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.
The following code(s) above E70.330 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E70.330:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
Approximate Synonyms
  • Chediak higashi syndrome
  • Chèdiak-higashi syndrome
  • Chédiak-higashi syndrome
Clinical Information
  • 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.
ICD-10-CM E70.330 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E70.330 to ICD-9-CM

Code History
  • 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
Code annotations containing back-references to E70.330:
  • 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

Diagnosis Index entries containing back-references to E70.330:
  • 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)

ICD-10-CM Codes Adjacent To E70.330
E70.310 X-linked ocular albinism
E70.311 Autosomal recessive ocular albinism
E70.318 Other ocular albinism
E70.319 …… unspecified
E70.32 Oculocutaneous albinism
E70.320 Tyrosinase negative oculocutaneous albinism
E70.321 Tyrosinase positive oculocutaneous albinism
E70.328 Other oculocutaneous albinism
E70.329 …… unspecified
E70.33 Albinism with hematologic abnormality
E70.330 Chediak-Higashi syndrome
E70.331 Hermansky-Pudlak syndrome
E70.338 Other albinism with hematologic abnormality
E70.339 …… unspecified
E70.39 Other specified albinism
E70.4 Disorders of histidine metabolism
E70.40 …… unspecified
E70.41 Histidinemia
E70.49 Other disorders of histidine metabolism
E70.5 Disorders of tryptophan metabolism
E70.8 Other disorders of aromatic amino-acid metabolism

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