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. E75- Disorders of sphingolipid metabolism and other lipid storage disorders
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2018/2019 ICD-10-CM Diagnosis Code E75.22

Gaucher disease

2016 2017 2018 2019 Billable/Specific Code

• E75.22 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2018/2019 edition of ICD-10-CM E75.22 became effective on October 1, 2018.
• This is the American ICD-10-CM version of E75.22 - other international versions of ICD-10 E75.22 may differ.

• Gaucher's disease
• Gauchers disease

• An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (glucosylceramidase) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the mononuclear phagocyte system. The characteristic gaucher cells, glycosphingolipid-filled histiocytes, displace normal cells in bone marrow and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement.
• An inherited lysosomal storage disease caused by deficiency of the enzyme glucocerebrosidase. It results in the accumulation of a fatty substance called glucocerebroside in mononuclear cells in the bone marrow, liver, spleen, brain, and kidneys. Signs and symptoms include hepatomegaly, splenomegaly, neurologic disorders, lymphadenopathy, skeletal disorders, anemia and thrombocytopenia.
• Autosomal recessive disorder caused by deficiency of the enzyme glucocerebrosidase featuring the pathological storage of glycosylceramide in mononuclear phagocytes; the most common subtype is the non-neuronopathic form, a slowly progressive condition characterized by hepatosplenomegaly and skeletal deformities; the neuronopathic forms are divided into infantile and juvenile forms; the infantile form presents at 4-5 months of age with anemia, loss of cognitive gains, neck retraction, dysphagia, and hepatosplenomegaly; the juvenile form features a slowly progressive loss of intellect, hepatosplenomegaly, ataxia, myoclonic seizures, and spasticity; the neuronopathic forms are characterized by neuronal loss with neuronophagia, and accumulation of glucocerebroside in neurons.
• Gaucher's disease is a rare, inherited disorder that causes too much of a substance called glucocerebroside to build up in your spleen, liver, lungs, bones and sometimes in your brain. The buildup prevents these organs from working properly.there are three types:
  • type 1, the most common form, causes liver and spleen enlargement, bone pain and broken bones, and, sometimes, lung and kidney problems. It does not involve the brain. It can occur at any age.
  • type 2, which causes severe brain damage, appears in infants. Most children who have it die by age 2.
  • in type 3, there may be liver and spleen enlargement, and signs of brain involvement appear gradually.
  gaucher's disease has no cure. Treatment options for types 1 and 3 include medicine and enzyme replacement therapy, which is usually very effective. There is no good treatment for the brain damage of type 2.

• 642 Inborn and other disorders of metabolism

Convert E75.22 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

• Code Also: I27.29
  ICD-10-CM Diagnosis Code I27.29

  Other secondary pulmonary hypertension

  2018 - New Code 2019 Billable/Specific Code

  Applicable To

  • Group 5 pulmonary hypertension
  • Pulmonary hypertension with unclear multifactorial mechanisms
  • Pulmonary hypertension due to hematologic disorders
  • Pulmonary hypertension due to metabolic disorders
  • Pulmonary hypertension due to other systemic disorders

  Code Also

  • other associated disorders, if known, such as:
  • chronic myeloid leukemia (C92.10- C92.22)
  • essential thrombocythemia (D47.3)
  • Gaucher disease (E75.22)
  • hypertensive chronic kidney disease with end stage renal disease (I12.0, I13.11, I13.2)
  • hyperthyroidism (E05.-)
  • hypothyroidism (E00-E03)
  • polycythemia vera (D45)
  • sarcoidosis (D86.-)

• Cerebroside lipidosis E75.22
• Degeneration, degenerative
  • brain (cortical) (progressive) G31.9
    ICD-10-CM Diagnosis Code G31.9

    Degenerative disease of nervous system, unspecified

    2016 2017 2018 2019 Billable/Specific Code
    • in
      • Gaucher's disease E75.22
• Gaucher's disease or splenomegaly E75.22 (adult) (infantile)
• Lipidosis E75.6
  ICD-10-CM Diagnosis Code E75.6

  Lipid storage disorder, unspecified

  2016 2017 2018 2019 Billable/Specific Code
  • cerebroside E75.22
• Splenomegaly, splenomegalia (Bengal) (cryptogenic) (idiopathic) (tropical) R16.1
  ICD-10-CM Diagnosis Code R16.1

  Splenomegaly, not elsewhere classified

  2016 2017 2018 2019 Billable/Specific Code

  Applicable To

  • Splenomegaly NOS
  • Gaucher's E75.22

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