D70.4

Other specified neutropenia

D70.4 is a billable diagnosis for other specified neutropenia, a hematologic disorder characterized by an absolute neutrophil count (ANC) significantly lower than the standard reference range, typically below 1,500 cells per microliter. This specific classification excludes congenital agranulocytosis, drug-induced variants, and neutropenia resulting from acute infections. It primarily encompasses cyclic (periodic) neutropenia, a rare condition often caused by mutations in the ELANE gene, where neutrophil levels fluctuate in a predictable 21-day cycle. It also covers neutropenia associated with specific metabolic defects like glycogen storage disease type Ib or rare immune-mediated pathways. Patients with this condition are at a significantly increased risk for bacterial and fungal infections due to the impaired innate immune response. Clinical management often involves monitoring for febrile episodes and the therapeutic use of Granulocyte Colony-Stimulating Factor (G-CSF) to elevate the ANC during periods of severe depletion.

Clinical Symptoms

  • Recurrent fever and chills
  • Mouth ulcers (stomatitis)
  • Severe gingivitis or gum swelling
  • Frequent pharyngitis and sore throat
  • Recurrent skin infections and cellulitis
  • Perianal abscesses
  • Chronic fatigue and malaise
  • Sinusitis
  • Otitis media (middle ear infection)
  • Lymphadenopathy (swollen lymph nodes during infection)

Common Causes

  • ELANE gene mutations (associated with cyclic neutropenia)
  • Glycogen storage disease type Ib
  • Chronic idiopathic neutropenia (not elsewhere classified)
  • Autoimmune neutropenia (secondary to specific antibodies)
  • Metabolic disorders affecting bone marrow function
  • Reticular dysgenesis
  • Hypersplenism leading to sequestration

Documentation & Coding Tips

Explicitly state the etiology of the neutropenia to differentiate between drug-induced, post-infectious, or specified idiopathic forms.

Example: Patient presents with chronic idiopathic neutropenia, stable for 12 months with no history of recurrent oral ulcers or skin infections. ANC remains consistent between 800 and 1000 cells/uL. Condition is stable on current monitoring schedule.

Billing Focus: Identify the neutropenia as 'other specified' rather than 'unspecified' to support D70.4 over D70.9.

Document the current Absolute Neutrophil Count (ANC) to establish clinical severity levels of the neutropenia.

Example: Laboratory results show an ANC of 450 cells/uL, indicating severe neutropenia. Patient is currently afebrile and asymptomatic. Diagnosis: Other specified neutropenia (chronic idiopathic).

Billing Focus: Specific laboratory values justify the level of Medical Decision Making and the severity of the diagnosis.

Record the frequency and duration of the neutropenic state to distinguish acute from chronic conditions.

Example: Other specified neutropenia characterized by persistent low neutrophil counts over the last 18 months, with quarterly CBC monitoring confirming ANC levels between 1100 and 1250.

Billing Focus: Documentation of duration supports the classification of a chronic specified hematological disorder.

Describe associated symptoms or lack thereof, such as frequent infections or aphthous ulcers, to support the clinical picture.

Example: Diagnosed with other specified neutropenia (autoimmune). Patient reports recurrent episodes of gingivitis and slow-healing skin abrasions when ANC drops below 500.

Billing Focus: Symptom documentation provides clinical evidence for the 'specified' designation in the code description.

Clarify if the neutropenia is secondary to a specific external cause or underlying systemic disease not captured by other D70 codes.

Example: Other specified neutropenia due to chronic splenic sequestration in the setting of portal hypertension. Neutrophil count is 1200 cells/uL.

Billing Focus: Specifying the underlying cause (e.g., splenic sequestration) prevents use of less specific codes.

Relevant CPT Codes