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 (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.