D70.0
BillableCongenital agranulocytosis
Congenital agranulocytosis
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Congenital neutropenia
- Infantile genetic agranulocytosis
- Kostmann's disease
Includes
Conditions included under this code
- agranulocytosis
- decreased absolute neurophile count (ANC)
Excludes 1
Codes that cannot be used together with this code (mutual exclusion)
Excludes 2
Conditions not included here, but the patient may have both
- •autoimmune disease (systemic) NOSM35.9
- •certain conditions originating in the perinatal periodP00-P96
- •complications of pregnancy, childbirth and the puerperiumO9A)O00
- •congenital malformations, deformations and chromosomal abnormalitiesQ00-Q99
- •endocrine, nutritional and metabolic diseasesE00-E88
- •human immunodeficiency virus [HIV] diseaseB20
- •injury, poisoning and certain other consequences of external causesS00-T88
- •neoplasmsC00-D49
- •symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classifiedR00-R94
Use Additional Code
Additional codes that should follow this code
- code for any associated:
- •feverR50.81
Code Also
A second code may be required; sequencing depends on circumstances
Related Codes(6)
Also Known As / Clinical Terms(122)
SNOMED CT
- Complete deafness8531006
- CDA - congenital dyserythropoietic anaemia52951008
- CDA - congenital dyserythropoietic anemia52951008
- Congenital dyserythropoietic anaemia52951008
- Congenital dyserythropoietic anemia52951008
- Agnogenic myeloid metaplasia52967002
- MF - Myelofibrosis52967002
- Myelofibrosis52967002
- Myelosclerosis52967002
- Congenital neutropenia89655007
- Primary neutropenia89655007
- Severe congenital neutropenia89655007
- Severe infantile genetic neutropenia89655007
- Congenital deafness95828007
- Giant platelet syndrome234478007
- X-linked severe congenital neutropenia718882006
- Trichothiodystrophy723551003
- Neutropenia, monocytopenia, deafness syndrome725137007
- Infantile agranulocytosis770942003
- Infantile genetic agranulocytosis770942003
- Kostmann syndrome770942003
- Severe congenital neutropaenia type 3770942003
- Severe congenital neutropenia type 3770942003
- Autosomal dominant severe congenital neutropaenia770947009
- Autosomal dominant severe congenital neutropenia770947009
- Congenital neutropenia, bone marrow fibrosis, nephromegaly syndrome775909002
- Congenital neutropenia, myelofibrosis, nephromegaly syndrome775909002
- VPS45 deficiency775909002
- X-linked dyserythropoietic anaemia with abnormal platelets and neutropenia782759001
- X-linked dyserythropoietic anemia with abnormal platelets and neutropenia782759001
- Autosomal recessive severe congenital neutropenia due to G6PC3 deficiency783058007
- Autosomal recessive severe congenital neutropenia due to glucose-6-phosphatase catalytic subunit 3 deficiency783058007
- Severe congenital neutropenia type 4783058007
- Severe congenital neutropenia, pulmonary hypertension, superficial venous angiectasis syndrome783058007
- Autosomal recessive severe congenital neutropenia due to JAGN1 (jagunal homolog 1) deficiency783199003
- Autosomal recessive severe congenital neutropenia due to JAGN1 deficiency783199003
- Autosomal recessive severe congenital neutropenia due to jagunal homolog 1 deficiency783199003
- Autosomal recessive severe congenital neutropenia due to C-X-C motif chemokine receptor 2 deficiency783200000
- Autosomal recessive severe congenital neutropenia due to CXCR2 (C-X-C motif chemokine receptor 2) deficiency783200000
- Autosomal recessive severe congenital neutropenia due to CXCR2 deficiency783200000
- Autosomal recessive severe congenital neutropenia due to CSF3R (colony stimulating factor 3 receptor) deficiency783201001
- Autosomal recessive severe congenital neutropenia due to CSF3R deficiency783201001
- Autosomal recessive severe congenital neutropenia due to colony stimulating factor 3 receptor deficiency783201001
- Onycho-tricho-dysplasia neutropenia syndrome1003381002
- Trichothiodystrophy type G1003381002
- Periodontitis due to Kostmann syndrome1184703009
- Periodontitis due to infantile genetic agranulocytosis1184703009
- Periodontitis due to congenital neutropenia1209075008
UMLS
- Autosomal dominant or sporadic congenital neutropeniaC0340970
- Congenital AgranulocytosisC0340970
- Congenital NeutropeniaC0340970
- Congenital agranulocytosisC0340970
- Congenital neutropeniaC0340970
- Congenital neutropenia (disorder)C0340970
- Infantile genetic agranulocytosisC0340970
- Primary neutropeniaC0340970
- Severe congenital neutropeniaC0340970
- Kostmann's diseaseC1853118
- Kostmann's syndromeC1853118
- Neutropenia, severe congenitalC1853118
- SCNC1853118
- Severe Congenital NeutropeniaC1853118
- Severe infantile genetic neutropeniaC1853118
- agranulocytosis genetic infantileC1853118
- congenital neutropeniaC1853118
- kostmann's syndromeC1853118
- severe congenital neutropeniaC1853118
Clinical Terms
- Giant platelet syndrome
- Autosomal dominant severe congenital neutropenia
- MF - Myelofibrosis
- Severe congenital neutropenia type 4
- Congenital Neutropenia
- Complete deafness
- Congenital dyserythropoietic anemia
- Trichothiodystrophy
- Kostmann syndrome
- Myelosclerosis
- VPS45 deficiency
- CDA - congenital dyserythropoietic anaemia
- Infantile agranulocytosis
- Autosomal recessive severe congenital neutropenia due to G6PC3 deficiency
- Kostmann's syndrome
- Periodontitis due to Kostmann syndrome
- Congenital dyserythropoietic anaemia
- Congenital neutropenia, myelofibrosis, nephromegaly syndrome
- Primary neutropenia
- Periodontitis due to infantile genetic agranulocytosis
- Agnogenic myeloid metaplasia
- Autosomal dominant severe congenital neutropaenia
- X-linked dyserythropoietic anaemia with abnormal platelets and neutropenia
- Autosomal recessive severe congenital neutropenia due to glucose-6-phosphatase catalytic subunit 3 deficiency
- Severe infantile genetic neutropenia
- Neutropenia, severe congenital
- Kostmann's disease
- severe congenital neutropenia
- Congenital neutropenia (disorder)
- SCN
- Autosomal recessive severe congenital neutropenia due to CSF3R (colony stimulating factor 3 receptor) deficiency
- Autosomal recessive severe congenital neutropenia due to CSF3R deficiency
- Autosomal recessive severe congenital neutropenia due to colony stimulating factor 3 receptor deficiency
- agranulocytosis genetic infantile
- Autosomal dominant or sporadic congenital neutropenia
- Congenital neutropenia, bone marrow fibrosis, nephromegaly syndrome
- Autosomal recessive severe congenital neutropenia due to jagunal homolog 1 deficiency
- Periodontitis due to congenital neutropenia
- CDA - congenital dyserythropoietic anemia
- Congenital deafness
- Autosomal recessive severe congenital neutropenia due to CXCR2 deficiency
- X-linked severe congenital neutropenia
- Infantile genetic agranulocytosis
- Myelofibrosis
- Severe congenital neutropenia, pulmonary hypertension, superficial venous angiectasis syndrome
- Autosomal recessive severe congenital neutropenia due to CXCR2 (C-X-C motif chemokine receptor 2) deficiency
- Autosomal recessive severe congenital neutropenia due to JAGN1 (jagunal homolog 1) deficiency
- Severe congenital neutropenia type 3
- Autosomal recessive severe congenital neutropenia due to C-X-C motif chemokine receptor 2 deficiency
- Severe congenital neutropaenia type 3
- Trichothiodystrophy type G
- X-linked dyserythropoietic anemia with abnormal platelets and neutropenia
- Neutropenia, monocytopenia, deafness syndrome
- Onycho-tricho-dysplasia neutropenia syndrome
- Autosomal recessive severe congenital neutropenia due to JAGN1 deficiency
Frequently Asked Questions
What is the ICD-10 code for congenital agranulocytosis?
The ICD-10-CM code for congenital agranulocytosis is D70.0. The full clinical description is "Congenital agranulocytosis". D70.0 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code D70.0 mean?
ICD-10-CM code D70.0 represents “Congenital agranulocytosis”. It is classified under Chapter 3: Diseases of the Blood and Blood-Forming Organs and is a billable/specific code that can be used on a claim.
Is D70.0 a billable code?
Yes, D70.0 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is D70.0 in?
D70.0 is in Chapter 3: Diseases of the Blood and Blood-Forming Organs (codes D50-D89).
What codes cannot be used with D70.0?
D70.0 has Excludes1 notes indicating codes that cannot be used together with it, including: neutropenic splenomegaly (D73.81); transient neonatal neutropenia (P61.5).
Are additional codes required with D70.0?
Yes, when using D70.0, also report: any associated:; fever (R50.81).
What SNOMED CT codes does D70.0 map to?
D70.0 maps to 20 SNOMED CT concepts: 52967002, 770947009, 783200000, 783201001, 783058007, and 15 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for D70.0?
D70.0 is linked to 2 UMLS Concept Unique Identifiers: C0340970, C1853118. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does D70.0 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like congenital agranulocytosis affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.
What is the ICD-11 equivalent of D70.0?
There is no direct ICD-11 mapping available for D70.0 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.
Automate ICD-10 Coding With AI
Send clinical text to the AutoICD API and get back structured ICD-10 codes with confidence scores. Integrates into any EHR or billing system in minutes.
Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.