2027 ICD-10-CM Diagnosis Code D61.02Shwachman-Diamond syndrome

ICD-10-CM Codes›D50–D89›D60-D64›D61

ICD-10-CM D61.02
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

D61.02 is a billable ICD-10-CM diagnosis code for Shwachman-Diamond syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 808 through 810. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 101 closely related codes. Coders also document this condition as pancreatic insufficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Aplastic anemia.

For Medicare Advantage risk adjustment, D61.02 maps to CMS-HCC Category 109 (Acquired Hemolytic, Aplastic, and Sideroblastic Anemias) under the V28 model, adding a risk factor of about 1.144 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
D61.02
Billable Status
Yes — Valid for Submission
Code Describes
Shwachman-Diamond syndrome
Short Description
Shwachman-Diamond syndrome
Same as the full description in the CMS dataset.
Parent Code
D61.0
Constitutional aplastic anemia

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD60-D64Aplastic and other anemias and other bone marrow failure syndromes
CategoryD61Other aplastic anemias and other bone marrow failure syndromes
This CodeD61.02Shwachman-Diamond syndrome

Medicare Risk Adjustment (HCC)Billing

D61.02 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 109— Acquired Hemolytic, Aplastic, and Sideroblastic Anemias
Payment HCC · PY 2027 one of 37 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+1.144
community, non-dual, aged · ranges 0.529–1.815 across segments
Hierarchy
Top of its hierarchy
HCC 109 is not superseded by any other condition category
Prior Model (CMS-HCC V24)
HCC 46
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
PACE (CMS-HCC V22): HCC 46 · ESRD (V21): HCC 46 · ESRD (V24): HCC 46
ESRD V21 weights: 0.180 dialysis, 0.766–1.325 functioning graft · ESRD V24 weights: 0.223 dialysis, 0.748–4.064 functioning graft
Part D (RxHCC)
RxHCC 96 — Acquired Hemolytic, Aplastic, and Sideroblastic Anemias
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Pancreatic insufficiency
  • Shwachman syndrome

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to D61.02: its own notes plus those printed at D61 and Chapter 3. 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.

From D61 Other aplastic anemias and other bone marrow failure syndromes applies to 13 codes
From Chapter 3 (D50-D89) Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism applies to 324 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • certain conditions originating in the perinatal period (P00-P96)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • human immunodeficiency virus [HIV] disease (B20)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

  • code, if applicable, for genetic susceptibility to other malignant neoplasm (Z15.09)

Code Also

A second code may be needed to fully describe the condition; the note gives no sequencing direction.

  • if applicable, associated conditions such as:
  • acute myeloblastic leukemia (C92.0-)
  • exocrine pancreatic insufficiency (K86.81)
  • myelodysplastic syndrome (D46.-)

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD003
Aplastic anemia
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Shwachman-Diamond Syndrome

    an inherited syndrome characterized by exocrine pancreatic insufficiency; hematologic abnormalities (e.g., bone marrow hypoplasia), and skeletal abnormalities (e.g., metaphyseal chondroplasia). germline mutations in the sbds gene are associated with shwachman-diamond syndrome.

Code History & ChangesHistory

Replacement D61.02 replaces the following previously assigned code(s):

  • D61.09 - Other constitutional aplastic anemia
FY 2024AddedAdded to the ICD-10-CM code setEffective October 1, 2023.
FY 2025–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About D61.02Overview

What is the ICD-10 code for Shwachman-Diamond syndrome?

The ICD-10-CM code for Shwachman-Diamond syndrome is D61.02 (sometimes written as D6102). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is D61.02 (Constitutional aplastic anemia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Shwachman-Diamond syndrome on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does D61.02 group to?

When Shwachman-Diamond syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 808, 809, 810, with relative weights from 1.0749 to 2.1706 depending on complications. Higher weights mean higher Medicare reimbursement.

Is D61.02 a CC or MCC?

CMS lists D61.02 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 101 closely related codes in its exclusion list.

Does D61.02 need an additional code?

When the condition applies, yes. The Use Additional Code note reads: code, if applicable, for genetic susceptibility to other malignant neoplasm (Z15.09). The additional code is reported after D61.02.

What HCC is D61.02?

D61.02 (Shwachman-Diamond syndrome) maps to CMS-HCC Category 109 (Acquired Hemolytic, Aplastic, and Sideroblastic Anemias), commonly written as HCC 109, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 46 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 96.

Does D61.02 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, D61.02 adds a risk adjustment factor of about 1.144 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.529 to 1.815 depending on the payment segment). HCC 109 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 109 category page.