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ICD10 code of Kostmann Syndrome and ICD9 code

What is the ICD10 code for Kostmann Syndrome? And the ICD9 code for Kostmann Syndrome?

ICD9 and ICD10 codes of Kostmann Syndrome

Kostmann Syndrome is a rare genetic disorder characterized by severe congenital neutropenia, leading to recurrent bacterial infections. The ICD-10 code for Kostmann Syndrome is D70.0. Unfortunately, there is no specific ICD-9 code for this condition as it has been replaced by the ICD-10 coding system. Proper diagnosis and management of Kostmann Syndrome are crucial to prevent complications and ensure appropriate treatment.


Kostmann Syndrome, also known as severe congenital neutropenia, is a rare genetic disorder characterized by a severe reduction in the number of neutrophils, a type of white blood cell responsible for fighting bacterial infections. To accurately classify and code this condition, medical professionals use the International Classification of Diseases (ICD) coding system.

In the current version of ICD, ICD-10, the specific code for Kostmann Syndrome is D70.9. The "D" indicates that it falls under the chapter for diseases of the blood and blood-forming organs. The code "70" pertains to neutropenia, and the ".9" signifies an unspecified type. This code is used to identify and track cases of Kostmann Syndrome in medical records, insurance claims, and statistical analysis.

In the previous version of ICD, ICD-9, Kostmann Syndrome was classified under a different coding system. It was identified by the code 288.0, which was used for agranulocytosis, a broader term encompassing severe neutropenia. This code helped healthcare professionals in accurately documenting and billing for the condition during that timeframe.

It is crucial for medical practitioners to assign the appropriate ICD code to Kostmann Syndrome to ensure accurate communication, effective treatment planning, and proper reimbursement. These codes also facilitate research, epidemiological studies, and the identification of trends in the prevalence and outcomes of this rare disorder.
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ICD9 and ICD10 codes of Kostmann Syndrome

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