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{{CMG}} {{AE}} {{HL}}
{{CMG}} {{AE}} {{HL}}
==Overview==
==Overview==
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy. Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.<ref name="NCCN">Adult T-Cell Leukemia/Lymphoma. NCCN Guidelines Version 2 (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on January, 25 2016</ref>  
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic [[stem cell]] transplant.<ref name="NCCN">Adult T-Cell Leukemia/Lymphoma. NCCN Guidelines Version 2 (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on January, 25 2016</ref>  
==Medical Therapy==
==Medical Therapy==
* The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
* The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
* Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy.
* Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy.
* Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy.
* Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy.
* Adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.
* Adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.
=== Management of Chronic/Smoldering Adult T-cell Leukemia===
=== Management of Chronic/Smoldering Adult T-cell Leukemia===
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:* Complete history and physical examination
:* Complete history and physical examination
:* Serum calcium level  
:* Serum calcium level  
:* Blood urea nitrogen  
:* [[Blood urea nitrogen]]
:* Serum creatinine level
:* Serum [[creatinine]] level
:* Serum LDH
:* Serum [[LDH]]
:* Chest and abdominal CT scan  
:* [[Chest]] and abdominal [[CT scan]]
* Skin directed therapies for the management of localized cutaneous lesions among such patients may include:  
* Skin directed therapies for the management of localized [[cutaneous]] lesions among such patients may include:  
:* Topical corticosteroids
:* Topical [[corticosteroid]]s
:* Topical chemotherapy (mechlorethamine)
:* Topical [[chemotherapy]] ([[mechlorethamine]])
:* Local radiation (8–36 Gy)
:* Local [[radiotherapy]] (8–36 Gy)
:* Topical retinoids (bexarotene, tazarotene)
:* Topical [[retinoid]]s ([[bexarotene]], [[tazarotene]])
:* Phototherapy (UVB, NB-UVB for patch/thin plaques; PUVA for thicker plaques)
:* [[Phototherapy]] ([[UVB]], NB-UVB for management of patch/thin plaques; [[PUVA]] for management of thicker plaques)
:* Topical imiquimod
:* Topical [[imiquimod]]
* Zidovudine and interferon combination therapy:
* [[Zidovudine]] and [[interferon]] combination therapy:
:* Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
:* Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
:* Patients who responded to the therapy should be continued on zidovudine and interferon therapy.  
:* Pati[[Link title]]ents who responded to the therapy should be continued on zidovudine and interferon therapy.  
:* While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
:* While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
* The criteria for complete remission of adult T-cell leukemia patients includes:   
* The criteria for complete remission of adult T-cell leukemia patients includes:   
:* Absence of lymphadenopathy
:* Absence of [[lymphadenopathy]]
:* Absence of hepatomegaly and splenomegaly
:* Absence of [[hepatomegaly]] and [[splenomegaly]]
:* Absence of cutaneous lesions  
:* Absence of [[cutaneous]] lesions  
:* Absence of malignant on peripheral blood smear
:* Absence of [[malignant]] cells on [[peripheral blood smear]]
:* Absence of malignant on bone marrow biopsy
:* Absence of malignant cells on [[bone marrow]] [[biopsy]]
===Management of Acute Adult T-cell Leukemia===
===Management of Acute Adult T-cell Leukemia===
* The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:  
* The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:  
:* Cyclophosphamide {{and}} doxorubicin {{and}} vincristine {{and}} prednisone (CHOP)  
:* [[Cyclophosphamide]] {{and}} [[doxorubicin]] {{and}} [[vincristine]] {{and}} [[prednisone]] (CHOP)  
:* Cyclophosphamide {{and}} doxorubicin {{and}}  vincristine {{and}} etoposide {{and}} prednisone (CHOEP)  
:* Cyclophosphamide {{and}} doxorubicin {{and}}  vincristine {{and}} [[etoposide]] {{and}} prednisone (CHOEP)  
:* Etoposide {{and}} prednisone {{and}} vincristine {{and}} cyclophosphamide {{and}} doxorubicin (Dose-adjusted EPOCH)
:* Etoposide {{and}} prednisone {{and}} vincristine {{and}} cyclophosphamide {{and}} doxorubicin (Dose-adjusted EPOCH)
:* Cyclophosphamide {{and}} vincristine {{and}} doxorubicin {{and}} dexamethasone (HyperCVAD) alternating with  high-dose methotrexate and cytarabine
:* Cyclophosphamide {{and}} vincristine {{and}} doxorubicin {{and}} [[dexamethasone]] (HyperCVAD) alternating with  high-dose [[methotrexate]] and [[cytarabine]]
* Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.  
* Consider allogeneic [[stem cell]] transplantion for patients who respond to first line chemotherapeutic agents.  
* Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:   
* Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:   
:* Bendamustine
:* [[Bendamustine]]
:* Belinostat  
:* [[Belinostat]]
:* Brentuximab vedotin for systemic CD30+ PTCL 
:* DHAP ([[dexamethasone]] {{and}} [[cisplatin]] {{and}} [[cytarabine]])  
:* DHAP (dexamethasone, cisplatin, cytarabine)  
:* ESHAP ([[etoposide]] {{and}} [[methylprednisolone]] {{and}} [[cytarabine]] {{and}}  
:* ESHAP (etoposide {{and}} methylprednisolone {{and}} cytarabine {{and}} cisplatin)
:* GDP ([[gemcitabine]] {{and}} [[dexamethasone]] {{and}} [[cisplatin]])
:* Dose-adjusted EPOCH
:* GemOx (gemcitabine {{and}} [[oxaliplatin]])
:* GDP (gemcitabine {{and}} dexamethasone {{and}} cisplatin)
:* ICE (ifosfamide {{and}} [[carboplatin]] {{and}} [[etoposide]])
:* GemOx (gemcitabine {{and}} oxaliplatin)
:* ICE (ifosfamide {{and}} carboplatin {{and}} etoposide)
:* Pralatrexated
:* Pralatrexated
:* Romidepsin
:* Romidepsin
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* Zidovudine and interferon combination therapy:
* Zidovudine and interferon combination therapy:
:* Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
:* Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
:* Patients who responded to the therapy may be further managed by either allogeneic stem cell transplantion or continue on zidovudine and interferon combination therapy.  
:* Patients who responded to the therapy may be further managed by either allogeneic [[stem cell]] transplantion or continue on zidovudine and interferon combination therapy.  
:* While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
:* While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
===Management of Adult T-cell Lymphoma===
===Management of Adult T-cell Lymphoma===

Revision as of 22:15, 25 January 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]

Overview

The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy. Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.[1]

Medical Therapy

  • The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
  • Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy.
  • Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy.
  • Adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.

Management of Chronic/Smoldering Adult T-cell Leukemia

  • Patients may be managed by observation and close follow-up for any symptomatic deterioration. Follow up tests for such patients may include:
  • Skin directed therapies for the management of localized cutaneous lesions among such patients may include:
  • Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
  • PatiLink titleents who responded to the therapy should be continued on zidovudine and interferon therapy.
  • While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
  • The criteria for complete remission of adult T-cell leukemia patients includes:

Management of Acute Adult T-cell Leukemia

  • The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:
  • Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.
  • Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:
  • Consider allogeneic stem cell transplantion for patients who respond to second line chemotherapeutic agents.
  • Zidovudine and interferon combination therapy:
  • Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
  • Patients who responded to the therapy may be further managed by either allogeneic stem cell transplantion or continue on zidovudine and interferon combination therapy.
  • While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.

Management of Adult T-cell Lymphoma

  • The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:
  • Cyclophosphamide AND doxorubicin AND vincristine AND prednisone (CHOP)
  • Cyclophosphamide AND doxorubicin AND vincristine AND etoposide AND prednisone (CHOEP)
  • Etoposide AND prednisone AND vincristine AND cyclophosphamide AND doxorubicin (Dose-adjusted EPOCH)
  • Cyclophosphamide AND vincristine AND doxorubicin AND dexamethasone (HyperCVAD) alternating with high-dose methotrexate and cytarabine
  • Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.
  • Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:
  • Bendamustine
  • Belinostat
  • Brentuximab vedotin for systemic CD30+ PTCL
  • DHAP (dexamethasone, cisplatin, cytarabine)
  • ESHAP (etoposide AND methylprednisolone AND cytarabine AND cisplatin)
  • Dose-adjusted EPOCH
  • GDP (gemcitabine AND dexamethasone AND cisplatin)
  • GemOx (gemcitabine AND oxaliplatin)
  • ICE (ifosfamide AND carboplatin AND etoposide)
  • Pralatrexated
  • Romidepsin
  • Consider allogeneic stem cell transplantion for patients who respond to second line chemotherapeutic agents.

Supportive Therapy

Opportunistic Infections Prophylaxis

  • Sulfamethoxazole/trimethoprim prophylaxis is recommended among adult T-cell leukemia patients to protect against opportunistic infections.

References

  1. ↑ Adult T-Cell Leukemia/Lymphoma. NCCN Guidelines Version 2 (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on January, 25 2016

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