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| ==Overview== | | <br> |
| 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]].<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><ref>{{cite journal |vauthors=Cornet E, Damaj G, Troussard X |title=New insights in the management of patients with hairy cell leukemia |journal=Curr Opin Oncol |volume=27 |issue=5 |pages=371–6 |year=2015 |pmid=26154707 |doi=10.1097/CCO.0000000000000214 |url=}}</ref>
| | {{familytree/start |summary=PE diagnosis Algorithm.}} |
| | | {{familytree | | | | | | A02 | | | | | |A02=<div style="width: 10em; padding:0.2em;">'''Initial patients evaluation'''</div>}} |
| ==Medical Therapy== | | {{familytree|boxstyle= border-top: 0px;| | | | | | A01 | | | | | | |A01=<div style="width: 15em; padding:1em;text-align:center">History<br>Physical examination<br>Complete blood count</div>}} |
| * There is no complete '''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>
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| * The mainstay of therapy for hairy cell leukemia patients is chemotherapy.
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| * 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>
| | {{familytree | | | | |,|-|^|-|.| | | | | | }} |
| * Indications to initiate medical therapy among patients with hairy cell leukemia include:
| | {{familytree | | | | B02 | |B01| | |B02=<div style="width: 10em; padding:1em;text-align:center">'''Asymptomatic patients with no therapeutic indications'''</div>|B01=<div style="width: 15em; padding:1em;text-align:center">'''Symptomatic patients or evidence of therapeutic indications '''</div>}} |
| :* The presence of systemic symptoms such as fever, night sweats, and significant weight loss
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| :* The presence of subcostal abdominal discomfort due to [[splenomegaly]]
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| :* A positive history of recurrent infections
| | {{familytree | | | | C02 | | C01| | |C01=<div style="width: 10em; padding:1em;"> |
| :* Hemoglobin concentration lower than 12 g/dl
| | '''[[Cladribine]]'''<br> |
| :* Platelets count lower than 100,000/mcl
| | '''[[Pentostatin]]'''</div>|C02=<div style="width: 15em; padding:1em;text-align:center">'''Patients managed by observation and close follow-up'''</div>}} |
| :* Absolute neutrophils count lower than 1000/mcl
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| * 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>
| | {{familytree | | | | | | |,|-|^|-|.| | | | | | | | | | | }} |
| :* [[Cladribine]]
| | {{familytree | | | | | | D02 | | D01 | | | | | | | | | |D01=<div style="width: 15em; padding:1em;text-align:center">'''No evidence of complete response'''</div>|D02=<div style="width: 15em; padding:1em;text-align:center">'''Complete response'''</div>}} |
| :* [[Pentostatin]]
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| :* [[Rituximab]]
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| :* [[Interferon alpha]]
| | {{familytree | | | | | | E02 | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:center"> |
| :* [[Vemurafenib]]
| | '''[[Retuximab]] alone'''<br> |
| ===First Line Therapy===
| | '''[[Interferon alpha]] alone'''<br> |
| * 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>
| | '''Alternative purine analogue {{withorwithout}} rituximab'''</div>|E02=<div style="width: 13em; padding:1em;text-align:center">'''Follow-up and close observation'''</div>}} |
| * 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>
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| * 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>
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| * Common side effects of such agents may include:
| | {{familytree | | | | | | E02 | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:center">'''No evidence of complete response'''</div>|E02=<div style="width: 13em; padding:1em;"> |
| :* Immune suppression
| | '''Relapse after one year: same initial purine analogue ± rituximab'''<br> |
| :* Acute kidney failure
| | '''Relapse before one year: alternative purine analogue ± rituximab'''</div>}} |
| :* Fatigue
| | {{familytree | | | | | | | | | | |!| | | | | | | | | | | }} |
| :* High fever
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| * 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.<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>
| | {{familytree | | | | | | | | | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:lef">'''[[Vemurafenib]]'''</div>}} |
| * A complete response to medical therapy among patients with hairy cell leukemia is defined by:
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| :* Resolution of the patient's symptoms
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| :* The absence of [[splenomegaly]] on physical exam
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| :* Recovery of the patients blood counts to the normal limits
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| :* The absence of malignant leukemic cells on blood smear or bone marrow aspiration
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| ===Relapsed Therapy===
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| * 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>
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| * 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>
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| * Whereas hairy cell leukemia patients who relapse '''before''' a period of one year are managed by an '''alternative''' purine analogue {{withorwithout}} [[rituximab]].
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| ===Refractory Therapy===
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| * 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>
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| :* [[Rituximab]] alone
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| :* [[Interferon alpha]] alone
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| :* An alternate purine analogue {{withorwithout}} [[rituximab]]
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| * 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>
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| * [[Interferon alpha]] is administered subcutaneously (3 million units) three times a week for a period of 12-18 months.
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| * The major side effect of rituximab treatment is serum sickness, whereas the major side effects of interferon alpha are flu-like symptoms and depression.
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| * 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]].<ref>{{cite journal |vauthors=Cornet E, Damaj G, Troussard X |title=New insights in the management of patients with hairy cell leukemia |journal=Curr Opin Oncol |volume=27 |issue=5 |pages=371–6 |year=2015 |pmid=26154707 |doi=10.1097/CCO.0000000000000214 |url=}}</ref>
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