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Hint: Know your anemia types.

Look up anemia in the ICD-10-CM Alphabetic Index, and you’re faced with a bewildering number of potential code options. Altogether, the Index contains codes for over 300 conditions under the main entry Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) and (profound).

But if you understand the five different forms of anemia identified by the American Society of Hematology (ASH), you’ll to narrow down your code selection quickly and easily. Here’s what you need to know to do that.

First, Know What to Do Before Definitive Dx Is Established

Labs frequently use the following codes to justify medical necessity for testing and for any abnormal findings when a provider has yet to establish a definitive diagnosis: 

Screening codes: You would use Z13.0 (Encounter for screening for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism) on encounters that screen for diseases of the blood and immune mechanism in asymptomatic patients. For patients with a personal history of blood or immune disorders, you would use Z86.2 (Personal history of diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism).

Abnormal blood findings: These can be found in the Abnormal findings on examination of blood, without diagnosis (R70-R79) codes, particularly:

• R71.8 (Other abnormality of red blood cells) such as anisocytosis and poikilocytosis
• R79.1 (Abnormal coagulation profile)
• R79.89 (Other specified abnormal findings of blood chemistry)
• R79.9 (Abnormal finding of blood chemistry, unspecified)

Then Understand the Anemia Types and Their Related ICD-10-CM Codes

Altogether, the ASH identifies five different types of anemia:

Iron-deficiency anemia is “the most common form of anemia,” according to the ASH. It occurs when someone does not produce enough iron — and, therefore, not enough red blood cells (RBCs) — due to a poor diet or loss of blood. Patients can also develop anemia if they need more RBCs than their body is currently producing, which happens during adolescent growth spurts and pregnancy.

Coding options: This is the easiest form of anemia to code. You’ll find all the codes you need in the D50.- (Iron deficiency anemia) group. Just be sure to pay attention to the Excludes1 note accompanying D50.0 (Iron deficiency anemia secondary to blood loss (chronic)), which tells you to code D62 (Acute posthemorrhagic anemia) or P61.3 (Congenital anemia from fetal blood loss) instead if your provider documents these types of blood-loss anemia.

Vitamin-deficiency anemia is also the result of a poor diet, in this case a diet deficient in folic acid or vitamin B12. A specific vitamin-deficiency anemia, known as pernicious anemia, occurs due to an issue with absorption of vitamin B12.

Coding options: The vitamin-deficiency anemia codes are similarly easy to deal with. You’ll use a code from D51.- (Vitamin B12 deficiency anemia) or D52.- (Folate deficiency anemia) as appropriate. For pernicious anemia, you’ll use D51.0 (Vitamin B12 deficiency anemia due to intrinsic factor deficiency).

Don’t forget: For D52.1 (Drug-induced folate deficiency anemia), you’ll need to apply an additional code from the T36-T50 (Poisoning by, adverse effect of and underdosing of drugs, medicaments and biological substances) group to identify the drug responsible for a patient’s anemia if it is caused by a drug.

Aplastic anemia is due to bone marrow disorders, which can either be genetic or caused by toxins or medications, such as chemotherapy or radiation therapy.

Coding options: Coding for the various aplastic anemias is challenging because there are plenty of instructions to which you need to pay attention.

For example, D63.0 (Anemia in neoplastic disease) has a Code first instruction telling you to code the associated neoplasm first, an Excludes1 instruction telling you to code D61.1 (Aplastic anemia due to antineoplastic chemotherapy) if the provider documents D63.0 and D61.1 together, and an Excludes2 instruction allowing you to code D63.0 along with D64.81 (Anemia due to antineoplastic chemotherapy) if the provider documents both.

The code is also subject to two ICD-10-CM sequencing guidelines — I.C.2.c.1 and I.C.2.c.2 — that instruct you to sequence first the code best associated with the admission/encounter for which the patient is being treated.

Hemolytic anemia can be acquired or inherited. People with hemolytic anemia cannot produce RBCs fast enough to replace ones that die off, which typically occurs over a 120-day period.

Coding options: To select the most appropriate hemolytic anemia code, you’ll need to walk through the maze of multiple diagnosis codes in the D55-D59 section of ICD-10-CM. That means knowing the inclusion terms. Knowing that your provider could use the terms alpha thalassemia major, hemoglobin H constant spring, hemoglobin H disease, hydrops fetalis due to alpha thalassemia, severe alpha thalassemia, or even triple gene defect alpha thalassemia as synonyms for alpha thalassemia, for example, will enable you to use D56.0 (Alpha thalassemia) correctly.

Remember: Though it is rare, patients undergoing antineoplastic therapy can acquire hemolytic anemia, which is why D59.0 (Drug-induced autoimmune hemolytic anemia) carries with it the same Use additional code instruction you see with other drug-induced anemias.

Sickle cell anemia is a type of inherited hemolytic anemia. It is the most common type of sickle-cell disorder (SCD) known as HbSS, a genetic disorder that causes red blood cells to die off quickly, producing abnormal hemoglobin (Hb) levels; or that causes the cells to block blood vessels completely, creating complications such as acute pain crises. Code HbSS with D57.0- (Hb-SS disease with crisis) or D57.1 (Sickle-cell disease without crisis) depending on the associated conditions.

Coding for SCD crisis: Also known as a vaso-occlusive crisis, this is one of the disease’s most serious complications. The pain, which can affect “the abdomen, chest, lower back, or arms and legs,” can be “triggered by high altitudes, dehydration, illness, stress, or temperature changes,” though “often a person does not know what triggers the crisis,” according to the National Heart, Lung, and Blood Institute (NHLBI).

Without crisis: If the patient is not undergoing an SCD crisis, your code choice is simply D57.1.

With crisis: Coding choices in this situation are a little more complicated and require a provider to give more information about the specific acute condition the patient is experiencing. You’ll begin by adding the 5^th character “1” to indicate the patient is undergoing a crisis, then you’ll add the appropriate 6^th character to specify the nature of the crisis:

• Acute chest syndrome (6^th character “1”).
• Splenic sequestration (6^th character “2”).
• Cerebral vascular involvement (6^th character “3”). Remember, follow the Code also instruction, which tells you to add a code from the I63.- (Cerebral infarction) group if applicable.
• Dactylitis (6^th character “4”).
• Other specified complications (6^th character “8”). Pay attention to the Use additional code instruction, which tells you to identify complications, such as K80.- (Cholelithiasis) or N48.32 (Priapism due to disease classified elsewhere).
• Unspecified (6^th character “9”). pay attention to the inclusion terms for D57.819 (Other sickle-cell disorders with crisis, unspecified), which tell you to code vaso-occlusive pain not otherwise specified to D57.819.

Bruce Pegg, BA, MA, CPC, CFPC, Managing Editor, AAPC