2026 ICD-10-CM Diagnosis Code D57.01Hb-SS disease with acute chest syndrome
ICD-10-CM Codes›D50–D89›D55-D59›D57
- Billable — Valid for Submission
- MCC — Major Complication or Comorbidity
- Risk Adjusts — HCC 107
- Chronic Condition
D57.01 is a billable ICD-10-CM diagnosis code for Hb-SS disease with acute chest syndrome. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 811 through 812. As a secondary diagnosis, it counts as a major complication or comorbidity (MCC) and places an inpatient stay in the highest severity level of its MS-DRG family. It does not count, however, when the principal diagnosis is one of 125 closely related codes. Coders also document this condition as acute chest syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Sickle cell trait/anemia.
For Medicare Advantage risk adjustment, D57.01 maps to CMS-HCC Category 107 (Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero) under the V28 model, adding a risk factor of about 0.457 for a community, non-dual, aged beneficiary in payment year 2026.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
D57.01 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 2026.
Source: CMS Payment Year 2026 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.
- Acute chest syndrome
- Sequelae of hereditary diseases
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
sickle-cell See Also: Disease, sickle-cell, by type, with crisis; D57.00
acute chest syndrome D57.01
Disease, diseased See Also: Syndrome;
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Acute Chest Syndrome
respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or dyspnea, often seen in patients with sickle cell anemia. multiple factors (e.g., infection, and pulmonary fat embolism) may contribute to the development of the syndrome.
Patient EducationClinical
Sickle Cell Disease
Sickle cell disease (SCD) is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells.
The full article covers:
- What is sickle cell disease (SCD)?
- What causes sickle cell disease (SCD)?
- Who is more likely to have sickle cell disease (SCD)?
- What are the symptoms of sickle cell disease (SCD)?
- How is sickle cell disease (SCD) diagnosed?
- What are the treatments for sickle cell disease (SCD)?
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert D57.01 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About D57.01Overview
What is the ICD-10 code for Hb-SS disease with acute chest syndrome?
The ICD-10-CM code for Hb-SS disease with acute chest syndrome is D57.01 (sometimes written as D5701). It is billable on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
Is D57.01 (Hb-SS disease with crisis) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report Hb-SS disease with acute chest syndrome on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
What MS-DRG does D57.01 group to?
When Hb-SS disease with acute chest syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 811, 812, with relative weights from 0.9182 to 1.4043 depending on complications. Higher weights mean higher Medicare reimbursement.
Is D57.01 a CC or MCC?
CMS lists D57.01 as an MCC (major complication or comorbidity) for FY 2026. Reported as a secondary diagnosis, it places the inpatient stay in the highest-weighted DRG of its severity family. It does not count when the principal diagnosis is one of the 125 closely related codes in its exclusion list.
What is the ICD-9 equivalent of D57.01?
Under the General Equivalence Mappings, Hb-SS disease with acute chest syndrome converts to ICD-9-CM 282.62 (Hb-SS disease w crisis) and 517.3 (acute chest syndrome). The mapping is approximate, so confirm the match fits the documentation.
What HCC is D57.01?
D57.01 (Hb-SS disease with acute chest syndrome) maps to CMS-HCC Category 107 (Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero), commonly written as HCC 107, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2026. 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 95.
Does D57.01 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, D57.01 adds a risk adjustment factor of about 0.457 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.303 to 1.939 depending on the payment segment). HCC 107 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 107 category page.