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HelpTest Code KLHCS Kelch-Like Protein 11 Antibody, Cell Binding Assay, Serum
Specimen Required
Patient Preparation: For optimal antibody detection, specimen collection is recommended before starting immunosuppressant medication or intravenous immunoglobulin (IVIg) treatment.
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Collection Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Submission Container/Tube: Plastic vial
Specimen Volume: 1 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
Secondary ID
622117Useful For
Positivity for Kelch-like protein 11 (KLHL11)-IgG is indicative of a paraneoplastic neurological syndrome. Positivity indicates a high likelihood of finding a testicular cancer. A rigorous search for cancer should be initiated after KLHL11 autoimmunity is confirmed.
Reflex Tests
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| K11TS | KLHL11 Ab IFA Titer, S | No | No |
Testing Algorithm
If the cell binding antibody result is reactive, then the immunofluorescence titer assay will be performed at an additional charge.
Method Name
Cell-Binding Assay (CBA)
Reporting Name
KLHL11-IgG CBA, SSpecimen Type
SerumSpecimen Minimum Volume
0.5 mL
Specimen Stability Information
| Specimen Type | Temperature | Time |
|---|---|---|
| Serum | Refrigerated (preferred) | 28 days |
| Frozen | 28 days | |
| Ambient | 72 hours |
Reject Due To
| Gross hemolysis | Reject |
| Gross lipemia | Reject |
| Gross icterus | Reject |
Clinical Information
Kelch-like protein 11 (KLHL11 or Kelch-like family member 11) IgG is a biomarker of paraneoplastic encephalitis, KLHL11 encephalitis is a unique paraneoplastic syndrome commonly associated with testicular germ cell tumors, mainly seminoma. Ataxia, diplopia, dysarthria, and vertigo are common presenting features of the rhombencephalitis phenotype. Hearing loss and tinnitus may precede other neurological signs and symptoms by weeks to months. A subset of patients also has clinical and magnetic resonance imaging (MRI) presentations consistent with limbic encephalitis. Most patients with this syndrome have inflammatory spinal fluid profiles, especially elevated oligoclonal bands. MRI of the brain demonstrates T2 fluid attenuated inversion recovery (T2/FLAIR) abnormalities involving the brainstem or limbic system. The accompanying neurological disorder is usually severe. Clinical improvement following treatment of cancer or immunotherapy has been reported.
Reference Values
Negative
Interpretation
Evaluating patients with paraneoplastic or autoimmune encephalitis (brainstem encephalitis or limbic encephalitis or cerebellar ataxia) using serum specimens.
Cautions
A negative Kelch-like protein 11 (KLHL11) antibody test result does not exclude autoimmune neurological disease or cancer.
Clinical Reference
1. Mandel-Brehm C, Dubey D, Kryzer TJ, et al. Kelch-like protein 11 antibodies in seminoma-associated paraneoplastic encephalitis. N Engl J Med. 2019;381:47-54
2. Dubey D, Wilson MR, Clarkson B, et al. Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic Kelch-like protein-11 encephalitis. JAMA Neurol. 2020;77(11):1-10
Method Description
Patient sample is applied to a composite slide containing transfected and nontransfected EU90 cells. After incubation and washing, fluorescein-conjugated goat-antihuman IgG is applied to detect the presence of patient IgG binding.(Package insert: EUROIMMUN; FA_110a-50_A_UK_B01, 12/2023)
Day(s) Performed
Monday through Sunday
Report Available
3 to 5 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
0432U
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| KLHCS | KLHL11-IgG CBA, S | 99072-1 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 622117 | KLHL11-IgG CBA, S | 99072-1 |