Sandbox: ATL
- 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 (836 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.