ICD-10-CM Code D47.02

Systemic mastocytosis

Version 2021 Billable Code

Valid for Submission

D47.02 is a billable code used to specify a medical diagnosis of systemic mastocytosis. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code D47.02 might also be used to specify conditions or terms like indolent systemic mastocytosis or malignant white blood cell disorder or systemic mast cell disease or systemic mastocytosis with associated clonal hematological non-mast cell lineage disease.

Short Description:Systemic mastocytosis
Long Description:Systemic mastocytosis

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 D47.02:

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.
  • Indolent systemic mastocytosis
  • Isolated bone marrow mastocytosis
  • Smoldering systemic mastocytosis
  • Systemic mastocytosis, with an associated hematological non-mast cell lineage disease (SM-AHNMD)

Code Also

Code Also
A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction.
  • , if applicable, any associated hematological non-mast cell lineage disease, such as:
  • acute myeloid leukemia C92.6 C92.A
  • chronic myelomonocytic leukemia C93.1
  • essential thrombocytosis D47.3
  • hypereosinophilic syndrome D72.1
  • myelodysplastic syndrome D46.9
  • myeloproliferative syndrome D47.1
  • non-Hodgkin lymphoma C82 C85
  • plasma cell myeloma C90.0
  • polycythemia vera D45

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • aggressive systemic mastocytosis C96.21
  • mast cell leukemia C94.3

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 D47.02 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:

  • Indolent systemic mastocytosis
  • Malignant white blood cell disorder
  • Systemic mast cell disease
  • Systemic mastocytosis with associated clonal hematological non-mast cell lineage disease

Clinical Information

  • MASTOCYTOSIS SYSTEMIC-. a group of disorders caused by the abnormal proliferation of mast cells in a variety of extracutaneous tissues including bone marrow liver spleen lymph nodes and gastrointestinal tract. systemic mastocytosis is commonly seen in adults. these diseases are categorized on the basis of clinical features pathologic findings and prognosis.

Diagnostic Related Groups

The ICD-10 code D47.02 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.


Replacement Code

D4702 replaces the following previously assigned ICD-10 code(s):

  • D47.0 - Histiocytic and mast cell tumors of uncertain behavior
  • D47.0 - Mast cell neoplasms of uncertain behavior

Convert D47.02 to ICD-9

  • 238.5 - Mastocytoma NOS (Approximate Flag)

Code Classification

  • Neoplasms (C00–D48)
    • Neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes (D37-D48)
      • Oth neoplm of uncrt behav of lymphoid, hematpoetc & rel tiss (D47)

Code History

  • 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

Systemic mastocytosis Systemic mastocytosis is a blood disorder that can affect many different body systems. Individuals with the condition can develop signs and symptoms at any age, but it usually appears after adolescence.Signs and symptoms of systemic mastocytosis often include extreme tiredness (fatigue), skin redness and warmth (flushing), nausea, abdominal pain, bloating, diarrhea, the backflow of stomach acids into the esophagus (gastroesophageal reflux), nasal congestion, shortness of breath, low blood pressure (hypotension), lightheadedness, and headache. Some affected individuals have attention or memory problems, anxiety, or depression. Many individuals with systemic mastocytosis develop a skin condition called urticaria pigmentosa, which is characterized by raised patches of brownish skin that sting or itch with contact or changes in temperature. Nearly half of individuals with systemic mastocytosis will experience severe allergic reactions (anaphylaxis).There are five subtypes of systemic mastocytosis, which are differentiated by their severity and the signs and symptoms. The mildest forms of systemic mastocytosis are the indolent and smoldering types. Individuals with these types tend to have only the general signs and symptoms of systemic mastocytosis described above. Individuals with smoldering mastocytosis may have more organs affected and more severe features than those with indolent mastocytosis. The indolent type is the most common type of systemic mastocytosis.The severe types include aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic neoplasm, and mast cell leukemia. These types are associated with a reduced life span, which varies among the types and affected individuals. In addition to the general signs and symptoms of systemic mastocytosis, these types typically involve impaired function of an organ, such as the liver, spleen, or lymph nodes. The organ dysfunction can result in an abnormal buildup of fluid in the abdominal cavity (ascites). Aggressive systemic mastocytosis is associated with a loss of bone tissue (osteoporosis and osteopenia) and multiple bone fractures. Systemic mastocytosis with an associated hematologic neoplasm and mast cell leukemia both involve blood cell disorders or blood cell cancer (leukemia). Mast cell leukemia is the rarest and most severe type of systemic mastocytosis.Individuals with the milder forms of the condition generally have a normal or near normal life expectancy, while those with the more severe forms typically survive months or a few years after diagnosis.
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