D70.0
BillableCongenital agranulocytosis
HCC Category Mapping
V28HCC 115 — Disorders of Immunity, Neutropenia
0.369V24HCC 47 — Disorders of Immunity
0.472ESRDHCC 47 — Disorders of Immunity
0.000What This Code Means
A rare inherited condition where a person is born with an inability to produce certain white blood cells called neutrophils.
Coding Tips
- •This is a congenital condition; verify the diagnosis is present from birth and not acquired later
- •Document family history when available as this is a genetic disorder
Clinical Significance
Congenital agranulocytosis, also known as Kostmann syndrome or severe congenital neutropenia, is a rare inherited bone marrow failure disorder characterized by severely reduced neutrophil counts from birth, leading to life-threatening bacterial infections. Patients require lifelong granulocyte colony-stimulating factor (G-CSF) therapy and have an increased risk of developing myelodysplastic syndrome or acute myeloid leukemia.
Documentation Requirements
- ✓Document absolute neutrophil count (typically <200/mcL), age of onset (infancy), genetic mutation if identified (HAX1, ELANE, G6PC3), G-CSF dosing and response, and infection history.
- ✓Record bone marrow biopsy findings showing maturation arrest at promyelocyte/myelocyte stage and screening for malignant transformation.
Commonly Confused Codes
D70.4 (Cyclic neutropenia) — periodic rather than constant neutropenia, also inheritedD70.8 (Other neutropenia) — acquired formsD70.9 (Neutropenia, unspecified) — should not be used when congenital agranulocytosis is documentedD71 (Functional disorders of polymorphonuclear neutrophils) — qualitative rather than quantitative defect.