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HTLV-1 infection in the United States appears to be rare.  
__NOTOC__
Although little serologic data exist, prevalence of infection is thought to be highest among blacks living in the Southeast.  
* The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
A prevalence rate of 30% has been found among black intravenous drug abusers in New Jersey, and a rate of 49% has been found in a similar group in New Orleans.
* Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of idovudine and interferon therapy.
It is possible that prevalence of infection is increasing in this risk group.
* 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.
Studies of HTLV-1 antibody indicate that the virus is endemic in southern Japan, in the Caribbean, South America, and in Africa.
* 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:
ATL is relatively uncommon among those infected with HTLV-1.  
:* Complete history and physical examination
The overall incidence of ATL is estimated at about 1 per 1,500 adult HTLV-1 carriers per year.
:* Serum calcium level
Those cases that have been reported have occurred mostly among persons from the Caribbean or blacks from the Southeast (National Institutes of Health, unpublished data).
:* Blood urea nitrogen
There appears to be a long latent period between HTLV-1 infection and the start of ATL.
:* Serum creatinine level
:* Serum LDH
:* Chest and abdominal CT scan
* Skin directed therapies for the management of localized cutaneous lesions among such patients may include:
:* Topical corticosteroids
:* Topical chemotherapy (mechlorethamine)
:* Local radiation (8–36 Gy)
:* Topical retinoids (bexarotene, tazarotene)
:* Phototherapy (UVB, NB-UVB for patch/thin  plaques; PUVA for thicker plaques)
:* Topical imiquimod
* 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 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: 
:* Absence of lymphadenopathy
:* Absence of hepatomegaly and splenomegaly
:* Absence of cutaneous lesions
:* Absence of malignant on peripheral blood smear
:* Absence of malignant on bone marrow biopsy
===Management of Acute Adult T-cell Leukemia===
* 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.  
* 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.

Latest revision as of 19:43, 25 January 2016

  • 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 idovudine 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:
  • Complete history and physical examination
  • Serum calcium level
  • Blood urea nitrogen
  • Serum creatinine level
  • Serum LDH
  • Chest and abdominal CT scan
  • Skin directed therapies for the management of localized cutaneous lesions among such patients may include:
  • Topical corticosteroids
  • Topical chemotherapy (mechlorethamine)
  • Local radiation (8–36 Gy)
  • Topical retinoids (bexarotene, tazarotene)
  • Phototherapy (UVB, NB-UVB for patch/thin plaques; PUVA for thicker plaques)
  • Topical imiquimod
  • 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 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:
  • Absence of lymphadenopathy
  • Absence of hepatomegaly and splenomegaly
  • Absence of cutaneous lesions
  • Absence of malignant on peripheral blood smear
  • Absence of malignant on bone marrow biopsy

Management of Acute Adult T-cell Leukemia

  • 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.
  • 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.
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