ICD-10 code for XXXXY syndrome

The ICD-10-CM code for XXXXY syndrome is Q98.1 (Klinefelter syndrome, male with more than two X chromosomes).

Within Q98 (other sex chromosome abnormalities, male phenotype, not elsewhere classified), Q98.1 is specifically for Klinefelter syndrome, male with more than two X chromosomes. Related codes cover Klinefelter syndrome karyotype 47, XXY (Q98.0), other male with 46, XX karyotype (Q98.3), Klinefelter syndrome, unspecified (Q98.4), karyotype 47, XYY (Q98.5), male with structurally abnormal sex chromosome (Q98.6), male with sex chromosome mosaicism (Q98.7), other specified sex chromosome abnormalities, male phenotype (Q98.8) and sex chromosome abnormality, male phenotype, unspecified (Q98.9).

It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.

In MS-DRG v44.0, it is part of the grouping logic for DRG 729 (Other Male Reproductive System Diagnoses with CC/MCC, relative weight 1.0544) and DRG 730 (Other Male Reproductive System Diagnoses without CC/MCC, relative weight 0.6597), in MDC 12 (Diseases and Disorders of the Male Reproductive System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.