2020 ICD-10-CM Diagnosis Code D76.1

Hemophagocytic lymphohistiocytosis

    2016 2017 2018 2019 2020 Billable/Specific Code
  • D76.1 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 D76.1 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of D76.1 - other international versions of ICD-10 D76.1 may differ.
Applicable To
  • Familial hemophagocytic reticulosis
  • Histiocytoses of mononuclear phagocytes
The following code(s) above D76.1 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 D76.1:
  • 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
  • Familial hemophagocytic lymphohistiocytosis
Clinical Information
  • A group of related disorders characterized by lymphocytosis; histiocytosis; and hemophagocytosis. The two major forms are familial and reactive.
  • A rare but potentially life-threatening disorder characterized by the proliferation of histiocytes and macrophages and phagocytosis of red blood cells, white blood cells, and platelets. It may be inherited or secondary, due to infections, autoimmune disorders, or underlying malignancies. Signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia.
  • A rare disorder in which histiocytes and lymphocytes (types of white blood cells) build up in organs including the skin, spleen, and liver, and destroy other blood cells. Hemophagocytic lymphohistiocytosis may be inherited or caused by certain conditions or diseases, including infections, immunodeficiency (inability of the body to fight infections), and cancer.
  • A rare, life-threatening disorder usually appearing during the first few months of life. It is caused by abnormalities in the prf1, unc13d, and stx11 genes. It is characterized by histiocytic proliferation and phagocytosis. Patients present with fever, lymphadenopathy, and hepatosplenomegaly.
  • Rare form of hemophagocytic lymphohistiocytosis with multiorgan involvement seen in infants and young children
ICD-10-CM D76.1 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 D76.1 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 D76.1:

ICD-10-CM Codes Adjacent To D76.1
D75 Other and unspecified diseases of blood and blood-forming organs
D75.0 Familial erythrocytosis
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

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