2027 ICD-10-CM Diagnosis Code Q98.0Klinefelter syndrome karyotype 47, XXY
ICD-10-CM Codes›Q00-Q99›Q90-Q99›Q98
- Billable — Valid for Submission
- POA Exempt
- Chronic Condition
Q98.0 is a billable ICD-10-CM diagnosis code for klinefelter syndrome karyotype 47, XXY. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 729 through 730. The code is exempt from POA reporting. Coders also document this condition as klinefelter syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Chromosomal abnormalities.
Code Identity
Code Classification
Present on Admission (POA)Billing
Q98.0 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Chromosomal abnormalities, not elsewhere classified (Q90-Q99).
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Klinefelter syndrome
- Klinefelter syndrome, male with 46,XX karyotype
- Klinefelter's syndrome, XXY
- Mosaic XY/XXY
- Sex chromosome mosaicism
- Sex phenotype-karyotype dissociation syndrome
- XX males
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to Q98.0: its own notes plus those printed at block Q90-Q99 and Chapter 17. A note printed at a category, block or chapter applies to every code under it.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- mitochondrial metabolic disorders (E88.4-)
Notes
General instructions on how these codes are used.
- Codes from this chapter are not for use on maternal records
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name Q98.0, its category, or a range that includes it.
Excludes1 5
These codes carry an Excludes1 note naming Q98.0: they are not reported together with it, unless the two conditions are unrelated.
- E29 Testicular dysfunction
- E25 Adrenogenital disorders
- Block Q50-Q56 Congenital malformations of genital organs
- Q56 Indeterminate sex and pseudohermaphroditism
- Z15 Genetic susceptibility to disease
Code First 1
These codes say to code first a condition named in the note, and Q98.0 is one of the conditions named.
- D84.81 Immunodeficiency due to conditions classified elsewhere
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
XXY syndrome Q98.0
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Klinefelter Syndrome
a form of male hypogonadism, characterized by the presence of an extra x chromosome, small testes, seminiferous tubule dysgenesis, elevated levels of gonadotropins, low serum testosterone, underdeveloped secondary sex characteristics, and male infertility (infertility, male). patients tend to have long legs and a slim, tall stature. gynecomastia is present in many of the patients. the classic form has the karyotype 47,xxy. several karyotype variants include 48,xxyy; 48,xxxy; 49,xxxxy, and mosaic patterns ( 46,xy/47,xxy; 47,xxy/48,xxxy, etc.).
Patient EducationClinical
Klinefelter Syndrome
Klinefelter syndrome (KS) is a genetic condition that happens when a male is born with an extra copy of the X chromosome. KS can affect different stages of physical, language, and social development. It also usually causes infertility.
The full article covers:
- What is Klinefelter syndrome (KS)?
- What causes Klinefelter syndrome (KS)?
- What are the symptoms of Klinefelter syndrome (KS)?
- How is Klinefelter syndrome (KS) diagnosed?
- What are the treatments for Klinefelter syndrome (KS)?
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert Q98.0 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About Q98.0Overview
What is the ICD-10 code for klinefelter syndrome karyotype 47, XXY?
The ICD-10-CM code for klinefelter syndrome karyotype 47, XXY is Q98.0 (sometimes written as Q980). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is Q98.0 a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report klinefelter syndrome karyotype 47, XXY on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does Q98.0 group to?
When klinefelter syndrome karyotype 47, XXY is the principal diagnosis on an inpatient stay, it groups to MS-DRG 729, 730, with relative weights from 0.6595 to 1.0553 depending on complications. Higher weights mean higher Medicare reimbursement.
Is Q98.0 exempt from POA reporting?
Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for klinefelter syndrome karyotype 47, XXY on inpatient claims. The code appears in the Chromosomal abnormalities, not elsewhere classified (Q90-Q99) range of the CMS exempt list.
What is the ICD-9 equivalent of Q98.0?
Under the General Equivalence Mappings, klinefelter syndrome karyotype 47, XXY converts to ICD-9-CM 758.7 (Klinefelter's syndrome). The mapping is approximate, so confirm the match fits the documentation.
