Oncology & Hematology Coding Alert

Follow grade in MDS, remission and relapse in multiple myeloma, crisis in SCA.

Begin the New Year with a recap of ICD-10 codes for some key hematological diagnoses important for your practice. Make note of parameters you need to look for to make your way to the most precise diagnosis coding.

Check High vs. Low grade in MDS

For high grade myelodysplastic syndrome (MDS), submit code D46.22 (Refractory anemia with excess ofblasts 2).

Excess blasts are key: The descriptor of code D46.22 is very specific as it clearly mentions that there is an excess of blasts. There are more blasts (10-20%) in the bone marrow when compared to refractory anemia with excess of blasts 1 (less than 10%). Hence it is called high grade MDS. According to the American Cancer Society, this type of MDS is about 50% likely to turn into acute myeloid leukemia.

Types and pathophysiology of MDS: The American Cancer Society has clearly explained the pathophysiology and types of MDS. You can read more at: https://www.cancer.org/cancer/myelodysplastic-syndrome/about/mds-types.html. The society describes 7 types of MDS in accordance with the World Health Organization (WHO) classification: Refractory cytopenia with unilineage dysplasia (RCUD), refractory anemia with ringed sideroblasts (RARS), refractory cytopenia with multilineage dysplasia (RCMD), refractory anemia with excess blasts-1 (RAEB-1), refractory anemia with excess blasts-2 (RAEB-2), myelodysplastic syndrome, unclassified (MDS-U), and myelodysplastic syndrome associated with isolated del(5q).

Listed below are more ICD-10 codes for MDS. Note that these apply to low grade MDS.

• D46.0,  Refractory anemia without ring sideroblasts, so stated
• D46.1, Refractory anemia with ring sideroblasts
• D46.20, Refractory anemia with excess of blasts, unspecified
• D46.21, Refractory anemia with excess of blasts 1
• D46.A, Refractory cytopenia with multilineage dysplasia
• D46.B, Refractory cytopenia with multilineage dysplasia and ring sideroblasts
• D46.4, Refractory anemia, unspecified

Check for Remission or Relapse in Multiple Myeloma

When reporting multiple myeloma in ICD-10-CM, you choose diagnosis codes depending upon whether or not your hematologist has documented remission for the patient. Report code C90.00 (Multiple myeloma not having achieved remission) for a patient who has not achieved remission. If, however, you have documentation in support of remission, report code C90.01 (Multiple myeloma in remission). When your hematologist documents a relapse in multiple myeloma, you report code C90.02 (Multiple myeloma in relapse).

Note: The code D47.Z9 (Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue) applies to solitary myeloma. You should not report this code for multiple myeloma. “According the US National Library of Medicine, “solitary myeloma is rare as compared with multiple myeloma, ” says Kelly C. Loya, CPC-I, CHC, CPhT, CRMA, Managing Director of Pinnacle Enterprise Risk Consulting Services, LLC (“PERCS”), a division of Pinnacle Healthcare Consulting.

Concentrate On Crisis in SCA

When sickle cell anemia (SCA) results in crisis, you should first confirm if your provider diagnosed an acute chest syndrome or a splenic sequestration. To choose the most specific ICD-10-CM code, you would need to know if the patient has Hb-SS disease and the nature of the crisis. Without that information you would have to choose the unspecified code. Select from the following codes:

• D57.00, Hb-SS disease with crisis, unspecified
• D57.01, Hb-SS disease with acute chest syndrome
• D57.02, Hb-SS disease with splenic sequestration

Why Hb-SS status? Hb-SS is a sickle cell disease in which the patient has inherited a sickle cell gene (‘S’) from each parent. Sickle cell anemia is another name for this disease.

Crisis in sickle cell anemia: Yale et al. have described various manifestations of vaso-occlusive disease in ‘Approach to the Vaso-occlusive Crisis in Adults with Sickle Cell Disease’ (Am Fam Physician. 2000Mar1;61(5):1349-1356). The occlusion in blood vessels can lead to pain in bones, joints, and other organs. In acute chest syndrome, the sickle shared cells obstruct the pulmonary circulation. In splenic sequestration, the sickle cells trap blood within the spleen causing enlargement of the spleen.

The D57.0- codes: ICD-10-CM will allow you to report both the Hb-SS and the type of crisis (acute chest syndrome or splenic sequestration) using a single code. The D57.0- codes are appropriate for Hb-SS disease with crisis, sickle-cell disease NOS with crisis, and Hb-SS disease with vaso-occlusive pain.

Don’t ignore any additional symptoms:  When reporting D57 codes, ICD-10-CM instructs you to use an additional code for any associated fever (R50.81, Fever presenting with conditions classified elsewhere).