Hairy cell leukemia medical therapy: Difference between revisions
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==Medical Therapy== | ==Medical Therapy== | ||
* There is no '''curative''' treatment for hairy cell leukemia. | * There is no '''curative''' treatment for hairy cell leukemia. | ||
* The mainstay of therapy for hairy cell leukemia patients is [[chemotherapy]]. | * 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. | * 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: | * 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 systemic symptoms such as [[fever]], [[night sweats]], and significant [[weight loss]] | ||
| Line 17: | Line 17: | ||
:* [[Platelets]] count lower than 100,000/μL | :* [[Platelets]] count lower than 100,000/μL | ||
:* Absolute [[neutrophils]] count lower than 1000/μL | :* Absolute [[neutrophils]] count lower than 1000/μL | ||
* Pharmacological agents used for the treatment of hairy cell leukemia patients include: | * Pharmacological agents used for the treatment of hairy cell leukemia patients include: | ||
:* [[Cladribine]] | :* [[Cladribine]] | ||
:* [[Pentostatin]] | :* [[Pentostatin]] | ||
| Line 24: | Line 24: | ||
:* [[Vemurafenib]] | :* [[Vemurafenib]] | ||
===First Line Therapy=== | ===First Line Therapy=== | ||
* The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either [[cladribine]] or [[pentostatin]]. | * The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either [[cladribine]] or [[pentostatin]]. | ||
* [[Cladribine]] is administered by a single daily IV infusion for a period of 5-7 days. | * [[Cladribine]] is administered by a single daily IV infusion for a period of 5-7 days. | ||
* [[Pentostatin]] is administered by a single IV infusion every 2 weeks for a period of 3-6 months. | * [[Pentostatin]] is administered by a single IV infusion every 2 weeks for a period of 3-6 months. | ||
* Common side effects of such agents may include: | * Common side effects of such agents may include: | ||
:* [[Immune suppression]] | :* [[Immune suppression]] | ||
| Line 32: | Line 32: | ||
:* [[Fatigue]] | :* [[Fatigue]] | ||
:* High [[fever]] | :* High [[fever]] | ||
* Hairy cell leukemia patients who '''demonstrate''' a complete response following initial medical therapy should be followed-up with close observation for any signs of relapse. | * Hairy cell leukemia patients who '''demonstrate''' a complete response following initial medical therapy should be followed-up with close observation for any signs of relapse. | ||
* A complete response to medical therapy among patients with hairy cell leukemia is defined by: | * A complete response to medical therapy among patients with hairy cell leukemia is defined by: | ||
:* Resolution of the patient's symptoms | :* Resolution of the patient's symptoms | ||
| Line 39: | Line 39: | ||
:* The absence of malignant leukemic cells on blood smear or [[bone marrow]] aspiration | :* The absence of malignant leukemic cells on blood smear or [[bone marrow]] aspiration | ||
===Relapsed Therapy=== | ===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. | * 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 {{withorwithout}} [[rituximab]]. | * Hairy cell leukemia patients who relapse '''after''' one year or more are be managed by the '''same''' initial [[purine]] analogue {{withorwithout}} [[rituximab]]. | ||
* Whereas hairy cell leukemia patients who relapse '''before''' a period of one year are managed by an '''alternative''' [[purine]] analogue {{withorwithout}} [[rituximab]]. | * Whereas hairy cell leukemia patients who relapse '''before''' a period of one year are managed by an '''alternative''' [[purine]] analogue {{withorwithout}} [[rituximab]]. | ||
===Refractory Therapy=== | ===Refractory Therapy=== | ||
* Hairy cell leukemia patients who '''do not demonstrate''' a complete response to medical therapy could be further managed by any of the following agents: | * 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 | :* [[Rituximab]] alone | ||
:* [[Interferon alpha]] alone | :* [[Interferon alpha]] alone | ||
:* An alternate purine analogue {{withorwithout}} [[rituximab]] | :* An alternate purine analogue {{withorwithout}} [[rituximab]] | ||
* Rituximab is administered by a single IV infusion every week for a period of 8 weeks. | * Rituximab is administered by a single IV infusion every week for a period of 8 weeks. | ||
* [[Interferon alpha]] is administered subcutaneously (3 million units) three times a week for a period of 12-18 months. | * [[Interferon alpha]] is administered subcutaneously (3 million units) three times a week for a period of 12-18 months. | ||
* The major side effect of rituximab treatment is [[serum sickness]], whereas the major side effects of interferon alpha are flu-like symptoms and [[depression]]. | * The major side effect of rituximab treatment is [[serum sickness]], whereas the major side effects of interferon alpha are flu-like symptoms and [[depression]]. | ||
Revision as of 17:34, 20 November 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
There is no curative treatment for hairy cell leukemia. The mainstay of therapy for hairy cell leukemia patients is chemotherapy. Pharmacological agents used for the treatment of hairy cell leukemia patients include cladribine, pentostatin, rituximab, and vemurafenib.
Medical Therapy
- There is no curative treatment for hairy cell leukemia.
- 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/μL
- Absolute neutrophils count lower than 1000/μL
- Pharmacological agents used for the treatment of hairy cell leukemia patients include:
First Line Therapy
- The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either cladribine or pentostatin.
- Cladribine is administered by a single daily IV infusion for a period of 5-7 days.
- Pentostatin is administered by a single IV infusion every 2 weeks for a period of 3-6 months.
- Common side effects of such agents may include:
- Hairy cell leukemia patients who demonstrate a complete response following initial medical therapy should be followed-up with close observation for any signs of relapse.
- 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
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.
Refractory Therapy
- 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
- Rituximab is administered by a single IV infusion every week for a period of 8 weeks.
- Interferon alpha is administered subcutaneously (3 million units) three times a week for a period of 12-18 months.
- The major side effect of rituximab treatment is serum sickness, whereas the major side effects of interferon alpha are flu-like symptoms and depression.
- Patients with progressive hairy cell leukemia who do not demonstrate a complete response to any of the aforementioned medical therapies should be managed with a BRAF kinase inhibitor, such as vemurafenib.[1]
The algorithm below summarizes the management approach for hairy cell leukemia patients:
Initial patients evaluation | |||||||||||||||||||||||||||
History Physical examination Complete blood count | |||||||||||||||||||||||||||
Asymptomatic patients with no therapeutic indications | Symptomatic patients or evidence of therapeutic indications | ||||||||||||||||||||||||||
Patients managed by observation and close follow-up | |||||||||||||||||||||||||||
Complete response | No evidence of complete response | ||||||||||||||||||||||||||
Follow-up and close observation | Retuximab alone | ||||||||||||||||||||||||||
Relapse after one year: same initial purine analogue ± rituximab | No evidence of complete response | ||||||||||||||||||||||||||
References
- ↑ Cornet E, Damaj G, Troussard X (2015). "New insights in the management of patients with hairy cell leukemia". Curr Opin Oncol. 27 (5): 371–6. doi:10.1097/CCO.0000000000000214. PMID 26154707.