ICD-10-CM 2027
ICD-10 code for bare lymphocyte syndrome
Major histocompatibility complex class I deficiency
From the official ICD-10-CM alphabetic index entry “Syndrome › bare lymphocyte”. Page updated September 29, 2026.
About coding bare lymphocyte syndrome
The ICD-10-CM code for bare lymphocyte syndrome is D81.6 (Major histocompatibility complex class I deficiency).
Within D81 (combined immunodeficiencies), D81.6 is specifically for major histocompatibility complex class I deficiency. Related codes cover severe combined immunodeficiency [SCID] with reticular dysgenesis (D81.0), severe combined immunodeficiency [SCID] with low T- and B-cell numbers (D81.1), severe combined immunodeficiency [SCID] with low or normal B-cell numbers (D81.2), adenosine deaminase [ADA] deficiency (D81.3), Nezelof's syndrome (D81.4), purine nucleoside phosphorylase [PNP] deficiency (D81.5), major histocompatibility complex class II deficiency (D81.7), other combined immunodeficiencies (D81.8) and 1 more.
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 808 (Major Hematological and Immunological Diagnoses Except Sickle Cell Crisis and Coagulation Disorders with MCC, relative weight 2.1705), DRG 809 (Major Hematological and Immunological Diagnoses Except Sickle Cell Crisis and Coagulation Disorders with CC, relative weight 1.2262) and DRG 810 (Major Hematological and Immunological Diagnoses Except Sickle Cell Crisis and Coagulation Disorders without CC/MCC, relative weight 1.075), in MDC 16 (Diseases and Disorders of the Blood and Blood Forming Organs and Immunological Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about bare lymphocyte syndrome ICD-10 codes
What is the ICD-10 code for bare lymphocyte syndrome?
- D81.6 — Major histocompatibility complex class I deficiency.
Is D81.6 billable?
- Yes. D81.6 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can D81.6 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict D81.6 as a principal diagnosis.
Is D81.6 a CC or MCC?
- D81.6 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.