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D57.42 ICD-10-CM Code: Sickle-cell thalassemia beta zero without crisis

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
2 inclusion terms · 1 Excludes1 · 1 use-additional code
Risk adjustment
CMS-HCC V28: 1 category · CMS-HCC V22 category 46 · RxHCC V08 category 95

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.

  • MS-DRG 791 — PREMATURITY WITH MAJOR PROBLEMS (MDC 15)
  • MS-DRG 793 — FULL TERM NEONATE WITH MAJOR PROBLEMS (MDC 15)
  • MS-DRG 811 — RED BLOOD CELL DISORDERS WITH MCC (MDC 16)
  • MS-DRG 812 — RED BLOOD CELL DISORDERS WITHOUT MCC (MDC 16)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Risk Adjustment (CMS-HCC)

Payment categories this diagnosis maps to under CMS-HCC V28, payment year 2026.

  • HCC 107 — Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero (supersedes HCC 108)

Other models: CMS-HCC V22 HCC 46 · RxHCC V08 HCC 95

Risk scores depend on the enrollee's full accepted diagnosis set and segment; a category mapping alone does not determine payment.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for D57.42 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Notes without a marker are published on D57.42 itself; “inherited from” names the category or block whose note applies here.

Inclusion Terms

Alternative terms the tabular list files under this code.

  • HbS-beta zero without crisis
  • Sickle-cell beta zero without crisis

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Source: inherited from D57

Use Additional Code

Supplementary codes the tabular list directs you to add.

  • Use additional code for any associated fever (R50.81)

Source: inherited from D57

Coder workflow for D57.42

MedCoder structured workflow — derived from this code’s own official record

Before you code D57.42

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with D57.42. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support a condition named in D57.42’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewD58

Consider D57.42. Then work the Use Additional Code note, and confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
The associated condition or complication
Whether the associated condition the title names is documented; the “with” convention presumes some links, and a provider statement that the conditions are unrelated defeats it (Guidelines I.A.15).
The conditions the Use Additional Code note names
Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.

Official instructions as workflow

  • Excludes1 — check before selecting D57.42(1 note)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with D57.42: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareD58

    See the official tabular notes · Guidelines I.A.12.a

  • Use Additional Code — after identifying D57.42(1 note)

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with D57.42 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.

    ReviewR50.81

    See the official tabular notes · Guidelines I.A.13

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Both the condition D57.42 describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewD58

Documentation: A condition the Use Additional Code note names is documented.

Coding question: Is a second code reported with D57.42?

Path: Review the Use Additional Code note and the code it names.

Reason: The additional code is reported when the record documents the condition; a conditional instruction applies only when its condition is met (Guidelines I.A.13).

ReviewR50.81

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Code Overview

Sickle-cell thalassemia beta zero without crisis is a billable ICD-10-CM diagnosis code (D57.42).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Indexed Clinical Terms (4)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Official Coding Guidelines

No excerpt in the ICD-10-CM Official Guidelines names this code specifically. Its chapter carries only this chapter-wide note:

Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

Reserved for future guideline expansion

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

Other codes that name D57.42 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 18 Excludes1 notes across 3 chapters: D56 — Thalassemia (via D57.4.-), D56.1 — Beta thalassemia (via D57.4.-), D56.5 — Hemoglobin E-beta thalassemia (via D57.4.-), D56.8 — Other thalassemias (via D57.-), P50 — Newborn affected by intrauterine (fetal) blood loss (via D57.-), P50-P61 — Hemorrhagic and hematological disorders of newborn (P50-P61) (via D57.-), P51 — Umbilical hemorrhage of newborn (via D57.-), P52 — Intracranial nontraumatic hemorrhage of newborn (via D57.-), P53 — Hemorrhagic disease of newborn (via D57.-), P54 — Other neonatal hemorrhages (via D57.-), P55 — Hemolytic disease of newborn (via D57.-), P56 — Hydrops fetalis due to hemolytic disease (via D57.-), P57 — Kernicterus (via D57.-), P58 — Neonatal jaundice due to other excessive hemolysis (via D57.-), P59 — Neonatal jaundice from other and unspecified causes (via D57.-), P60 — Disseminated intravascular coagulation of newborn (via D57.-), P61 — Other perinatal hematological disorders (via D57.-), R71 — Abnormality of red blood cells (via D57.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Referenced by 27 Code First instructions across 6 chapters: H36 — Retinal disorders in diseases classified elsewhere (via D57.-), I21.A1 — Myocardial infarction type 2 (via D57.-), M14.8 — Arthropathies in other specified diseases classified elsewhere (via D57.-), M90.5 — Osteonecrosis in diseases classified elsewhere (via D57.-), N08 — Glomerular disorders in diseases classified elsewhere (via D57.-), R50.81 — Fever presenting with conditions classified elsewhere (via D57.-), T36 — Poisoning by, adverse effect of and underdosing of systemic antibiotics (via D57.-), T36-T39 — Antibiotics & Anti-infectives (T36-T39) (via D57.-), T36-T50 — Poisoning by, adverse effects of and underdosing of drugs, medicaments and biological substances (T36-T50) (via D57.-), T37 — Poisoning by, adverse effect of and underdosing of other systemic anti-infectives and antiparasitics (via D57.-), T38 — Poisoning by, adverse effect of and underdosing of hormones and their synthetic substitutes and antagonists, not elsewhere classified (via D57.-), T39 — Poisoning by, adverse effect of and underdosing of nonopioid analgesics, antipyretics and antirheumatics (via D57.-), T40 — Poisoning by, adverse effect of and underdosing of narcotics and psychodysleptics [hallucinogens] (via D57.-), T40-T41 — Analgesics & Antipyretics (T40-T41) (via D57.-), T41 — Poisoning by, adverse effect of and underdosing of anesthetics and therapeutic gases (via D57.-), T42 — Poisoning by, adverse effect of and underdosing of antiepileptic, sedative- hypnotic and antiparkinsonism drugs (via D57.-), T42-T43 — Anticonvulsants & Psychotropics (T42-T43) (via D57.-), T43 — Poisoning by, adverse effect of and underdosing of psychotropic drugs, not elsewhere classified (via D57.-), T44 — Poisoning by, adverse effect of and underdosing of drugs primarily affecting the autonomic nervous system (via D57.-), T44-T46 — Cardiovascular & Gastrointestinal (T44-T46) (via D57.-), +7 more.

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

Referenced by 1 Code Also instruction: Z71.87 — Encounter for pediatric-to-adult transition counseling (via D57.-).

These codes suggest coding this condition alongside when both are present.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.

Named in the grouper logic of 4 MS-DRGs: DRG 791 (MDC 15), DRG 793 (MDC 15), DRG 811 (MDC 16), DRG 812 (MDC 16).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):BLD005 — Sickle cell trait/anemia (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero) for risk-adjusted payment.

D57.00 — Hb-SS disease with crisis, unspecified, D57.01 — Hb-SS disease with acute chest syndrome, D57.02 — Hb-SS disease with splenic sequestration, D57.03 — Hb-SS disease with cerebral vascular involvement, D57.04 — Hb-SS disease with dactylitis, D57.09 — Hb-SS disease with crisis with other specified complication, D57.1 — Sickle-cell disease without crisis, D57.431 — Sickle-cell thalassemia beta zero with acute chest syndrome, D57.432 — Sickle-cell thalassemia beta zero with splenic sequestration, D57.433 — Sickle-cell thalassemia beta zero with cerebral vascular involvement, D57.434 — Sickle-cell thalassemia beta zero with dactylitis, D57.438 — Sickle-cell thalassemia beta zero with crisis with other specified complication, D57.439 — Sickle-cell thalassemia beta zero with crisis, unspecified

Related risk categories

These categories interact through CMS's HCC hierarchy — one can suppress the other's risk-adjustment weight when both are present on a claim.

Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Sickle cell trait/anemia).

D57.218 — Sickle-cell/Hb-C disease with crisis with other specified complication, D57.219 — Sickle-cell/Hb-C disease with crisis, unspecified, D57.3 — Sickle-cell trait, D57.40 — Sickle-cell thalassemia without crisis, D57.411 — Sickle-cell thalassemia, unspecified, with acute chest syndrome, D57.412 — Sickle-cell thalassemia, unspecified, with splenic sequestration, D57.413 — Sickle-cell thalassemia, unspecified, with cerebral vascular involvement, D57.414 — Sickle-cell thalassemia, unspecified, with dactylitis, D57.418 — Sickle-cell thalassemia, unspecified, with crisis with other specified complication, D57.419 — Sickle-cell thalassemia, unspecified, with crisis, D57.431 — Sickle-cell thalassemia beta zero with acute chest syndrome, D57.432 — Sickle-cell thalassemia beta zero with splenic sequestration, D57.433 — Sickle-cell thalassemia beta zero with cerebral vascular involvement, D57.434 — Sickle-cell thalassemia beta zero with dactylitis, D57.438 — Sickle-cell thalassemia beta zero with crisis with other specified complication, D57.439 — Sickle-cell thalassemia beta zero with crisis, unspecified, D57.44 — Sickle-cell thalassemia beta plus without crisis, D57.451 — Sickle-cell thalassemia beta plus with acute chest syndrome, D57.452 — Sickle-cell thalassemia beta plus with splenic sequestration, D57.453 — Sickle-cell thalassemia beta plus with cerebral vascular involvement, +30 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main term “Arthropathy”; these codes share that main term but sit in a different category of the Tabular List.

A04.6 — Enteritis due to Yersinia enterocolitica (postinfectious NEC, in, enteritis due to Yersinia enterocolitica), A50.55 — Late congenital syphilitic arthropathy (in, syphilis, congenital), A52.16 — Charcôt's arthropathy (tabetic) (tabetic), A52.77 — Syphilis of bone and joint (in, syphilis), B19.9 — Unspecified viral hepatitis without hepatic coma (in, viral hepatitis NEC), B99 — Other and unspecified infectious diseases (postinfectious NEC), C90.0 — Multiple myeloma (in, multiple myeloma), C95.9 — Leukemia, unspecified (in, leukemia NEC), C96.A — Histiocytic sarcoma (in, malignant histiocytosis), D49.9 — Neoplasm of unspecified behavior of unspecified site (in, neoplastic disease NOS), D58.2 — Other hemoglobinopathies (in, hemoglobinopathy NEC), D66 — Hereditary factor VIII deficiency (hemophilic NEC), D69.0 — Allergic purpura (in, Henoch-Schönlein purpura), D75.9 — Disease of blood and blood-forming organs, unspecified (in, blood disorder NOS), D86.86 — Sarcoid arthropathy (in, sarcoidosis), E03.9 — Hypothyroidism, unspecified (in, hypothyroidism), E05.90 — Thyrotoxicosis, unspecified without thyrotoxic crisis or storm (in, thyrotoxicosis), E21.3 — Hyperparathyroidism, unspecified (hemophilic NEC, in, hyperparathyroidism NEC), E22.0 — Acromegaly and pituitary gigantism (in, acromegaly), E34.9 — Endocrine disorder, unspecified (in, endocrine disease NOS), +112 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Iron Panel

Contextual Map

Every relationship of D57.42 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run D57.42 with these 17 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes (18)

Referenced by Code First instructions (27)

Referenced by Code Also instructions

Clinical classification (CCSR)

Risk adjustment (CMS-HCC)

  • HCC 107 — Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero [CMS-HCC]: “Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero — supersedes HCC 108 (Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major)”— CMS-HCC V28 · 2026

MS-DRG Grouper

MDC crossing

Index entries

  • Disease, diseased, hemoglobin or Hb, S or SS, beta zero[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Disease, diseased, hemoglobin or Hb, S or SS, beta zero, without crisis[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Disease, diseased, sickle-cell, thalassemia, beta zero[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Disease, diseased, sickle-cell, thalassemia, beta zero, without crisis[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (40)

Change history

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Coding guidelines Official source data
ICD-10-CM Official Guidelines for Coding and Reporting (FY2026), quoted by section Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page
Risk adjustment Official source data
2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder structured relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "D57.42 — Sickle-cell thalassemia beta zero without crisis." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/d57.42-sickle-cell-thalassemia-beta-zero-without-crisis

Change history

  • FY2021 — October 1, 2020
    Added to the code set
    Sickle-cell thalassemia beta zero without crisis
    FY2021 changes

Nearest Codes in This Family

Official ICD-10-CM classifications closest to D57.42 in its code family, with their registry titles.

View all codes in the D57 family