D61.09 - Other constitutional aplastic anemia

Approximate Synonyms

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

• Autosomal dominant aplasia and myelodysplasia
• Complete deafness
• Congenital deafness
• Congenital progressive bone marrow failure, B-cell immunodeficiency, skeletal dysplasia syndrome
• Constitutional aplastic anemia
• Constitutional aplastic anemia
• Constitutional aplastic anemia
• Constitutional aplastic anemia
• Constitutional aplastic anemia
• Constitutional aplastic anemia with malformation
• Estren-Dameshek anemia
• Fanconi's anemia
• Hereditary isolated aplastic anemia
• Immuno-osseous dysplasia
• Myelodysplastic syndrome with multilineage dysplasia
• Pancytopenia with developmental delay syndrome
• WT limb blood syndrome

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 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 10/01/2022 through 09/30/2023.

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Patient Education

Aplastic Anemia

What is aplastic anemia?

Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make enough new blood cells. It happens when there is damage to stem cells inside your bone marrow. There are different types of aplastic anemia, including Fanconi anemia.

What causes aplastic anemia?

The causes of aplastic anemia can include:

• Autoimmune disorders, which are the most common cause
• Certain inherited gene changes, such as the one that can cause Fanconi anemia
• Toxic substances, such as pesticides, arsenic, and benzene
• Radiation therapy and chemotherapy for cancer
• Certain medicines
• Viral infections such as hepatitis, Epstein-Barr virus, or HIV
• Pregnancy

In many people, the cause is unknown. This is called idiopathic aplastic anemia.

What are the symptoms of aplastic anemia?

Aplastic anemia can develop suddenly or slowly. It can be mild or severe. The symptoms of aplastic anemia can include:

• Fatigue
• Weakness
• Dizziness
• Shortness of breath
• Easy bruising or bleeding

What other problems can aplastic anemia cause?

Aplastic anemia can cause other problems, including frequent infections and bleeding. It raises your risk of developing a serious blood disorder.

If not treated, aplastic anemia can also lead to heart problems such as an arrhythmia (a problem with the rate or rhythm of your heartbeat), an enlarged heart, or heart failure.

How is aplastic anemia diagnosed?

To find out if you have aplastic anemia, your doctor will:

• Take your medical and your family medical histories
• Do a physical exam
• Order tests, such as tests to check if you have low numbers of cells in your bone marrow and blood

What are the treatments for aplastic anemia?

If you have aplastic anemia, your doctor will create a treatment plan for you. The plan will be based on how severe the anemia is and what is causing it. Treatments can include:

• Blood transfusions
• Blood and marrow stem cell transplants
• Medicines to suppress your immune system

Because of the risk of blood disorders, your doctor will monitor your condition and screen you for blood disorders regularly.

NIH: National Heart, Lung, and Blood Institute

Fanconi anemia

Fanconi anemia is a condition that affects many parts of the body. People with this condition may have bone marrow failure, physical abnormalities, organ defects, and an increased risk of certain cancers.

The major function of bone marrow is to produce new blood cells. These include red blood cells, which carry oxygen to the body's tissues; white blood cells, which fight infections; and platelets, which are necessary for normal blood clotting. Approximately 90 percent of people with Fanconi anemia have impaired bone marrow function that leads to a decrease in the production of all blood cells (aplastic anemia). Affected individuals experience extreme tiredness (fatigue) due to low numbers of red blood cells (anemia), frequent infections due to low numbers of white blood cells (neutropenia), and clotting problems due to low numbers of platelets (thrombocytopenia). People with Fanconi anemia may also develop myelodysplastic syndrome, a condition in which immature blood cells fail to develop normally.

More than half of people with Fanconi anemia have physical abnormalities. These abnormalities can involve irregular skin coloring such as unusually light-colored skin (hypopigmentation) or café-au-lait spots, which are flat patches on the skin that are darker than the surrounding area. Other possible symptoms of Fanconi anemia include malformed thumbs or forearms and other skeletal problems including short stature; malformed or absent kidneys and other defects of the urinary tract; gastrointestinal abnormalities; heart defects; eye abnormalities such as small or abnormally shaped eyes; and malformed ears and hearing loss. People with this condition may have abnormal genitalia or malformations of the reproductive system. As a result, most affected males and about half of affected females cannot have biological children (are infertile). Additional signs and symptoms can include abnormalities of the brain and spinal cord (central nervous system), including increased fluid in the center of the brain (hydrocephalus) or an unusually small head size (microcephaly).

Individuals with Fanconi anemia have an increased risk of developing a cancer of blood-forming cells in the bone marrow called acute myeloid leukemia (AML) or tumors of the head, neck, skin, gastrointestinal system, or genital tract. The likelihood of developing one of these cancers in people with Fanconi anemia is between 10 and 30 percent.

Code History

• 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 (First year ICD-10-CM implemented into the HIPAA code set)