Sandbox: HCL therapy: Difference between revisions
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==Overview== | ==Overview== | ||
==Medical Therapy== | ==Medical Therapy== | ||
* 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, | * 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. | * 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> | ||
* Indications to initiate medical therapy among patients with hairy cell leukemia include: | * 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 systemic symptoms such as fever, night sweats, and significant weight loss | ||
| Line 11: | Line 11: | ||
:* Platelets count lower than 100,000/mcl | :* Platelets count lower than 100,000/mcl | ||
:* Absolute neutrophils count lower than 1000/mcl | :* Absolute neutrophils count lower than 1000/mcl | ||
* Pharmacological agents used for the treatment of hairy cell leukemia patients include: | * 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> | ||
:* [[Cladribine]] | :* [[Cladribine]] | ||
:* [[Pentostatin]] | :* [[Pentostatin]] | ||
| Line 18: | Line 18: | ||
:* [[Vemurafenib]] | :* [[Vemurafenib]] | ||
===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]]. | * 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> | ||
* 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. | * Pentostatin is administered by a single IV infusion every 2 weeks for a period of 3-6 months. | ||
| Line 26: | Line 26: | ||
:* Fatigue | :* Fatigue | ||
:* High fever | :* High fever | ||
* 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. | * 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> | ||
* A complete response to medical therapy among patients with hairy cell leukemia is defined by: | * A complete response to medical therapy among patients with hairy cell leukemia is defined by: | ||
:* Resolution of the patient's symptoms | :* Resolution of the patient's symptoms | ||
| Line 33: | Line 33: | ||
:* The absence of malignant leukemic cells on blood smear or bone marrow aspiration | :* The absence of malignant leukemic cells on blood smear or bone marrow aspiration | ||
===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. | * 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> | ||
* Hairy cell leukemia patients who relapse '''after''' one year or more are be managed by the '''same''' initial purine analogue {{withorwithout}} [[rituximab]]. | * 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]]. | * 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: | * 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> | ||
:* [[Rituximab]] alone | :* [[Rituximab]] alone | ||
:* [[Interferon alpha]] alone | :* [[Interferon alpha]] alone | ||
Revision as of 01:19, 30 October 2015
Overview
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/mcl
- Absolute neutrophils count lower than 1000/mcl
- Pharmacological agents used for the treatment of hairy cell leukemia patients include:[2]
First Line Therapy
- The preferred pharmacological agent used for the initial management of hairy cell leukemia could be either cladribine or pentostatin.[1]
- 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.
- Common side effects of such agents may include:
- Immune suppression
- Acute kidney failure
- Fatigue
- High fever
- 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]
- 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]
- Hairy cell leukemia patients who relapse after one year or more are be managed by the same initial purine analogue ± rituximab.
- 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]
- 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]
- 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.
- ↑ 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 Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015