Excludes1 pair

D56.4 vs D58.2

D56.4 and D58.2 are not coded together. ICD-10-CM declares them mutually exclusive with an Excludes1 note — the two conditions are not expected to occur together, so exactly one of them is normally correct for a given encounter. The Official Guidelines carve out one exception: if the two conditions are clearly unrelated to each other, both may be reported.

The official note, declared on D58.2
hereditary persistence of fetal hemoglobin [HPFH] (D56.4)
D56.4

Hereditary persistence of fetal hemoglobin [HPFH]

Billable
Chapter 3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Section
D55-D59
Indexed under
  • Hemoglobin › fetal, hereditary persistence (HPFH)
  • Persistence, persistent › hemoglobin, fetal (hereditary) (HPFH)
  • Persistence, persistent › fetal › hemoglobin, hereditary (HPFH)
D58.2

Other hemoglobinopathies

Billable
Chapter 3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Section
D55-D59
Use when the record says
  • Abnormal hemoglobin NOS
  • Congenital Heinz body anemia
  • Hb-C disease
  • Hb-D disease
  • Hb-E disease
  • Hemoglobinopathy NOS
  • Unstable hemoglobin hemolytic disease
Indexed under
  • Heinz body anemia, congenital
  • Hemoglobinopathy (mixed)
  • Abnormal, abnormality, abnormalities › hemoglobin (disease)
  • Anemia › Heinz body, congenital
  • Hemoglobin › Constant Spring

Excludes1 is not always absolute: where two conditions covered by the note are genuinely unrelated, CMS guidance permits reporting both. Document the reasoning rather than assuming it. More on Excludes1 vs Excludes2 · FY2027