ICD-10-CM Code D56.1

Beta thalassemia

Version 2021 Billable Code

Valid for Submission

D56.1 is a billable code used to specify a medical diagnosis of beta thalassemia. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code D56.1 might also be used to specify conditions or terms like a>gamma< beta^+^ hpfh and beta^0^ thalassemia in cis, beta plus thalassemia, beta plus thalassemia, beta plus thalassemia, beta plus thalassemia, beta thalassemia, etc

Short Description:Beta thalassemia
Long Description:Beta thalassemia

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 D56.1:

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.
  • Beta thalassemia major
  • Cooley's anemia
  • Homozygous beta thalassemia
  • Severe beta thalassemia
  • Thalassemia intermedia
  • Thalassemia major

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.
  • beta thalassemia minor D56.3
  • beta thalassemia trait D56.3
  • delta-beta thalassemia D56.2
  • hemoglobin E-beta thalassemia D56.5
  • sickle-cell beta thalassemia D57.4

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 D56.1 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:

  • A>gamma< beta^+^ HPFH AND beta^0^ thalassemia in cis
  • Beta plus thalassemia
  • Beta plus thalassemia
  • Beta plus thalassemia
  • Beta plus thalassemia
  • Beta thalassemia
  • Beta thalassemia intermedia
  • Beta thalassemia X-linked thrombocytopenia syndrome
  • Beta zero thalassemia
  • beta^+^ Thalassemia, normal Hb A>2<, type 1, silent
  • beta^+^ Thalassemia, normal Hb A>2<, type 2
  • beta^0^ Thalassemia, deletion type
  • beta^0^ Thalassemia, nondeletion type
  • Dominant beta-thalassemia
  • Hereditary persistence of fetal hemoglobin G gamma beta plus thalassemia
  • Hereditary persistence of fetal hemoglobin thalassemia
  • Hereditary persistence of fetal hemoglobin thalassemia
  • Homozygous beta thalassemia
  • HPFH A gamma beta^+^ thalassemia
  • Sickle cell beta plus thalassemia
  • Sickle cell-beta-thalassemia
  • Sickle cell-thalassemia disease
  • Thalassemia intermedia
  • Thalassemia major
  • Thalassemia with other hemoglobinopathy

Clinical Information

  • BETA THALASSEMIA-. a disorder characterized by reduced synthesis of the beta chains of hemoglobin. there is retardation of hemoglobin a synthesis in the heterozygous form thalassemia minor which is asymptomatic while in the homozygous form thalassemia major cooley's anemia mediterranean anemia erythroblastic anemia which can result in severe complications and even death hemoglobin a synthesis is absent.

Convert D56.1 to ICD-9

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)

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


Also called: Cooley's anemia, Mediterranean anemia

Thalassemias are inherited blood disorders. If you have one, your body makes fewer healthy red blood cells and less hemoglobin. Hemoglobin is a protein that carries oxygen to the body. That leads to anemia. Thalassemias occur most often among people of Italian, Greek, Middle Eastern, Southern Asian, and African descent.

Thalassemias can be mild or severe. Some people have no symptoms or mild anemia. The most common severe type in the United States is called Cooley's anemia. It usually appears during the first two years of life. People with it may have severe anemia, slowed growth and delayed puberty, and problems with the spleen, liver, heart, or bones.

Doctors diagnose thalassemias using blood tests. Treatments include blood transfusions and treatment to remove excess iron from the body. If you have mild symptoms or no symptoms, you may not need treatment. In some severe cases, you may need a bone marrow transplant.

NIH: National Heart, Lung, and Blood Institute

  • Thalassemia (Medical Encyclopedia)

[Learn More]

Beta thalassemia Beta thalassemia is a blood disorder that reduces the production of hemoglobin. Hemoglobin is the iron-containing protein in red blood cells that carries oxygen to cells throughout the body.In people with beta thalassemia, low levels of hemoglobin lead to a lack of oxygen in many parts of the body. Affected individuals also have a shortage of red blood cells (anemia), which can cause pale skin, weakness, fatigue, and more serious complications. People with beta thalassemia are at an increased risk of developing abnormal blood clots.Beta thalassemia is classified into two types depending on the severity of symptoms: thalassemia major (also known as Cooley's anemia) and thalassemia intermedia. Of the two types, thalassemia major is more severe.The signs and symptoms of thalassemia major appear within the first 2 years of life. Children develop life-threatening anemia. They do not gain weight and grow at the expected rate (failure to thrive) and may develop yellowing of the skin and whites of the eyes (jaundice). Affected individuals may have an enlarged spleen, liver, and heart, and their bones may be misshapen. Some adolescents with thalassemia major experience delayed puberty. Many people with thalassemia major have such severe symptoms that they need frequent blood transfusions to replenish their red blood cell supply. Over time, an influx of iron-containing hemoglobin from chronic blood transfusions can lead to a buildup of iron in the body, resulting in liver, heart, and hormone problems.Thalassemia intermedia is milder than thalassemia major. The signs and symptoms of thalassemia intermedia appear in early childhood or later in life. Affected individuals have mild to moderate anemia and may also have slow growth and bone abnormalities.
[Learn More]