1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. E70- Disorders of aromatic amino-acid metabolism
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2020 ICD-10-CM Diagnosis Code E70.330

Chediak-Higashi syndrome

2016 2017 2018 2019 2020 Billable/Specific Code

• E70.330 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2020 edition of ICD-10-CM E70.330 became effective on October 1, 2019.
• This is the American ICD-10-CM version of E70.330 - other international versions of ICD-10 E70.330 may differ.

• Chediak higashi syndrome
• Chèdiak-higashi syndrome
• Chédiak-higashi syndrome

• A form of phagocyte bactericidal dysfunction characterized by unusual oculocutaneous albinism, high incidence of lymphoreticular neoplasms, and recurrent pyogenic infections. In many cell types, abnormal lysosomes are present leading to defective pigment distribution and abnormal neutrophil functions. The disease is transmitted by autosomal recessive inheritance and a similar disorder occurs in the beige mouse, the aleutian mink, and albino hereford cattle.
• A rare autosomal recessive immunodeficiency disorder characterized by abnormal intracellular protein transport. Chediak-higashi syndrome (chs) is characterized by immune deficiency; partial oculocutaneous albinism; a bleeding disorder due to deficient platelet dense bodies; neutropenia; neutrophils with impaired chemotaxis and bactericidal activity; recurrent infection; and abnormal natural killer (nk) cell function. Chs may be associated with hepatosplenomegaly, lymphadenopathy, anemia, thrombocytopenia, roentgenologic changes in bones, lungs and heart, and skin and psychomotor abnormalities; it is often fatal in childhood as a result of infection or an accelerated lymphoma-like phase. Chs occurs in mink, cattle, and mice, as well as man.
• Form of phagocyte bactericidal dysfunction characterized by unusual oculocutaneous albinism, high incidence of lymphoreticular neoplasms, and recurrent pyogenic infections; in many cell types, abnormal lysosomes are present leading to defective pigment distribution and abnormal neutrophil functions; transmitted by autosomal recessive inheritance and a similar disorder occurs in the beige mouse, the aleutian mink, and albino hereford cattle.

• 642 Inborn and other disorders of metabolism

Convert E70.330 to ICD-9-CM

• 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
• 2017 (effective 10/1/2016): No change
• 2018 (effective 10/1/2017): No change
• 2019 (effective 10/1/2018): No change
• 2020 (effective 10/1/2019): No change

• Type 1 Excludes: D72.0
  , E70.32
  ICD-10-CM Diagnosis Code D72.0

  Genetic anomalies of leukocytes

  2016 2017 2018 2019 2020 Billable/Specific Code

  Applicable To

  • Alder (granulation) (granulocyte) anomaly
  • Alder syndrome
  • Hereditary leukocytic hypersegmentation
  • Hereditary leukocytic hyposegmentation
  • Hereditary leukomelanopathy
  • May-Hegglin (granulation) (granulocyte) anomaly
  • May-Hegglin syndrome
  • Pelger-Huët (granulation) (granulocyte) anomaly
  • Pelger-Huët syndrome

  Type 1 Excludes

  • Chédiak (-Steinbrinck)-Higashi syndrome (E70.330)
  ICD-10-CM Diagnosis Code E70.32

  Oculocutaneous albinism

  2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code

  Type 1 Excludes

  • Chediak-Higashi syndrome (E70.330)
  • Hermansky-Pudlak syndrome (E70.331)

• Albinism, albino E70.30
  ICD-10-CM Diagnosis Code E70.30

  Albinism, unspecified

  2016 2017 2018 2019 2020 Billable/Specific Code
  • with hematologic abnormality E70.339
    ICD-10-CM Diagnosis Code E70.339

    Albinism with hematologic abnormality, unspecified

    2016 2017 2018 2019 2020 Billable/Specific Code
    • Chédiak-Higashi syndrome E70.330
• Anomaly, anomalous (congenital) (unspecified type) Q89.9
  ICD-10-CM Diagnosis Code Q89.9

  Congenital malformation, unspecified

  2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt

  Applicable To

  • Congenital anomaly NOS
  • Congenital deformity NOS
  • Chédiak-Higashi E70.330 (-Steinbrinck) (congenital gigantism of peroxidase granules)
• Chédiak-Higashi E70.330 (-Steinbrinck) syndrome (congenital gigantism of peroxidase granules)
• Disease, diseased - see also Syndrome
  • Chédiak-Steinbrinck E70.330 (-Higashi) (congenital gigantism of peroxidase granules)
• Syndrome - see also Disease
  • Béquez César E70.330 (-Steinbrinck-Chédiak-Higashi)
  • Chédiak-Higashi E70.330 (-Steinbrinck)

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.