ICD-10-CM Code D76.3

Other histiocytosis syndromes

Version 2021 Billable Code

Valid for Submission

D76.3 is a billable code used to specify a medical diagnosis of other histiocytosis syndromes. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code D76.3 might also be used to specify conditions or terms like benign cephalic histiocytosis, chronic histiocytosis, cutaneous reticulohistiocytosis, dermal dendritic cell histiocytosis, diffuse normolipemic plane xanthomatosis, familial sea-blue histiocytosis, etc

Short Description:Other histiocytosis syndromes
Long Description:Other histiocytosis syndromes

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 guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code D76.3:

Inclusion Terms

Inclusion 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.
  • Reticulohistiocytoma (giant-cell)
  • Sinus histiocytosis with massive lymphadenopathy
  • Xanthogranuloma

Index to Diseases and Injuries

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 the code D76.3 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Benign cephalic histiocytosis
  • Chronic histiocytosis
  • Cutaneous reticulohistiocytosis
  • Dermal dendritic cell histiocytosis
  • Diffuse normolipemic plane xanthomatosis
  • Familial sea-blue histiocytosis
  • Genetic disorder of lipid storage
  • Hemolytic erythrophagocytic syndrome
  • Hereditary progressive mucinous histiocytosis
  • Histiocytic syndrome
  • Histiocytosis-lymphadenopathy plus syndrome
  • Juvenile xanthogranuloma
  • Juvenile xanthogranuloma of iris
  • Juvenile xanthogranuloma of skin
  • Malakoplakia
  • Malakoplakia
  • Mucinous histiocytosis of the colon
  • Multiple eruptive juvenile xanthogranuloma
  • Necrobiotic xanthogranuloma
  • Necrobiotic xanthogranuloma with paraproteinemia
  • Non-Langerhans cell histiocytic dermatosis
  • Panniculitis secondary to histiocytic disorder
  • Periodontitis co-occurrent and due to histiocytosis syndrome
  • Polyostotic sclerosing histiocytosis
  • Progressive nodular histiocytosis
  • Retroperitoneal xanthogranuloma
  • Sea-blue histiocyte syndrome
  • Sinus histiocytosis with massive lymphadenopathy
  • Solitary eruptive xanthogranuloma
  • Solitary reticulohistiocytoma
  • Subcutaneous xanthogranulomatosis
  • Undetermined cell histiocytosis
  • Xanthogranuloma
  • Xanthogranuloma of choroid plexus

Diagnostic Related Groups

The ICD-10 code D76.3 is grouped in the following groups for version MS-DRG V38.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2020 through 09/30/2021.


Convert D76.3 to ICD-9

  • 288.4 - Hemophagocytic syndromes (Approximate Flag)

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
    • Other disorders of blood and blood-forming organs (D70-D77)
      • Oth dis with lymphoreticular and reticulohistiocytic tissue (D76)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

Lymphatic Diseases

The lymphatic system is a network of tissues and organs. It is made up of

  • Lymph - a fluid that contains white blood cells that defend against germs
  • Lymph vessels - vessels that carry lymph throughout your body. They are different from blood vessels.
  • Lymph nodes - glands found throughout the lymph vessels. Along with your spleen, these nodes are where white blood cells fight infection.

Your bone marrow and thymus produce the cells in lymph. They are part of the system, too.

The lymphatic system clears away infection and keeps your body fluids in balance. If it's not working properly, fluid builds in your tissues and causes swelling, called lymphedema. Other lymphatic system problems can include infections, blockage, and cancer.

  • Cancer and lymph nodes (Medical Encyclopedia)
  • Cystic hygroma (Medical Encyclopedia)
  • Groin lump (Medical Encyclopedia)
  • Lymph node biopsy (Medical Encyclopedia)
  • Lymph system (Medical Encyclopedia)
  • Lymphadenitis (Medical Encyclopedia)
  • Lymphangitis (Medical Encyclopedia)
  • Lymphofollicular hyperplasia (Medical Encyclopedia)
  • Neck lump (Medical Encyclopedia)
  • Swollen lymph nodes (Medical Encyclopedia)

[Learn More]