Chronic myeloid leukemia(CML) is a myeloproliferative disease which leads the unregulated growth of myeloid cells in the bone marrow. It is characterized by the presence of Philadelphia chromosome. Reciprocal transloc...Chronic myeloid leukemia(CML) is a myeloproliferative disease which leads the unregulated growth of myeloid cells in the bone marrow. It is characterized by the presence of Philadelphia chromosome. Reciprocal translocation of the ABL gene from chromosome 9 to 22 t(9; 22)(q34; q11.2) generate a fusion gene(BCRABL). BCR-ABL protein had constitutive tyrosine kinase activity that is a primary cause of chronic phase of CML. Tyrosine kinase inhibitors(TKIs) are now considered standard therapy for patients with CML. Even though, successful treatment with the TKIs, allogeneic stem cell transplantation(ASCT) is still an important option for the treatment of CML, especially for patients who are resistant or intolerant to at least one second generation TKI or for patients with blastic phase. Today, we know that there is no evidence for increased transplantrelated toxicity and negative impact of survival with pretransplant TKIs. However, there are some controversies about timing of ASCT, the optimal conditioning regimens and donor source. Another important issue is that BCRABL signaling is not necessary for survival of CML stem cell and TKIs were not effective on these cells. So, ASCT may play a role to eliminate CML stem cells. In this article, we review the diagnosis, management and treatment of CML. Later, we present our center's outcomes of ASCT for patients with CML and then, we discuss the place of ASCT in CML treatment in the TKIs era.展开更多
Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress ...Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress in about 20% of such patients. About 30% of patients have been reported to develop advancedstage disease and, at present, there is no cure for the disease. A number of systemic approaches have been used for advanced-stage mycosis fungoides(IIB-IV) and transformed disease. Aggressive approaches seem to be warranted in such patients. The scope of this review is the stem cell transplantation in mycosis fungoides and its leukemic variant, Sèzary syndrome.展开更多
Primary cutaneous B cell lymphoma(PCBCL) is defined as B cell lymphomas that presents in the skin without any evidence of extra-cutaneous involvement at diagnosis. They are the second most common type of primary cutan...Primary cutaneous B cell lymphoma(PCBCL) is defined as B cell lymphomas that presents in the skin without any evidence of extra-cutaneous involvement at diagnosis. They are the second most common type of primary cutaneous lymphomas accounting for 25%-30%. Since the prognosis and treatment differ from systemic lymphomas involving the skin, differential diagnosis is very important. PCBCL is a heterogeneous group of disease comprising different B cell lymphomas with distinct treatment and prognosis. PCBCL is divided into 5 subclasses according to World Health Organization and European Organization of Research and Treatment of Cancer classification. Primary cutaneous marginal zone lymphoma and primary cutaneous follicle centerlymphoma are indolent forms and often confined to skin at presentation and during the course of the disease. But primary cutaneous diffuse large B cell lymphoma, leg type and intravascular large B cell lymphoma are more aggressive forms that may disseminate to extra-cutaneous tissues. There is not a treatment consensus since they are rare entities. Local therapies like radiotherapy, surgery or intralesional steroids are options for localized disease in indolent forms. More disseminated disease may be treated with a systemic therapy like single agent rituximab. However combination chemotherapies which are used in systemic lymphomas are also required for aggressive PCBCL. Although indolent forms have relatively better prognosis, early relapses and disseminated diseases are mostly observed in aggressive form with a consequent poor prognosis.展开更多
文摘Chronic myeloid leukemia(CML) is a myeloproliferative disease which leads the unregulated growth of myeloid cells in the bone marrow. It is characterized by the presence of Philadelphia chromosome. Reciprocal translocation of the ABL gene from chromosome 9 to 22 t(9; 22)(q34; q11.2) generate a fusion gene(BCRABL). BCR-ABL protein had constitutive tyrosine kinase activity that is a primary cause of chronic phase of CML. Tyrosine kinase inhibitors(TKIs) are now considered standard therapy for patients with CML. Even though, successful treatment with the TKIs, allogeneic stem cell transplantation(ASCT) is still an important option for the treatment of CML, especially for patients who are resistant or intolerant to at least one second generation TKI or for patients with blastic phase. Today, we know that there is no evidence for increased transplantrelated toxicity and negative impact of survival with pretransplant TKIs. However, there are some controversies about timing of ASCT, the optimal conditioning regimens and donor source. Another important issue is that BCRABL signaling is not necessary for survival of CML stem cell and TKIs were not effective on these cells. So, ASCT may play a role to eliminate CML stem cells. In this article, we review the diagnosis, management and treatment of CML. Later, we present our center's outcomes of ASCT for patients with CML and then, we discuss the place of ASCT in CML treatment in the TKIs era.
文摘Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress in about 20% of such patients. About 30% of patients have been reported to develop advancedstage disease and, at present, there is no cure for the disease. A number of systemic approaches have been used for advanced-stage mycosis fungoides(IIB-IV) and transformed disease. Aggressive approaches seem to be warranted in such patients. The scope of this review is the stem cell transplantation in mycosis fungoides and its leukemic variant, Sèzary syndrome.
文摘Primary cutaneous B cell lymphoma(PCBCL) is defined as B cell lymphomas that presents in the skin without any evidence of extra-cutaneous involvement at diagnosis. They are the second most common type of primary cutaneous lymphomas accounting for 25%-30%. Since the prognosis and treatment differ from systemic lymphomas involving the skin, differential diagnosis is very important. PCBCL is a heterogeneous group of disease comprising different B cell lymphomas with distinct treatment and prognosis. PCBCL is divided into 5 subclasses according to World Health Organization and European Organization of Research and Treatment of Cancer classification. Primary cutaneous marginal zone lymphoma and primary cutaneous follicle centerlymphoma are indolent forms and often confined to skin at presentation and during the course of the disease. But primary cutaneous diffuse large B cell lymphoma, leg type and intravascular large B cell lymphoma are more aggressive forms that may disseminate to extra-cutaneous tissues. There is not a treatment consensus since they are rare entities. Local therapies like radiotherapy, surgery or intralesional steroids are options for localized disease in indolent forms. More disseminated disease may be treated with a systemic therapy like single agent rituximab. However combination chemotherapies which are used in systemic lymphomas are also required for aggressive PCBCL. Although indolent forms have relatively better prognosis, early relapses and disseminated diseases are mostly observed in aggressive form with a consequent poor prognosis.
