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==Overview==
<br>
==Medical Therapy==
{{familytree/start |summary=PE diagnosis Algorithm.}}
* The mainstay of therapy for hairy cell leukemia patients is chemotherapy.
{{familytree | | | | | | A02 | | | | | |A02=<div style="width: 10em; padding:0.2em;">'''Initial patients evaluation'''</div>}}
* Asymptomatic hairy cell leukemia patients, with no indications for therapy, may be managed by observation and close follow-up.
{{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>}}
* 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]]
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:* A positive history of recurrent infections
{{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>}}
:* 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
{{familytree | | | | C02 | | C01| | |C01=<div style="width: 10em; padding:1em;">
* Pharmacological agents used for the treatment of hairy cell leukemia patients include:
'''[[Cladribine]]'''<br>
:* [[Cladribine]]  
'''[[Pentostatin]]'''</div>|C02=<div style="width: 15em; padding:1em;text-align:center">'''Patients managed by observation and close follow-up'''</div>}}
:* [[Pentostatin]]
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:* [[Rituximab]]
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:* [[Interferon alpha]]
{{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>}}
:* [[Vemurafenib]]
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===First Line Therapy===
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* Preferred pharmacological agents used for the initial management of hairy cell leukemia could be either [[cladribine]] or [[pentostatin]].
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* Hairy cell leukemia patients who '''demonstrate''' a complete response to the initial medical therapy should be managed by follow-up and close observation for any signs of relapse.
'''[[Retuximab]] alone'''<br>
* Hairy cell leukemia patients who '''do not demonstrate''' a complete response to medical therapy could be further managed by any of the following agents:
'''[[Interferon alpha]] alone'''<br>
:* [[Rituximab]] alone
'''Alternative purine analogue {{withorwithout}} rituximab'''</div>|E02=<div style="width: 13em; padding:1em;text-align:center">'''Follow-up and close observation'''</div>}}
:* [[Interferon alpha]] alone
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:* An alternate purine analogue {{withorwithout}} [[rituximab]]
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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 [[vemurafenib]].
{{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;">
* A complete response to medical therapy among patients with hairy cell leukemia is defined by:
'''Relapse after one year: same initial purine analogue ± rituximab'''<br>
:* Resolution of the patient's symptoms
'''Relapse before one year: alternative purine analogue ± rituximab'''</div>}}
:* 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
{{familytree | | | | | | | | | | E01 | | | | | | | | | | |E01=<div style="width: 17em; padding:1em;text-align:lef">'''[[Vemurafenib]]'''</div>}}
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<br>

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