ICD-10-CM Code D76.1

Hemophagocytic lymphohistiocytosis

Version 2020 Billable Code

Valid for Submission

D76.1 is a billable code used to specify a medical diagnosis of hemophagocytic lymphohistiocytosis. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code D76.1 might also be used to specify conditions or terms like familial hemophagocytic lymphohistiocytosis, fatal infectious mononucleosis, fatal infectious mononucleosis associated with x-linked lymphoproliferative syndrome, hemolytic erythrophagocytic syndrome, hemophagocytic lymphohistiocytosis, lipochrome histiocytosis - familial, etc

ICD-10:D76.1
Short Description:Hemophagocytic lymphohistiocytosis
Long Description:Hemophagocytic lymphohistiocytosis

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 D76.1:

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.
  • Familial hemophagocytic reticulosis
  • Histiocytoses of mononuclear phagocytes

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 D76.1 are found in the index:


Synonyms

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

  • Familial hemophagocytic lymphohistiocytosis
  • Fatal infectious mononucleosis
  • Fatal infectious mononucleosis associated with X-linked lymphoproliferative syndrome
  • Hemolytic erythrophagocytic syndrome
  • Hemophagocytic lymphohistiocytosis
  • Lipochrome histiocytosis - familial
  • Macrophage activation syndrome

Clinical Information

  • LYMPHOHISTIOCYTOSIS HEMOPHAGOCYTIC-. a group of related disorders characterized by lymphocytosis; histiocytosis; and hemophagocytosis. the two major forms are familial and reactive.

Diagnostic Related Groups

The ICD-10 code D76.1 is grouped in the following groups for version MS-DRG V37.0 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 10/01/2019 through 09/30/2020.

  • 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

  • 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

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.


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Familial hemophagocytic lymphohistiocytosis Familial hemophagocytic lymphohistiocytosis is a disorder in which the immune system produces too many activated immune cells (lymphocytes) called T cells, natural killer cells, B cells, and macrophages (histiocytes). Excessive amounts of immune system proteins called cytokines are also produced. This overactivation of the immune system causes fever and damages the liver and spleen, resulting in enlargement of these organs.Familial hemophagocytic lymphohistiocytosis also destroys blood-producing cells in the bone marrow, a process called hemophagocytosis. As a result, affected individuals have low numbers of red blood cells (anemia) and a reduction in the number of platelets, which are involved in clotting. A reduction in platelets may cause easy bruising and abnormal bleeding.The brain may also be affected in familial hemophagocytic lymphohistiocytosis. As a result, affected individuals may experience irritability, delayed closure of the bones of the skull in infants, neck stiffness, abnormal muscle tone, impaired muscle coordination, paralysis, blindness, seizures, and coma. In addition to neurological problems, familial hemophagocytic lymphohistiocytosis can cause abnormalities of the heart, kidneys, and other organs and tissues. Affected individuals also have an increased risk of developing cancers of blood-forming cells (leukemia and lymphoma).Signs and symptoms of familial hemophagocytic lymphohistiocytosis usually become apparent during infancy, although occasionally they appear later in life. They usually occur when the immune system launches an exaggerated response to an infection, but may also occur in the absence of infection. Without treatment, most people with familial hemophagocytic lymphohistiocytosis survive only a few months.
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