Procedure Codes and Description
81381 HLA CLASS I TYPING, HIGH RESOLUTION (IE, ALLELES OR ALLELE GROUPS); ONE ALLELE OR ALLELE GROUP (EG, B*57:01P), EACH
81383 HLA CLASS II TYPING, HIGH RESOLUTION (IE, ALLELES OR ALLELE GROUPS); ONE ALLELE OR ALLELE GROUP (EG, HLA-DQB1*06:02P), EACH
Coverage Indications, Limitations, and/or Medical Necessity
Indications and Limitations of Coverage
This policy provides limited coverage for HLA-B*15:02 genotype testing when the following criteria are met:
Patient is of Asian and Oceanian ancestry; AND
Initial treatment with carbamazepine, phenytoin or fosphenytoin is planned
Background
In 2004, researchers reported individuals with the HLA-B*1502 had an increased risk to develop Stevens-Johnson syndrome (SIS) or toxic epidermal necrolysis (TEN) when exposed to carbamazepine2. SJS and TEN, considered two variants of a disease continuum, are severe, sometimes lethal diseases of the skin and mucous membranes. A third, intermediate condition is called SJS/TEN. The most serious cases result in separation of the epidermis from the dermis in large sheets, which can also lead to infection. Sloughing can also occur in the bronchial, gastrointestinal and ocular epithelia.
Estimates indicate 10-15% of the population from China, Thailand, Malaysia, Indonesia, the Philippines, and Taiwan carry the HLA-B*1502 allele. South Asians, including Indians, appear to have an intermediate chance of having HLA-B*1502, averaging 2 to 4%, but it is higher in some subgroups. Oceanians also have an increased incidence of HLA-B *1502 serotype. The incidence of the HLA-B*1502 serotype in the European Caucasian population has been reported at less than 0.1% 1, in the African population as 0.2% and in the Native American and Hispanic populations as 0% 3.
In 2007, the FDA issued a black box label warning for carbamazepine stating, “Patients with ancestry in genetically at-risk populations should be screened for the presence of HLA-B*1502 prior to initiating treatment.” More recent evidence has supported the FDA recommendations 4 and at least one study has demonstrated that prospective screening of HLA-B*1502 has reduced the incidence of SJS/TEN in a Chinese population 5.
Bill Type Codes:
Contractors may specify Bill Types to help providers identify those Bill Types typically used to report this service. Absence of a Bill Type does not guarantee that the policy does not apply to that Bill Type. Complete absence of all Bill Types indicates that coverage is not influenced by Bill Type and the policy should be assumed to apply equally to all claims.
999x Not Applicable
Revenue Codes:
Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory. Unless specified in the policy, services reported under other Revenue Codes are equally subject to this coverage determination. Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the policy should be assumed to apply equally to all Revenue Codes.
N/A
Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy (sudden loss of voluntary muscle tone), and uncontrollable sleep episodes. Most cases of narcolepsy are sporadic, with symptoms beginning around the time of adolescence.
According to the International Classification of Sleep Disorders, Third Edition (ICSD-3) and the Diagnostic and Statistical Manual of Mental Disorder, Fifth Edition (DSM-5), narcolepsy is diagnosed by a combination of physical exam, medical history, polysomnogram, multiple sleep latency testing (MSLT), and low CSF hypocretin-1 levels. Current recommended treatment options include stimulants and antidepressants. At this time, treatment is aimed towards the control of symptoms and is not curative.12
Narcolepsy has a multifactorial etiology, likely caused by the interaction between genetic risk factors and environmental exposures. Research efforts to identify the genetic contributors to narcolepsy have focused on an association between certain human leukocyte antigen (HLA) haplotypes and narcolepsy risk. The HLA complex encodes greater than 200 genes responsible for the recognition of foreign antigens. These genes are highly polymorphic, and certain alleles have long been known to confer risk for autoimmune disorders.
A variation of the HLA-DQB1 gene called HLA-DQB1*06:02 has been strongly associated with narcolepsy, particularly in individuals who also have cataplexy and a loss of hypocretins. Several genetic association studies in ethnically diverse populations have found a robust association between narcolepsy and the HLA-DQB1*06:02 allele. However, 15 to 25% of unaffected individuals in the general population also carry this risk haplotype, suggesting that it is necessary but not sufficient for the development of narcolepsy.6 Additionally, persons with narcolepsy and cataplexy have been identified without the HLA-DQB1*06:02 marker.4 More recent studies further suggest that predisposition to narcolepsy may be the result of complex genetic associations between multiple risk alleles.11
Despite multiple studies replicating the association between HLA-DQB1*06:02 and narcolepsy in different ethnic groups, the overall contribution of HLA variation to disease risk is low. Monozygotic twin studies have shown only partial concordance (25-31%), indicating that environmental factors play a large role in the etiology of narcolepsy.8 Recent studies have suggested that exposure to streptococcus, H1N1, and the H1N1 vaccine may also increase the risk for narcolepsy, specifically among individuals with the HLA-DQB1*06:02 allele.3,14,4
Although research suggests a strong association between HLA-DQB1*06:02 and narcolepsy risk, at this time there is no evidence for any diagnostic utility of HLA typing.5
ICD-10 Codes that Support Medical Necessity
ICD-10 CODE DESCRIPTION
B02.22 Postherpetic trigeminal neuralgia
F31.0 Bipolar disorder, current episode hypomanic
F31.10 Bipolar disorder, current episode manic without psychotic features, unspecified
F31.11 Bipolar disorder, current episode manic without psychotic features, mild
F31.12 Bipolar disorder, current episode manic without psychotic features, moderate
F31.13 Bipolar disorder, current episode manic without psychotic features, severe
F31.2 Bipolar disorder, current episode manic severe with psychotic features
F31.30 Bipolar disorder, current episode depressed, mild or moderate severity, unspecified
F31.31 Bipolar disorder, current episode depressed, mild
F31.32 Bipolar disorder, current episode depressed, moderate
F31.4 Bipolar disorder, current episode depressed, severe, without psychotic features
F31.5 Bipolar disorder, current episode depressed, severe, with psychotic features
F31.60 Bipolar disorder, current episode mixed, unspecified
F31.61 Bipolar disorder, current episode mixed, mild
F31.62 Bipolar disorder, current episode mixed, moderate
F31.63 Bipolar disorder, current episode mixed, severe, without psychotic features
F31.64 Bipolar disorder, current episode mixed, severe, with psychotic features
F31.70 Bipolar disorder, currently in remission, most recent episode unspecified
F31.71 Bipolar disorder, in partial remission, most recent episode hypomanic
F31.72 Bipolar disorder, in full remission, most recent episode hypomanic
F31.73 Bipolar disorder, in partial remission, most recent episode manic
F31.74 Bipolar disorder, in full remission, most recent episode manic
F31.75 Bipolar disorder, in partial remission, most recent episode depressed
F31.76 Bipolar disorder, in full remission, most recent episode depressed
F31.77 Bipolar disorder, in partial remission, most recent episode mixed
F31.78 Bipolar disorder, in full remission, most recent episode mixed
F31.81 Bipolar II disorder
F31.89 Other bipolar disorder
F31.9 Bipolar disorder, unspecified
G40.001 Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset, not intractable, with status epilepticus
G40.009 Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset, not intractable, without status epilepticus
G40.011 Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset, intractable, with status epilepticus
G40.019 Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset, intractable, without status epilepticus
G40.101 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures, not intractable, with status epilepticus
G40.109 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures, not intractable, without status epilepticus
G40.111 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures, intractable, with status epilepticus
G40.119 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures, intractable, without status epilepticus
G40.201 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures, not intractable, with status epilepticus
G40.209 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures, not intractable, without status epilepticus
G40.211 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures, intractable, with status epilepticus
G40.219 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures, intractable, without status epilepticus
G40.301 Generalized idiopathic epilepsy and epileptic syndromes, not intractable, with status epilepticus
G40.309 Generalized idiopathic epilepsy and epileptic syndromes, not intractable, without status epilepticus
G40.311 Generalized idiopathic epilepsy and epileptic syndromes, intractable, with status epilepticus
G40.319 Generalized idiopathic epilepsy and epileptic syndromes, intractable, without status epilepticus
G40.401 Other generalized epilepsy and epileptic syndromes, not intractable, with status epilepticus
G40.409 Other generalized epilepsy and epileptic syndromes, not intractable, without status epilepticus
G40.411 Other generalized epilepsy and epileptic syndromes, intractable, with status epilepticus
G40.419 Other generalized epilepsy and epileptic syndromes, intractable, without status epilepticus
G40.501 Epileptic seizures related to external causes, not intractable, with status epilepticus
G40.509 Epileptic seizures related to external causes, not intractable, without status epilepticus
G40.801 Other epilepsy, not intractable, with status epilepticus
G40.802 Other epilepsy, not intractable, without status epilepticus
G40.803 Other epilepsy, intractable, with status epilepticus
G40.804 Other epilepsy, intractable, without status epilepticus
G40.811 Lennox-Gastaut syndrome, not intractable, with status epilepticus
G40.812 Lennox-Gastaut syndrome, not intractable, without status epilepticus
G40.813 Lennox-Gastaut syndrome, intractable, with status epilepticus
G40.814 Lennox-Gastaut syndrome, intractable, without status epilepticus
G40.821 Epileptic spasms, not intractable, with status epilepticus
G40.822 Epileptic spasms, not intractable, without status epilepticus
G40.823 Epileptic spasms, intractable, with status epilepticus
G40.824 Epileptic spasms, intractable, without status epilepticus
G40.89 Other seizures
G40.901 Epilepsy, unspecified, not intractable, with status epilepticus
G40.909 Epilepsy, unspecified, not intractable, without status epilepticus
G40.911 Epilepsy, unspecified, intractable, with status epilepticus
G40.919 Epilepsy, unspecified, intractable, without status epilepticus
G50.0 Trigeminal neuralgia
G52.1 Disorders of glossopharyngeal nerve
Z17.0 Estrogen receptor positive status [ER+]
Z94.0 Kidney transplant status
Z94.1 Heart transplant status
Z94.2 Lung transplant status
Z94.3 Heart and lungs transplant status
Z94.4 Liver transplant status
Z94.81 Bone marrow transplant status
Z94.82 Intestine transplant status
Z94.83 Pancreas transplant status
Z94.84 Stem cells transplant status
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