Hairy cell leukemia medical therapy: Difference between revisions
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==Overview== | ==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]]. | 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== | ==Medical Therapy== | ||
* There is no '''curative''' treatment for hairy cell leukemia.<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref> | * There is no '''curative''' treatment for hairy cell leukemia.<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref> | ||
* 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.<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref> | * Asymptomatic hairy cell leukemia patients, with no indications for therapy, may be managed by observation and close follow-up.<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref> | ||
| 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:<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | * Pharmacological agents used for the treatment of hairy cell leukemia patients include:<ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | ||
:* [[Cladribine]] | :* [[Cladribine]] | ||
:* [[Pentostatin]] | :* [[Pentostatin]] | ||
| Line 25: | Line 25: | ||
===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]].<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | * The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either [[cladribine]] or [[pentostatin]].<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | ||
* [[Cladribine]] is administered by a single daily IV infusion for a period of 5-7 days.<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref> | * [[Cladribine]] is administered by a single daily IV infusion for a period of 5-7 days.<ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref> | ||
* [[Pentostatin]] is administered by a single IV infusion every 2 weeks for a period of 3-6 months.<ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | * [[Pentostatin]] is administered by a single IV infusion every 2 weeks for a period of 3-6 months.<ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | ||
* Common side effects of such agents may include: | * Common side effects of such agents may include: | ||
| Line 40: | Line 40: | ||
===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.<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | * The optimal therapy for patients who relapse after a complete response depends on the duration of disease-free period following the initial medical therapy.<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | ||
* Hairy cell leukemia patients who relapse '''after''' one year or more are be managed by the '''same''' initial [[purine]] analogue {{withorwithout}} [[rituximab]].<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref> | * Hairy cell leukemia patients who relapse '''after''' one year or more are be managed by the '''same''' initial [[purine]] analogue {{withorwithout}} [[rituximab]].<ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref> | ||
* 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:<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | * Hairy cell leukemia patients who '''do not demonstrate''' a complete response to medical therapy could be further managed by any of the following agents:<ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | ||
:* [[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.<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | * Rituximab is administered by a single IV infusion every week for a period of 8 weeks.<ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref> | ||
* [[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 03:17, 28 October 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.[1][2]
- 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.[1]
- 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
First Line Therapy
- The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either cladribine or pentostatin.[1][3]
- Cladribine is administered by a single daily IV infusion for a period of 5-7 days.[2]
- Pentostatin is administered by a single IV infusion every 2 weeks for a period of 3-6 months.[3]
- 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.[1][3]
- 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.[1][3]
- Hairy cell leukemia patients who relapse after one year or more are be managed by the same initial purine analogue ± rituximab.[2]
- 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:[1][2][3]
- 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.[2][3]
- 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.[4]
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
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015
- ↑ 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.