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D57.434

Billable

Sickle-cell thalassemia beta zero with dactylitis

HCC Category Mapping

V28HCC 107Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero
0.727
V24HCC 46Severe Hematological Disorders
0.666
ESRDHCC 46Severe Hematological Disorders
0.000
RxHCCHCC 95Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero
0.000

What This Code Means

A combination blood disorder with sickle-cell and thalassemia beta-zero causing dactylitis, which is painful swelling of the hands and feet due to bone infarction.

Coding Tips

  • Dactylitis is often called 'hand-foot syndrome'; ensure documentation specifies which extremities are affected and confirms the diagnosis
  • This complication can occur with or without other crisis symptoms; code accordingly based on whether a broader crisis is also present

Clinical Significance

Dactylitis in sickle-cell thalassemia beta zero presents with the same vaso-occlusive hand and foot swelling seen in Hemoglobin SS disease, typically in infants and young children. Because beta-zero sickle-cell thalassemia has a clinical phenotype indistinguishable from Hemoglobin SS, dactylitis may be equally severe and serve as an early predictor of a severe disease course requiring aggressive management.

Documentation Requirements

  • Documentation must confirm sickle-cell thalassemia beta zero genotype and the diagnosis of dactylitis with identification of affected extremities, swelling, and tenderness.
  • Record pain assessment, any imaging findings, treatment administered, and clinical course.
  • Note the patient's age and whether this presentation contributed to the initial sickle cell diagnosis.

Commonly Confused Codes

Code Hierarchy

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