Hairy Cell Leukemia (HCL); Symptoms, Diagnosis , Prognosis & Treatment

Hairy cell leukemia treatment

Hairy cell leukemia treatment

The treatment approach for hairy cell leukemia depends on the stability and the symptoms of the patient. Patients with stable cell counts in their complete blood test might benefit from a conservative stay-and-watch approach using continuous blood tests and recurrent physical examinations to determine whether or not it is necessary to start treatment. In some cases, patients will remain asymptomatic over the course of the disease, and some of them never start therapy, especially in the case of elderly men who are diagnosed in later years.

However, I when the blood cell count shows persistently declining numbers and the patient starts displaying symptoms, patients might start therapy with purine analogs to avoid reaching very low levels of pancytopenia. During the first stage of therapy, it is possible that blood cell counts become lower, so it is recommended to follow-up patients closely and start treatment before blood values are excessively low.

The most commonly prescribed medications are cladribine and pentostatin, and they are often used in monotherapy. The both have similar side effects, and they are equally effective in improving the symptoms and increasing the duration of remission. Monitoring during treatment is recommended to keep track of adverse effects. Other medications that might be used include a granulocyte colony-stimulating factor to increase the neutrophil count, but the clinical advantage of using this drug is not yet clear.

Additionally, treatment of patients with hairy cell leukemia should include follow-up of the renal function and early detection and treatment of infections. Antibiotic and antifungal treatments are usually required at some point in the disease, and pentostatin is the most nephrotoxic drug and requires monitoring of the renal function. In the case of using pentostatin, it is important to increase fluid intake and perform continuous tests of serum creatinine.

Some patients with hairy cell leukemia may need splenectomy, surgical resection of the spleen. This is only required in patients that do not respond to treatment and those with severe thrombocytopenia and hemorrhagic episodes. The most appropriate surgical method is laparoscopy, and after this procedure, up to 70% of patients improve their cell counts in all three bloodlines.

References

Cannon, T., Mobarek, D., Wegge, J., & Tabbara, I. A. (2008). Hairy cell leukemia: current concepts. Cancer investigation, 26(8), 860-865.

Tiacci, E., Trifonov, V., Schiavoni, G., Holmes, A., Kern, W., Martelli, M. P., … & Sportoletti, P. (2011). BRAF mutations in hairy-cell leukemia. New England Journal of Medicine, 364(24), 2305-2315.

Tiacci, E., Pettirossi, V., Schiavoni, G., & Falini, B. (2017). Genomics of hairy cell leukemia. Journal of Clinical Oncology, 35(9), 1002.

Getta, B. M., Woo, K. M., Devlin, S., Park, J. H., Abdel‐Wahab, O., Saven, A., … & Tallman, M. S. (2016). Treatment outcomes and secondary cancer incidence in young patients with hairy cell leukaemia. British journal of haematology, 175(3), 402-409.

Troussard, X., & Cornet, E. (2017). Hairy cell leukemia 2018: Update on diagnosis, risk‐stratification, and treatment. American journal of hematology, 92(12), 1382.