Sandbox: HCL therapy
Overview
Medical Therapy
- The mainstay of therapy for hairy cell leukemia patients is chemotherapy.
- Asymptomatic hairy cell leukemia patients, with no indications for therapy, may be managed by observation and close follow-up.
- Indications to initiate medical therapy among patients with hairy cell leukemia include:
- The presence of systemic symptoms such as fever, night sweats, and significant weight loss
- The presence of subcostal abdominal discomfort due to splenomegaly
- A positive history of recurrent infections
- Hemoglobin concentration lower than 12 g/dl
- Platelets count lower than 100,000/mcl
- Absolute neutrophils count lower than 1000/mcl
- Pharmacological agents used for the treatment of hairy cell leukemia patients include:
First Line Therapy
- Preferred pharmacological agents used for the initial management of hairy cell leukemia could be either cladribine or pentostatin.
- Hairy cell leukemia patients who demonstrate a complete response to the initial medical therapy should be managed by follow-up and close observation for any signs of relapse.
- Hairy cell leukemia patients who do not demonstrate a complete response to medical therapy could be further managed by any of the following agents:
- Rituximab alone
- Interferon alpha alone
- An alternate purine analogue ± rituximab
- Patients with progressive hairy cell leukemia who do not demonstrate a complete response to any of the aforementioned medical therapies should be managed with vemurafenib.
- A complete response to medical therapy among patients with hairy cell leukemia is defined by:
- Resolution of the patient's symptoms
- The absence of splenomegaly on physical exam
- Recovery of the patients blood counts to the normal limits
- The absence of malignant leukemic cells on blood smear or bone marrow aspiration
Refractory/Relapsed Therapy
- The optimal therapy for patients who relapse after a complete response depends on the duration of disease-free period following the initial medical therapy.
- Hairy cell leukemia patients who relapse after one year or more are be managed by the same initial purine analogue ± rituximab.
- Whereas hairy cell leukemia patients who relapse before a period of one year are managed by an alternative purine analogue ± rituximab.