What is ICD-10 code D58.0?

D58.0 is the ICD-10-CM diagnosis code for Hereditary spherocytosis. A genetic blood disorder where red blood cells are abnormally shaped like spheres, making them fragile and prone to breaking down prematurely, leading to anemia. D58.0 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. D58.0 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. D58.0 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. D58.0 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

Which HCC does D58.0 map to under V28?

D58.0 does not map to a CMS-HCC V28 payment category. Check the current CMS-HCC V28 model file for payment year 2026 rather than an older V24 tip sheet.

What does D58.0 pay in RAF?

D58.0 has no separately stated V28 community RAF weight. If it is not a V28 payment HCC, it adds nothing to a community RAF score.

What is the V28 vs V24 difference for D58.0?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. D58.0 was a payment HCC under V24 (Coagulation Defects and Other Specified Hematological Disorders) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

2026 ICD-10-CM D58.0: Hereditary spherocytosis

D58.0 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC coding software

ICD-10-CM Code View

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Code lookupD58.0Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Hemolytic anemias (D55-D59)

D58.0

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Hereditary spherocytosis

A genetic blood disorder where red blood cells are abnormally shaped like spheres, making them fragile and prone to breaking down prematurely, leading to anemia.

Buddy Insight

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 48

Coagulation Defects and Other...

RAF 0.192

ACA/HHS

N/A

—

Not risk-adjusted for this model/period.

Not mapped

ESRD/PACE

Mapped

HCC 48

Coagulation Defects and Other...

RAF 0.063

RXHCC

N/A

—

Not risk-adjusted for this model/period.

Not mapped

Code Book Path

Official

D58Other hereditary hemolytic anemias

D58.0Hereditary spherocytosis

Inclusion Terms

Official

Acholuric (familial) jaundice
Congenital (spherocytic) hemolytic icterus
Minkowski-Chauffard syndrome

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D58.0 in this effective period.

Related Child Codes

Official

D58.1Hereditary elliptocytosis

D58.2Other hemoglobinopathies

D58.8Other specified hereditary hemolytic anemias

D58.9Hereditary hemolytic anemia, unspecified

Includes

Official

ICD-10-CM does not list Includes notes for D58.0 in this effective period.

Excludes 1

Official

hemolytic anemia of the newborn (P55.-)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for D58.0 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D58.0 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D58.0 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Document the hereditary nature of the condition with family history when available.

Record peripheral blood smear findings showing spherocytes and osmotic fragility test results.

Include hemoglobin level, reticulocyte count, indirect bilirubin, lactate dehydrogenase, and haptoglobin values.

Document disease severity (mild, moderate, or severe), spleen size, history of gallstones, and whether splenectomy has been performed or is planned.

MEAT Support

HCC Buddy guidance

Document the hereditary nature of the condition with family history when available.

Record peripheral blood smear findings showing spherocytes and osmotic fragility test results.

Include hemoglobin level, reticulocyte count, indirect bilirubin, lactate dehydrogenase, and haptoglobin values.

Document disease severity (mild, moderate, or severe), spleen size, history of gallstones, and whether splenectomy has been performed or is planned.

Audit Caution

HCC Buddy guidance

Do not confuse hereditary spherocytosis with autoimmune hemolytic anemia, which also shows spherocytes on smear -

the direct antiglobulin (Coombs) test distinguishes them. Ensure the condition is documented as hereditary rather than acquired. Code any associated complications separately, including cholelithiasis (K80.x) or splenomegaly (R16.1) when present.

Common Mistakes

HCC Buddy guidance

D59.10-D59.19 (Autoimmune hemolytic anemia) can produce spherocytes on blood smear but is acquired, not hereditary -

differentiate with direct antiglobulin test (positive in autoimmune, negative in hereditary spherocytosis). D58.8 (Other specified hereditary hemolytic anemias) should not be used when hereditary spherocytosis is specifically documented. D58.9 (Hereditary hemolytic anemia, unspecified) is less specific and should be avoided when spherocytosis is confirmed.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is D58.0 an HCC code?

Yes. D58.0 maps to Coagulation Defects and Other Specified Hematological Disorders under the V24 model but is not retained in V28.

Code: D58.0
Description: Hereditary spherocytosis
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 48, Coagulation Defects and Other Specified Hematological Disorders

0.192

ESRDHCC 48, Coagulation Defects and Other Specified Hematological Disorders

0.063

Each model's RAF is its CMS base weight for that model's standard population, so weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

Work D58.0 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for D58.0

For D58.0 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
EEvaluate: test results, medication response, or physical findings reviewed by the provider
AAssess: explicit mention in the assessment or plan with acknowledgment of status
TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed D58.0 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

D58.0 is the ICD-10-CM diagnosis code for hereditary spherocytosis. A genetic blood disorder where red blood cells are abnormally shaped like spheres, making them fragile and prone to breaking down prematurely, leading to anemia. D58.0 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering hemolytic anemias (d55-d59).

Under the older CMS-HCC V24 model, D58.0 maps to Coagulation Defects and Other Specified Hematological Disorders (HCC 48) with a community, non-dual, aged base RAF weight of 0.192. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

This is an inherited condition; document family history when available. Because D58.0 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for D58.0 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•This is an inherited condition; document family history when available
•Specify if complications like gallstones or spleen enlargement are present

Clinical Significance

Hereditary spherocytosis is the most common inherited hemolytic anemia in people of Northern European descent, caused by defects in red blood cell membrane proteins (spectrin, ankyrin, band 3, or protein 4.2) that result in spherically shaped, fragile red blood cells. These abnormal cells are selectively trapped and destroyed in the spleen, leading to chronic hemolytic anemia of variable severity. Complications include gallstone formation from chronic bilirubin excess, aplastic crises from parvovirus B19 infection, and splenomegaly.

Documentation Requirements

✓Document the hereditary nature of the condition with family history when available.
✓Record peripheral blood smear findings showing spherocytes and osmotic fragility test results.
✓Include hemoglobin level, reticulocyte count, indirect bilirubin, lactate dehydrogenase, and haptoglobin values.
✓Document disease severity (mild, moderate, or severe), spleen size, history of gallstones, and whether splenectomy has been performed or is planned.

Commonly Confused Codes

•D59.10-D59.19 (Autoimmune hemolytic anemia) can produce spherocytes on blood smear but is acquired, not hereditary -
•differentiate with direct antiglobulin test (positive in autoimmune, negative in hereditary spherocytosis). D58.8 (Other specified hereditary hemolytic anemias) should not be used when hereditary spherocytosis is specifically documented. D58.9 (Hereditary hemolytic anemia, unspecified) is less specific and should be avoided when spherocytosis is confirmed.