D57.42

Billable

Sickle-cell thalassemia beta zero without crisis

HCC Category Mapping

V28HCC 107 — Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero

0.727

V24HCC 46 — Severe Hematological Disorders

0.666

ESRDHCC 46 — Severe Hematological Disorders

0.000

RxHCCHCC 95 — Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero

0.000

What This Code Means

This is a genetic blood disorder where a person has both sickle cell disease and a severe form of thalassemia (a condition affecting hemoglobin production), but they are not currently experiencing a painful crisis or acute complication. The person's red blood cells are abnormally shaped and their body cannot produce normal hemoglobin.

Coding Tips

•Use this code only when the patient has confirmed sickle-cell thalassemia beta zero and is NOT in crisis; if crisis is present, use D57.41 instead
•This is a combination diagnosis code that captures both conditions simultaneously, so do not code sickle cell disease and thalassemia separately

Clinical Significance

Sickle-cell thalassemia beta zero without crisis represents the chronic baseline state of patients who co-inherit a sickle hemoglobin gene and a beta-zero thalassemia gene (producing no beta-globin from the thalassemia allele). This genotype produces a clinical phenotype virtually identical to Hemoglobin SS disease because all beta-globin produced is the sickle variant. These patients have severe chronic hemolytic anemia, frequent vaso-occlusive crises, and the same organ damage risk as Hemoglobin SS patients.

Documentation Requirements

✓Documentation must confirm sickle-cell thalassemia beta zero genotype through hemoglobin electrophoresis (showing predominantly Hemoglobin S with elevated Hemoglobin F and absent Hemoglobin A) or molecular genetic testing.
✓Confirm no acute crisis at the encounter.
✓Record baseline hemoglobin, reticulocyte count, medications (hydroxyurea dosing), transfusion schedule, and comprehensive organ surveillance results including echocardiogram, renal function, ophthalmologic exam, and transcranial Doppler results.

Commonly Confused Codes

D57.42 vs. D57.1 (Hemoglobin SS without crisis) -clinically similar but genetically distinct; hemoglobin electrophoresis can distinguish them. D57.42 vs. D57.44 (Beta plus without crisis) -beta-zero has more severe phenotype than beta-plus. D57.42 vs. D57.40 (Unspecified sickle-cell thalassemia without crisis) -D57.42 provides critical specificity for accurate HCC mapping.

Code Hierarchy

└D57Sickle-cell disorders └D57.4Sickle-cell thalassemia └D57.42Sickle-cell thalassemia beta zero without crisis

└D57.42Sickle-cell thalassemia beta zero without crisis