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) NOS (M35.9)
- certain conditions originating in the perinatal period (P00-P96)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- human immunodeficiency virus [HIV] disease (B20)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Use Additional Code
Additional codes that should follow this code
- code for any associated:
- fever (R50.81)
Also Known As / Clinical Terms
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
Frequently Asked Questions
What is ICD-10 code D70.0?
ICD-10-CM code D70.0 represents "Congenital agranulocytosis". It 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 you should also code: code for 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.
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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.