Sandbox: HCL therapy: Difference between revisions

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==Overview==
<br>
==Medical Therapy==
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* There is no '''curative''' treatment for hairy cell leukemia.<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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* 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.
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* Indications to initiate medical therapy among patients with hairy cell leukemia include:
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:* 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]]
{{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
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:* Hemoglobin concentration lower than 12 g/dl
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:* Platelets count lower than 100,000/mcl
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:* Absolute neutrophils count lower than 1000/mcl
'''[[Cladribine]]'''<br>
* Pharmacological agents used for the treatment of hairy cell leukemia patients include:
'''[[Pentostatin]]'''</div>|C02=<div style="width: 15em; padding:1em;text-align:center">'''Patients managed by observation and close follow-up'''</div>}}
:* [[Cladribine]]  
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:* [[Pentostatin]]
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:* [[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]]
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:* [[Vemurafenib]]
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===First Line Therapy===
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* The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either [[cladribine]] or [[pentostatin]].
'''[[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:
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:* Immune suppression
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:* 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.
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* 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
{{familytree | | | | | | | | | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:lef">'''[[Vemurafenib]]'''</div>}}
:* The absence of [[splenomegaly]] on physical exam
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:* Recovery of the patients blood counts to the normal limits
<br>
:* 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 {{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===
* 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 {{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>
* [[Interferon alpha]] is administered by a single IV infusion (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 [[vemurafenib]].

Latest revision as of 02:16, 30 October 2015


 
 
 
 
 
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
Interferon alpha alone

Alternative purine analogue ± rituximab
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Relapse after one year: same initial purine analogue ± rituximab

Relapse before one year: alternative purine analogue ± rituximab
 
No evidence of complete response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 


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