Sandbox: ATL: Difference between revisions

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or small volume lymphadenopathy, absence of hypercalcaemia and normal or only slightly raised lactate dehydrogenase (LDH) (less than twice the upper normal limit value). Patients with  unlike the chronic form, the white blood cell count is normal and there is no lymphocytosis and less than 3% atypical circulating lymphocytes. Less than a third of patients present with lymphoma with no evidence of blood involvement. There are several reports documenting disease progression to the acute form in patients with chronic and smouldering ATLL. Haemophagocytic syndrome as the first sign of transformation has been described in smouldering ATLL.12
__NOTOC__
 
* The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
In addition to the symptoms related to the neoplastic condition, patients with ATLL are immunocompromised and develop opportunistic infections that complicate the disease course and make its management more difficult. Infestation by Strongyloides stercoralis is frequent and may be severe and fatal. This association and the fact that a third of the patients with strongyloidiasis and positive serology for HTLV‐I have a clonal integration of the provirus in their lymphocytes, have led to the hypothesis that this parasite plays a role in the development of ATLL in healthy carriers.13
* 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.
Most patients with medulloblastoma are initially asymptomatic. If left untreated, most of the patients with medulloblastoma will develop ataxia, nystagmus, and positional dizziness within a few months.
=== Management of Chronic/Smoldering Adult T-cell Leukemia===
===Acute ALT===
* Patients may be managed by observation and close follow-up for any symptomatic deterioration. Follow up tests for such patients may include:
The clinical course is aggressive with a median survival of less than 12 months in the acute and lymphoma forms.
:* Complete history and physical examination
his is characterised by the presence of systemic symptoms, organomegaly, in particular lymphadenopathy, and a leukaemic picture. Skin lesions Hypercalcaemia with or without lytic bone lesions
:* Serum calcium level
===Chronic===
:* Blood urea nitrogen
The chronic form is characterised by lymphocytosis which may be stable for months or even years, skin manifestations, no organomegaly
:* Serum creatinine level
===Smolud===
:* Serum LDH
smouldering ATLL are usually asymptomatic or manifest skin rashes that respond to topical steroids and/or lung infiltrates;
:* Chest and abdominal CT scan
 
* Skin directed therapies for the management of localized cutaneous lesions among such patients may include:
==Complications==
:* Topical corticosteroids
===prognosis===
:* Topical chemotherapy (mechlorethamine)
high lactate dehydrogenase (LDH)
:* Local radiation (8–36 Gy)
high leukemic counts
:* Topical retinoids (bexarotene, tazarotene)
hypercalcemia
:* Phototherapy (UVB, NB-UVB for patch/thin  plaques; PUVA for thicker plaques)
poor clinical performance status
:* 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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