Cure What Ails Your Chronic Lymphocytic Leukemia Coding
From ICD-10-CM to CPT® to HCPCS Level II, capture correct codes in your CLL claims.
Chronic lymphocytic leukemia (CLL), reported using ICD-10-CM code C91.10 Chronic lymphocytic leukemia of B-cell type not having achieved remission, is the most common type of adult leukemia in the western world. Here’s what you should know to properly code the condition, testing, and treatment.
CLL is typically a B-cell lymphocytic disorder, or rarely of T-cell lymphocyte origin. In simplified terms, B-cells produce antibodies that bind to antigens and neutralize them. T-cells help to remove good cells that are already infected.
There is no clear definitive sign or symptom that would indicate a diagnosis of CLL. The diagnosis is made based on a combination of signs and symptoms, followed by laboratory testing. Signs and symptoms commonly associated with CLL include:
- Weakness –M62.81 Muscle weakness (generalized)
- Fatigue – R53.83 Other fatigue
- Weight loss – R63.4 Abnormal weight loss
- Chills – R68.83 Chills (without fever)
- Fever – R50.9 Fever, unspecified
- Night sweats – R61 Generalized hyperhidrosis
- Swollen lymph nodes – R59.9 Enlarged lymph nodes, unspecified
- Hepatomegaly – R16.0 Hepatomegaly, not elsewhere classified
- Splenomegaly – R16.1 Splenomegaly, not elsewhere classified
The survival rate for CLL varies by staging, but the average five-year survival rate for CLL patients is 83 percent.
Staging is used to help predict CLL progression and for development of treatment plans. Two staging systems are used by physicians: the Rai staging system and the Binet staging system (see Table A and Table B).
Table A: The Rai staging system classifies CLL into one of five stages based on factors at the time of diagnosis.
|0||Low||Abnormal increase in the number of lymphocytes in the blood and marrow. The diagnosis of CLL requires the presence of at least 5,000 clonal B cells per microliter in peripheral blood. Associated with a good outcome.|
|I||Intermediate||Abnormal increase in the number of lymphocytes in the blood and marrow and enlarged (swollen) lymph nodes. Associated with a good outcome.|
|II||Intermediate||Abnormal increase in the number of lymphocytes in the blood and marrow and enlarged (swollen) lymph nodes, liver, or spleen. Associated with a poor outcome.|
|III||High||Abnormal increase in the number of lymphocytes in the blood and marrow and anemia. Associated with a poor outcome.|
|IV||High||Abnormal increase in the number of lymphocytes in the blood and marrow and low platelet count. Associated with a poor outcome.|
Table B: The Binet staging system classifies CLL into one of three stages.
|A Stage||Abnormal increase in the number of lymphocytes in the blood and less than three swollen lymph nodes that can be felt under the skin. Associated with a good outcome.|
|B Stage||Abnormal increase in the number of lymphocytes in the blood and three or more swollen lymph nodes that can be felt under the skin. Associated with a poor outcome.|
|C Stage||Abnormal increase in the number of lymphocytes in the blood, three or more swollen lymph nodes that can be felt under the skin, and anemia or low platelet count. Associated with a poor outcome.|
Additional testing used to assess a CLL prognosis, and often associated with faster-growing leukemias, include:
Blood lymphocyte doubling time is defined as the period needed for lymphocytes to double in number. This is a calculation based on multiple absolute lymphocyte counts (85025 Blood count; complete (CBC), automated (Hgb, Hct, RBC, WBC and platelet count) and automated differential WBC count). A lymphocyte doubling time greater than 12 months is associated with a very good prognosis; whereas, a lymphocyte doubling time less than or equal to 12 months is associated with a poor prognosis and rapid disease progression.
Beta 2-microglobulin (82232 Beta-2 microglobulin): High beta 2-microglobulin in CLL has been found to correlate mainly with advanced stage of the disease and high lymphocyte count and is routinely used as a marker for poor prognosis. Levels greater than two times the upper limit of normal are associated with a less than 10-year median survival chance.
CD38 expression (88342 Immunohistochemistry or immunocytochemistry, per specimen; initial single antibody stain procedure) of less than 30 percent is associated with a good outcome. Greater than or equal to 30 percent is associated with a poor outcome. Low numbers of CD38 with a positive immunoglobulin variable heavy chain (IgVH) gene mutation are associated with a good prognosis.
IgVH gene mutation (81263 IGH@ (Immunoglobulin heavy chain locus) (eg, leukemia and lymphoma, B-cell), variable region somatic mutation analysis) is a significant prognostic marker in CLL. Greater than 2 percent is associated with a good outcome, while less than or equal to 2 percent is associated with a poor outcome and a significantly shorter complete remission rate.
Zeta-chain Associated Protein (ZAP) 70 (88184 Flow cytometry, cell surface, cytoplasmic, or nuclear marker, technical component only; first marker): Greater than or equal to 10 percent ZAP 70 expression in CD5/CD19 positive cells are associated with poor outcome.
NOTCH1 gene mutations (81407 Molecular pathology procedure, Level 8): NOTCH1 mutations in CLL are associated with a poor prognosis.
SF3B1 gene mutations (81479 Unlisted molecular pathology procedure): These mutations occur in 10-15 percent of CLL patients and are associated with a poor prognosis.
TP53 gene mutations (81405 Molecular pathology procedure, Level 6): These mutations are detected in at least 50 percent of all adult tumors, and generally are associated with a poor prognosis.
Initial treatment for CLL will vary on the ability of the patient to tolerate the side effects. In healthy patients, common treatment options include:
- Fludarabine (J9185 Injection, floxuridine, 500 mg), cyclophosphamide (J9070 Cyclophosphamide, 100 mg), and rituximab (J9310 Injection, rituximab, 100 mg)
- Bendamustine (J9033 Injection, bendamustine HCL (treanda), 1 mg)
- Ibrutinib (J8999 Prescription drug, oral, chemotherapeutic, NOS)
- Fludarabine (J9185) and rituximab (J9310)
- High-dose prednisone (J7506 Prednisone, oral, per 5 mg) and rituximab (J9310)
- Pentostatin (J9268 Injection, pentostatin, 10 mg), cyclophosphamide (J9070), and rituximab (J9310)
- Alemtuzumab (J0202 Injection, alemtuzumab, 1 mg) and rituximab (J9310)
An estimated 20,940 people in the United States will be diagnosed with CLL this year, according to Cancer.Net. Chances are good that you’ll code a claim for at least one of them.
American Cancer Society 2017. https://www.lls.org/facts-and-statistics/facts-and-statistics-overview., Accessed May 14, 2018.
American Cancer Society 2017: www.cancer.net/cancer-types/leukemia-chronic-lymphocytic-cll/statistics and www.cancer.org/cancer/chronic-lymphocytic-leukemia/treating/treatment-by-risk-group.html
Leukemia & Lymphoma Society, Facts and Statistics:
Leukemia & Lymphoma Society, CLL Staging:
Vinolas, N, Reverter, JC, Urbano-Ispizua, A, Montserrat E, and Rozman C. (1987) Lymphocyte Doubling time in Chronic Lymphocytic Leukemia: An Update of its Prognostic Significance. Blood Cells. 12(2):457-70.
Durig J, Naschar M, Schmucker U, Renzing-Kohler K, et. al. (Jan. 9, 2002) CD38 Expression is an Important Prognostic Marker in Chronic Lymphocytic Leukemia. Leukemia 16: 30-35.
Lin KI, Tam CS, Keating MJ, et. al. (April 2, 2009) Relevance of the Immunoglobulin VH somatic mutation status in patients with chronic lymphocytic leukemia treated with fludarabine, cyclophosphamide, and rituximab (FCR) or related chemoimmunotherapy regimens. Blood, 113(14): 3168-3171.
NeoGenomics Laboratories, Inc., IgVH Mutation Analysis:
NeoGenomics Laboratories, Inc., NOTCH1 Mutation Analysis:
NeoGenomics Laboratories, Inc., SF3B1 Mutation Analysis:
NeoGenomics Laboratories, Inc., TP53 Mutation Analysis:
Leukemia – Chronic Lymphocytic – CLL: Statistics (2018, January 1). Retrieved from