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| ==Overview== | | <br> |
| ==Medical Therapy== | | {{familytree/start |summary=PE diagnosis Algorithm.}} |
| * 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>
| | {{familytree | | | | | | A02 | | | | | |A02=<div style="width: 10em; padding:0.2em;">'''Initial patients evaluation'''</div>}} |
| * The mainstay of therapy for hairy cell leukemia patients is chemotherapy.
| | {{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>}} |
| * 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 | | | | | | |!| | | | | | | | | | | | }} |
| :* The presence of systemic symptoms such as fever, night sweats, and significant weight loss | | {{familytree | | | | |,|-|^|-|.| | | | | | }} |
| :* The presence of subcostal abdominal discomfort due to [[splenomegaly]] | | {{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>}} |
| :* A positive history of recurrent infections
| | {{familytree | | | | |!| | | |!| | | | }} |
| :* Hemoglobin concentration lower than 12 g/dl
| | {{familytree | | | | |!| | | |!| | | | }} |
| :* Platelets count lower than 100,000/mcl
| | {{familytree | | | | C02 | | C01| | |C01=<div style="width: 10em; padding:1em;"> |
| :* Absolute neutrophils count lower than 1000/mcl
| | '''[[Cladribine]]'''<br> |
| * 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>
| | '''[[Pentostatin]]'''</div>|C02=<div style="width: 15em; padding:1em;text-align:center">'''Patients managed by observation and close follow-up'''</div>}} |
| :* [[Cladribine]]
| | {{familytree | | | | | | | | |!| | | | | | | | | | | | | }} |
| :* [[Pentostatin]]
| | {{familytree | | | | | | |,|-|^|-|.| | | | | | | | | | | }} |
| :* [[Rituximab]]
| | {{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>}} |
| :* [[Interferon alpha]]
| | {{familytree | | | | | | |!| | | |!| | | | | | | | | | | }} |
| :* [[Vemurafenib]]
| | {{familytree | | | | | | |!| | | |!| | | | | | | | | | | }} |
| ===First Line Therapy=== | | {{familytree | | | | | | E02 | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:center"> |
| * 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>
| | '''[[Retuximab]] alone'''<br> |
| * 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>
| | '''[[Interferon alpha]] alone'''<br> |
| * Pentostatin is administered by a single IV infusion every 2 weeks for a period of 3-6 months.
| | '''Alternative purine analogue {{withorwithout}} rituximab'''</div>|E02=<div style="width: 13em; padding:1em;text-align:center">'''Follow-up and close observation'''</div>}} |
| * Common side effects of such agents may include:
| | {{familytree | | | | | | |!| | | |!| | | | | | | | | | | }} |
| :* Immune suppression | | {{familytree | | | | | | |!| | | |!| | | | | | | | | | | }} |
| :* Acute kidney failure | | {{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;"> |
| :* Fatigue | | '''Relapse after one year: same initial purine analogue ± rituximab'''<br> |
| :* High fever
| | '''Relapse before one year: alternative purine analogue ± rituximab'''</div>}} |
| * 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>
| | {{familytree | | | | | | | | | | |!| | | | | | | | | | | }} |
| * A complete response to medical therapy among patients with hairy cell leukemia is defined by:
| | {{familytree | | | | | | | | | | |!| | | | | | | | | | | }} |
| :* Resolution of the patient's symptoms
| | {{familytree | | | | | | | | | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:lef">'''[[Vemurafenib]]'''</div>}} |
| :* The absence of [[splenomegaly]] on physical exam
| | {{familytree/end}} |
| :* Recovery of the patients blood counts to the normal limits
| | <br> |
| :* 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>
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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]].
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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=== | |
| * 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>
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| :* [[Rituximab]] alone | |
| :* [[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>
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| * [[Interferon alpha]] is administered by a single IV infusion (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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