum
<1:240
Neuron-restricted patterns of IgG staining that do not fulfill criteria for Purkinje cell cytoplasmic antibody type 2 may be reported as "unclassified antineuronal IgG." Complex patterns that include non-neuronal elements may be reported as "uninterpretable."
Interpretation
A positive value (at 1:240 dilution or higher) is consistent with neurological autoimmunity and justifies a thorough search for a lung cancer, particularly small-cell carcinoma. The cancers are usually limited in metastasis. An extrapulmonary primary small-cell carcinoma (eg, skin, breast, larynx, cervix, prostate) should be considered.
Purkinje cell antibody type 2 is found in less than 2% of patients with uncomplicated small-cell lung carcinoma.
Cautions
Western blot with native neuronal proteins may be required to detect a positive result when interfering autoantibodies preclude interpretation of immunofluorescence pattern.
Supportive Data
Purkinje cell antibody type 2 (PCA-2) binds to the cytoplasm of cerebellar neurons in a characteristic pattern. Western blots of reduced/denatured cerebellar and small-cell lung carcinoma proteins reveal a common antigenic band, approximately 280 kDa.(1) Nine of 10 seropositive patients initially identified had a subacute neurological presentation (elements of encephalomyeloneuropathy), and 9 of 10 had lung cancer confirmed.(1) Similar neurological and oncological correlations have been observed in 104 subsequently identified seropositive patients.(VA Lennon, unpublished data)
Clinical Reference
1. Galanis E, Frytak S, Rowland KM, et al: Neuronal autoantibody titers in the course of small-cell lung carcinoma and platinum-associated neuropathy. Cancer Immunol Immunother. 1999 May-June;48(2-3):85-90
2. Vernino S, Lennon VA: New Purkinje cell antibody (PCA-2): marker of lung cancer-related neurological autoimmunity. Ann Neurol. 2000 Mar;47(3):297-305
3. McKeon A, Tracy JA, Pittock SJ, Parisi JE, Klein CJ, Lennon VA. Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond. Arch Neurol. 2011 Oct;68(10):1282-9. doi: 10.1001/archneurol.2011.128
4. Pittock SJ, Kryzer TJ, Lennon VA: Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol. 2004 Nov;56(5):715-719
Method Description
The patient's sample is tested by a standardized immunofluorescence assay that uses a composite frozen section of mouse cerebellum, kidney, and gut tissues. After incubation with sample and washing, fluorescein-conjugated goat-antihuman IgG is applied. Neuron-specific autoantibodies are identified by their characteristic fluorescence staining patterns. Samples that are scored positive for any neuronal nuclear or cytoplasmic autoantibody are titrated to an endpoint. Interference by coexisting non-neuron-specific autoantibodies can usually be eliminated by serologic absorption.(Honorat JA, Komorowski L, Josephs KA, et al: IgLON5 antibody: neurological accompaniments and outcomes in 20 patients. Neurol Neuroimmunol Neuroinflamm 2017 Jul 18;4(5):e385. doi: 10.1212/NXI.0000000000000385)
Day(s) Performed
Monday through Sunday
Report Available
5 to 8 daysSpecimen Retention Time
28 daysPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
86256
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| PC2TS | PCA-2 Titer, S | 94351-4 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 43438 | PCA-2 Titer, S | 94351-4 |