2020 ICD-10-CM Diagnosis Code D76.3

Other histiocytosis syndromes

    2016 2017 2018 2019 2020 Billable/Specific Code
  • D76.3 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 D76.3 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of D76.3 - other international versions of ICD-10 D76.3 may differ.
Applicable To
  • Reticulohistiocytoma (giant-cell)
  • Sinus histiocytosis with massive lymphadenopathy
  • Xanthogranuloma
The following code(s) above D76.3 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 D76.3:
  • D50-D89
    2020 ICD-10-CM Range D50-D89

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

    Type 2 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • certain conditions originating in the perinatal period (P00-P96)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • human immunodeficiency virus [HIV] disease (B20)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
  • D76
    ICD-10-CM Diagnosis Code D76

    Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • (Abt-) Letterer-Siwe disease (C96.0)
    • eosinophilic granuloma (C96.6)
    • Hand-Schüller-Christian disease (C96.5)
    • histiocytic medullary reticulosis (C96.9)
    • histiocytic sarcoma (C96.A)
    • histiocytosis X, multifocal (C96.5)
    • histiocytosis X, unifocal (C96.6)
    • Langerhans-cell histiocytosis, multifocal (C96.5)
    • Langerhans-cell histiocytosis NOS (C96.6)
    • Langerhans-cell histiocytosis, unifocal (C96.6)
    • leukemic reticuloendotheliosis (C91.4-)
    • lipomelanotic reticulosis (I89.8)
    • malignant histiocytosis (C96.A)
    • malignant reticulosis (C86.0)
    • nonlipid reticuloendotheliosis (C96.0)
    Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
Approximate Synonyms
  • Acute histiocytosis
  • Chronic histiocytosis
  • Hemophagocytic syndrome
  • Hemophagocytic syndromes
  • Histiocytic syndrome
  • Histiocytosis
  • Histiocytosis, acute
  • Histiocytosis, chronic
  • Histiocytosis, undetermined cell
  • Juvenile xanthogranuloma
  • Langerhans cell histiocytosis, disseminated (clinical)
  • Rosai dorfman syndrome
  • Sinus histiocytosis with massive lymphadenopathy
  • Undetermined cell histiocytosis
  • Xanthogranuloma
Clinical Information
  • A morphologic finding indicating the presence of histiocytic infiltrates within distended lymph node sinuses.
  • A rare non-neoplastic disorder of unknown etiology characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes. It is usually manifested with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. . it can affect extranodal sites, including the skin, bones, and the respiratory tract. It usually regresses spontaneously.
  • Benign, non-langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. It is often referred to as sinus histiocytosis with massive lymphadenopathy.
  • Condition of faulty lipid metabolism in which yellow nodules of lipoid matter are deposited in the skin and mucosae, giving rise to granulomatous reactions.
ICD-10-CM D76.3 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 814 Reticuloendothelial and immunity disorders with mcc
  • 815 Reticuloendothelial and immunity disorders with cc
  • 816 Reticuloendothelial and immunity disorders without cc/mcc

Convert D76.3 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

Diagnosis Index entries containing back-references to D76.3:
  • Granuloma L92.9
    ICD-10-CM Diagnosis Code L92.9

    Granulomatous disorder of the skin and subcutaneous tissue, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Type 2 Excludes
    • reticulohistiocytic D76.3
  • Histiocytosis D76.3
    • lipid, lipoid D76.3
    • non-Langerhans cell D76.3
    • polyostotic sclerosing D76.3
    • sinus, with massive lymphadenopathy D76.3
    • syndrome NEC D76.3
  • Lipoid - see also condition
  • Reticulohistiocytoma D76.3 (giant-cell)
  • Syndrome - see also Disease
    • histiocytic D76.3
    • histiocytosis NEC D76.3
  • Xanthogranuloma D76.3

ICD-10-CM Codes Adjacent To D76.3
D75.1 Secondary polycythemia
D75.8 Other specified diseases of blood and blood-forming organs
D75.81 Myelofibrosis
D75.82 Heparin induced thrombocytopenia (HIT)
D75.89 Other specified diseases of blood and blood-forming organs
D75.9 Disease of blood and blood-forming organs, unspecified
D75.A Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
D76 Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
D76.1 Hemophagocytic lymphohistiocytosis
D76.2 Hemophagocytic syndrome, infection-associated
D76.3 Other histiocytosis syndromes
D77 Other disorders of blood and blood-forming organs in diseases classified elsewhere
D78 Intraoperative and postprocedural complications of the spleen
D78.0 Intraoperative hemorrhage and hematoma of the spleen complicating a procedure
D78.01 …… on the spleen
D78.02 Intraoperative hemorrhage and hematoma of the spleen complicating other procedure
D78.1 Accidental puncture and laceration of the spleen during a procedure
D78.11 …… on the spleen
D78.12 Accidental puncture and laceration of the spleen during other procedure
D78.2 Postprocedural hemorrhage of the spleen following a procedure
D78.21 …… on the spleen

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