D70.0 is a billable ICD-10 code used to specify a medical diagnosis of congenital agranulocytosis. The code is valid during the fiscal year 2023 from October 01, 2022 through September 30, 2023 for the submission of HIPAA-covered transactions.
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Autosomal dominant severe congenital neutropenia
- Autosomal recessive severe congenital neutropenia due to CSF3R deficiency
- Autosomal recessive severe congenital neutropenia due to CXCR2 deficiency
- Autosomal recessive severe congenital neutropenia due to G6PC3 deficiency
- Autosomal recessive severe congenital neutropenia due to JAGN1 deficiency
- Complete deafness
- Congenital deafness
- Congenital dyserythropoietic anemia
- Congenital neutropenia
- Congenital neutropenia, myelofibrosis, nephromegaly syndrome
- Genetic defect of hair shaft
- Giant platelet syndrome
- Kostmann syndrome
- Neutropenia, monocytopenia, deafness syndrome
- Onycho-tricho-dysplasia neutropenia syndrome
- Periodontitis due to infantile genetic agranulocytosis
- X-linked dyserythropoietic anemia with abnormal platelets and neutropenia
- X-linked severe congenital neutropenia
- CDAN1 Gene|CDAN1|CDAN1|Congenital Dyserythropoietic Anemia, Type I Gene-. this gene may be involved in nuclear membrane maintenance.
- CDAN1 wt Allele|CDA-I|CDA1|CDAI|Codanin Gene|Congenital Dyserythropoietic Anemia, Type I wt Allele|DLT|Discs Lost Homolog Gene|Discs Lost, Drosophila, Homolog of Gene|PRO1295|UNQ664/PRO1295-. human cdan1 wild-type allele is located in the vicinity of 15q15.2 and is approximately 14 kb in length. this allele, which encodes codanin-1 protein, may play a role in the maintenance of the nuclear envelope. mutation of the gene is associated with congenital dyserythropoietic anemia type i.
- Congenital Dyserythropoietic Anemia-. a rare group of disorders that result in anemia that is caused by ineffective erythropoiesis, which is associated with multinuclear erythroblasts, and which may present in childhood. the most common mutations are in the cdan1 and sec23b genes.
- Congenital Dyserythropoietic Anemia Type II|CDA II|CDAN2|HEMPAS|Hereditary Erythroblastic Multinuclearity with Positive Acidified-Serum Test|SEC23B-CDG-. an autosomal recessive subtype of congenital dyserythropoietic anemia caused by mutation(s) in the sec23b gene, encoding protein transport protein sec23b.
- Congenital Dyserythropoietic Anemia Type IV|CDAN4-. an autosomal dominant sub-type of congenital dyserythropoietic anemia caused by mutation(s) in the klf1 gene, encoding krueppel-like factor 1.
- SEC23B wt Allele|CDA-II|CDAII|CDAN2|Congenital Dyserythropoietic Anemia, Type II Gene|HEMPAS|RP11-379J5.1|Sec23 Homolog B (S. cerevisiae) wt Allele-. human sec23b wild-type allele is located in the vicinity of 20p11.23 and is approximately 54 kb in length. this allele, which encodes protein transport protein sec23b, is involved in the transport of vesicles from the endoplasmic reticulum to the golgi. mutation of the gene is associated with congenital dyserythropoietic anemia type ii.
Tabular List of Diseases and Injuries
The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to this diagnosis code:
Inclusion TermsInclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
- Congenital neutropenia
- Infantile genetic agranulocytosis
- Kostmann's disease
Index to Diseases and Injuries References
The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for this diagnosis code are found in the injuries and diseases index:
- - Agranulocytosis (chronic) (cyclical) (genetic) (infantile) (periodic) (pernicious) - See Also: Neutropenia; - D70.9
- - congenital - D70.0
- - Leukopenia - D72.819
- - Neutropenia, neutropenic (chronic) (genetic) (idiopathic) (immune) (infantile) (malignant) (pernicious) (splenic) - D70.9
- - congenital (primary) - D70.0
Convert to ICD-9 Code
|Source ICD-10 Code||Target ICD-9 Code|
|D70.0||288.01 - Congenital neutropenia|
Your blood is living tissue made up of liquid and solids. The liquid part, called plasma, is made of water, salts and protein. Over half of your blood is plasma. The solid part of your blood contains red blood cells, white blood cells and platelets.
Blood disorders affect one or more parts of the blood and prevent your blood from doing its job. They can be acute or chronic. Many blood disorders are inherited. Other causes include other diseases, side effects of medicines, and a lack of certain nutrients in your diet.
Types of blood disorders include:
- Platelet disorders, excessive clotting, and bleeding problems, which affect how your blood clots
- Anemia, which happens when your blood does not carry enough oxygen to the rest of your body
- Cancers of the blood, such as leukemia and myeloma
- Eosinophilic disorders, which are problems with one type of white blood cell.
[Learn More in MedlinePlus]
Severe congenital neutropenia
Severe congenital neutropenia is a condition that increases the risk of repeated infections in affected individuals. People with this condition have an abnormally low level (deficiency) of neutrophils, a type of white blood cell that plays a role in inflammation and in fighting infection. The shortage of neutrophils, called neutropenia, is apparent at birth or soon afterward. It leads to frequent infections beginning in infancy, including infections of the sinuses, lungs, and liver. Affected individuals can also develop fevers and inflammation of the gums (gingivitis) and skin. Approximately 40 percent of affected people have decreased bone density (osteopenia) and may develop osteoporosis, a condition that makes bones progressively more brittle and likely to fracture. In people with severe congenital neutropenia, bone disorders can begin at any time from infancy through adulthood.
Approximately 20 percent of people with severe congenital neutropenia develop certain cancerous conditions of the blood, particularly myelodysplastic syndrome or leukemia during adolescence.
Some people with severe congenital neutropenia have additional health problems such as seizures, developmental delay, or heart and genital abnormalities.
[Learn More in MedlinePlus]
- FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
- FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
- FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
- FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)