2024 ICD-10-CM Diagnosis Code D64.4

Congenital dyserythropoietic anemia

ICD-10-CM Code:
Short Description:
Congenital dyserythropoietic anemia
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    • Aplastic and other anemias and other bone marrow failure syndromes
      • Other anemias

D64.4 is a billable diagnosis code used to specify a medical diagnosis of congenital dyserythropoietic anemia. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

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

  • Congenital dyserythropoietic anemia
  • Congenital dyserythropoietic anemia
  • Congenital dyserythropoietic anemia
  • Congenital dyserythropoietic anemia
  • Congenital dyserythropoietic anemia type IV
  • Congenital dyserythropoietic anemia, type I
  • Congenital dyserythropoietic anemia, type II
  • Congenital dyserythropoietic anemia, type III
  • Cytochrome-c oxidase deficiency
  • Exocrine pancreatic insufficiency
  • GATA binding protein 1 related thrombocytopenia with dyserythropoiesis
  • Giant platelet syndrome
  • Pancreatic insufficiency
  • Pancreatic insufficiency, dyserythropoietic anemia, calvarial hyperostosis syndrome
  • X-linked congenital dyserythropoietic anemia with thrombocytopenia
  • X-linked dyserythropoietic anemia with abnormal platelets and neutropenia

Clinical Classification

Clinical Category:
Aplastic anemia
CCSR Category Code:
Inpatient Default CCSR:
Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.
Outpatient Default CCSR:
Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.

Clinical Information

  • Exocrine Pancreatic Insufficiency-. a malabsorption condition resulting from greater than 10% reduction in the secretion of pancreatic digestive enzymes (lipase; proteases; and amylase) by the exocrine pancreas into the duodenum. this condition is often associated with cystic fibrosis and with chronic pancreatitis.
  • CDAN1 Gene|CDAN1|CDAN1|Congenital Dyserythropoietic Anemia, Type I Gene-. this gene may be involved in nuclear membrane maintenance.
  • CDAN1 wt Allele|CDA-I|CDA1|CDAI|Codanin Gene|Congenital Dyserythropoietic Anemia, Type I wt Allele|DLT|Discs Lost Homolog Gene|Discs Lost, Drosophila, Homolog of Gene|PRO1295|UNQ664/PRO1295-. human cdan1 wild-type allele is located in the vicinity of 15q15.2 and is approximately 14 kb in length. this allele, which encodes codanin-1 protein, may play a role in the maintenance of the nuclear envelope. mutation of the gene is associated with congenital dyserythropoietic anemia type i.
  • Congenital Dyserythropoietic Anemia-. a rare group of disorders that result in anemia that is caused by ineffective erythropoiesis, which is associated with multinuclear erythroblasts, and which may present in childhood. the most common mutations are in the cdan1 and sec23b genes.
  • Congenital Dyserythropoietic Anemia Type II|CDA II|CDAN2|HEMPAS|Hereditary Erythroblastic Multinuclearity with Positive Acidified-Serum Test|SEC23B-CDG-. an autosomal recessive subtype of congenital dyserythropoietic anemia caused by mutation(s) in the sec23b gene, encoding protein transport protein sec23b.
  • Congenital Dyserythropoietic Anemia Type IV|CDAN4-. an autosomal dominant sub-type of congenital dyserythropoietic anemia caused by mutation(s) in the klf1 gene, encoding krueppel-like factor 1.
  • SEC23B wt Allele|CDA-II|CDAII|CDAN2|Congenital Dyserythropoietic Anemia, Type II Gene|HEMPAS|RP11-379J5.1|Sec23 Homolog B (S. cerevisiae) wt Allele-. human sec23b wild-type allele is located in the vicinity of 20p11.23 and is approximately 54 kb in length. this allele, which encodes protein transport protein sec23b, is involved in the transport of vesicles from the endoplasmic reticulum to the golgi. mutation of the gene is associated with congenital dyserythropoietic anemia type ii.

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert D64.4 to ICD-9-CM

  • ICD-9-CM Code: 285.8 - Anemia NEC
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education


If you have anemia, your blood does not carry enough oxygen to the rest of your body. The most common cause of anemia is not having enough iron. Your body needs iron to make hemoglobin. Hemoglobin is an iron-rich protein that gives the red color to blood. It carries oxygen from the lungs to the rest of the body.

Anemia has three main causes: blood loss, lack of red blood cell production, and high rates of red blood cell destruction.

Conditions that may lead to anemia include:

  • Heavy periods
  • Pregnancy
  • Ulcers
  • Colon polyps or colon cancer
  • Inherited disorders
  • A diet that does not have enough iron, folic acid or vitamin B12
  • Blood disorders such as sickle cell anemia and thalassemia, or cancer
  • Aplastic anemia, a condition that can be inherited or acquired
  • G6PD deficiency, a metabolic disorder

Anemia can make you feel tired, cold, dizzy, and irritable. You may be short of breath or have a headache.

Your doctor will diagnose anemia with a physical exam and blood tests. Treatment depends on the kind of anemia you have.

NIH: National Heart, Lung, and Blood Institute

[Learn More in MedlinePlus]

Congenital dyserythropoietic anemia

Congenital dyserythropoietic anemia (CDA) is an inherited blood disorder that affects the development of red blood cells. This disorder is one of many types of anemia, which is a condition characterized by a shortage of red blood cells. This shortage prevents the blood from carrying an adequate supply of oxygen to the body's tissues. The resulting symptoms can include tiredness (fatigue), weakness, pale skin, and other complications.

Researchers have identified three major types of CDA: type I, type II, and type III. The types have different genetic causes and different but overlapping patterns of signs and symptoms.

CDA type I is characterized by moderate to severe anemia. It is usually diagnosed in childhood or adolescence, although in some cases, the condition can be detected before birth. Many affected individuals have yellowing of the skin and eyes (jaundice) and an enlarged liver and spleen (hepatosplenomegaly). This condition also causes the body to absorb too much iron, which builds up and can damage tissues and organs. In particular, iron overload can lead to an abnormal heart rhythm (arrhythmia), congestive heart failure, diabetes, and chronic liver disease (cirrhosis). Rarely, people with CDA type I are born with skeletal abnormalities, most often involving the fingers and/or toes.

The anemia associated with CDA type II can range from mild to severe, and most affected individuals have jaundice, hepatosplenomegaly, and the formation of hard deposits in the gallbladder called gallstones. This form of the disorder is usually diagnosed in adolescence or early adulthood. An abnormal buildup of iron typically occurs after age 20, leading to complications including heart disease, diabetes, and cirrhosis.

The signs and symptoms of CDA type III tend to be milder than those of the other types. Most affected individuals do not have hepatosplenomegaly, and iron does not build up in tissues and organs. In adulthood, abnormalities of a specialized tissue at the back of the eye (the retina) can cause vision impairment. Some people with CDA type III also have a blood disorder known as monoclonal gammopathy, which can lead to a cancer of white blood cells (multiple myeloma).

Several other variants of CDA have been described, although they appear to be rare and not much is known about them. Once researchers discover the genetic causes of these variants, some of them may be grouped with the three major types of CDA.

[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.


[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.