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1.
Br J Dermatol ; 157(3): 591-5, 2007 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17697078

RESUMO

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade malignant lymphoma that presents in the skin with no evidence of extracutaneous localization at diagnosis. We present an 80-year-old woman with B-cell chronic lymphocytic leukaemia (CLL) who developed multifocal PCMZL lesions 14 months after CLL diagnosis. PCMZL was clonally similar to the original bone marrow (BM) CLL cells. The specific translocation t(14;18) (q32;q21) with breakpoints in IGH and BCL2 loci was found in a skin specimen, but was absent in BM and peripheral blood (PB) cells. In contrast, a 13q deletion was found in BM and PB CLL cells. The patient was treated with chlorambucil and complete response of PCMZL was achieved. To our knowledge this is the first patient with CLL in whom PCMZL has been diagnosed.


Assuntos
Leucemia Linfocítica Crônica de Células B/patologia , Linfoma de Células B/patologia , Neoplasias Cutâneas/patologia , Idoso de 80 Anos ou mais , Antineoplásicos Alquilantes/uso terapêutico , Clorambucila/uso terapêutico , Deleção Cromossômica , Cromossomos Humanos Par 14 , Cromossomos Humanos Par 18 , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/genética , Linfoma de Células B/tratamento farmacológico , Linfoma de Células B/genética , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/genética , Translocação Genética , Resultado do Tratamento
2.
Ann Hematol ; 85(6): 366-73, 2006 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-16523310

RESUMO

Patients with Philadelphia chromosome-positive (Ph+) and/or BCR-ABL+ acute lymphoblastic leukemia (ALL) have extremely poor prognoses. Most of these patients have additional, heterogenous karyotype abnormalities, the majority of which have uncertain clinical significance. In this study we analyzed the clinical characteristics, karyotype abnormalities, and outcome of 77 patients with Ph+ and/or BCR-ABL+ ALL registered in Poland in 1997-2004. In 31/55 patients with known karyotype, the sole t(9;22)(q34;q11) abnormality had been diagnosed; in one patient, variant translocation t(4;9;22)(q21q31.1;q34;q11), and additional abnormalities in 23 (42%) patients, had been diagnosed. The characteristics of the patients with Ph chromosome and additional abnormalities were not significantly different when compared with the entire analyzed group. Out of 77 patients, 54 (70%) achieved first complete remission (CR1) after one or more induction cycles. The overall survival (OS) probability of 2 years was 63, 43, and 17% for patients treated with allogeneic stem cell transplantation (alloSCT), autologous SCT, and chemotherapy, respectively (log rank p=0.002). Median OS from the time of alloSCT was significantly longer for patients transplanted in CR1 compared with alloSCT in CR >1 (p=0.032). There were no significant differences in CR rate, disease-free survival (DFS), and OS for patients with t(9;22) and additional abnormalities compared with the whole group. Only WBC >20 G/l at diagnosis adversely influenced OS probability (log rank p=0.0017). In conclusion, our data confirm poor outcome of Ph+ and/or BCR-ABL+ ALL. Only patients who received alloSCT in CR1 had longer DFS and OS. We have shown that additional karyotype abnormalities did not influence the clinical characteristics of the patients; however, their influence on treatment results needs to be further assessed.


Assuntos
Proteínas de Fusão bcr-abl/genética , Leucemia Mielogênica Crônica BCR-ABL Positiva/terapia , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Citarabina/administração & dosagem , Intervalo Livre de Doença , Feminino , Transplante de Células-Tronco Hematopoéticas , Humanos , Cariotipagem , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnóstico , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Masculino , Mercaptopurina/administração & dosagem , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Polônia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Indução de Remissão , Estudos Retrospectivos , Resultado do Tratamento
3.
Br J Dermatol ; 153(4): 833-7, 2005 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-16181471

RESUMO

Richter syndrome (RS) is a transformation to high-grade non-Hodgkin lymphoma in patients with chronic lymphocytic leukaemia (CLL). RS may develop in lymph nodes or rarely extranodally. Skin localization of RS has been described in only a few cases. We present a 77-year-old woman who developed isolated diffuse large B-cell lymphoma (LBCL) in the skin of the nose without any other symptoms of RS. The LBCL in the skin was clonally distinct from the original bone marrow CLL cells. Moreover, LBCL cells were positive for LMP-1 segment of Epstein-Barr virus and overexpressed p53 protein. The patient was successfully treated with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and adjuvant local radiotherapy.


Assuntos
Leucemia Linfocítica Crônica de Células B/patologia , Linfoma de Células B/patologia , Células-Tronco Neoplásicas/patologia , Neoplasias Nasais/patologia , Neoplasias Cutâneas/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Feminino , Humanos , Linfoma de Células B/tratamento farmacológico , Segunda Neoplasia Primária/tratamento farmacológico , Segunda Neoplasia Primária/patologia , Neoplasias Nasais/tratamento farmacológico , Prednisona/uso terapêutico , Neoplasias Cutâneas/tratamento farmacológico , Síndrome , Vincristina/uso terapêutico
4.
Ann Hematol ; 84(9): 557-64, 2005 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-15856358

RESUMO

Purine nucleoside analogues, cladribine (2-chlorodeoxyadenosine, 2-CdA) and fludarabine (FAMP) are active agents in acute myeloid leukemias (AMLs). Synergistic interaction between FAMP or 2-CdA with cytarabine (cytosine arabinoside, Ara-C) has been demonstrated in preclinical and clinical studies. The current multicenter phase II study was initiated to evaluate the efficacy and toxicity of induction treatment consisting of 2-CdA (5 mg/m2), Ara-C (2 g/m2), mitoxantrone (MIT, 10 mg/m2) and granulocyte colony-stimulating factor (G-CSF) (CLAG-M) in refractory AML. In case of partial remission, a second CLAG-M was administered. Patients in complete remission (CR) received consolidation courses based on high-dose Ara-C and MIT with or without 2-CdA. Forty-three patients from five centers were registered: 25 primary resistant and 18 relapsed. CR was achieved in 21 (49%) patients, 20 (47%) were refractory and 2 (5%) died early. Hematologic toxicity was the most prominent toxicity of this regimen. The overall survival (OS; 1 year) for the 42 patients as a whole and the 20 patients in CR were 43% and 73%, respectively. Disease-free survival (1 year) was 68.6%. None of the analyzed prognostic factors influenced the CR and OS probability significantly. We conclude that CLAG-M regimen has significant antileukemia activity in refractory AML, which seems to be better than the activity of many other regimens. The toxicity of the treatment is acceptable.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Mieloide/tratamento farmacológico , Doença Aguda , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/toxicidade , Cladribina/administração & dosagem , Citarabina/administração & dosagem , Quimioterapia Combinada , Feminino , Fator Estimulador de Colônias de Granulócitos/administração & dosagem , Doenças Hematológicas/induzido quimicamente , Transplante de Células-Tronco Hematopoéticas , Humanos , Leucemia Mieloide/mortalidade , Masculino , Pessoa de Meia-Idade , Mitoxantrona/administração & dosagem , Indução de Remissão/métodos , Terapia de Salvação/métodos , Análise de Sobrevida
5.
Neoplasma ; 49(3): 155-8, 2002.
Artigo em Inglês | MEDLINE | ID: mdl-12098000

RESUMO

Hemostatic disorders mainly due to thrombocytopenia represent an important clinical problem in patients with myelodysplastic syndromes (MDS). Much less is known about the possible coagulation abnormalities. Thirty patients with MDS were studied. Activity of cancer procoagulant (CP), concentrations of activation markers of coagulation and fibrinolysis such as thrombin-antithrombin complexes (TAT), prothrombin fragment 1+2 (F1+2) and D-dimers (DD) as well as standard coagulation tests were determined. Coagulation abnormalities concerned mainly patients with RAEB and RAEB-t. In this group the mean values of TATand F1+2 concentrations were significantly higher than in control indicating chronic coagulation activation similar to that observed in acute leukemias. CP activity in MDS patients did not differ from the control group.


Assuntos
Transtornos da Coagulação Sanguínea/sangue , Cisteína Endopeptidases/sangue , Síndromes Mielodisplásicas/sangue , Proteínas de Neoplasias , Antitrombina III , Humanos , Fragmentos de Peptídeos/sangue , Peptídeo Hidrolases/sangue , Protrombina , Tempo de Protrombina
6.
Leuk Lymphoma ; 42(4): 789-96, 2001 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-11697509

RESUMO

Richter's syndrome (RS) refers to the development of aggressive non-Hodgkin's lymphoma (NHL) during the course of chronic lymphocytic leukaemia (CCL). It occurs in approximately 3% of patients with CLL. The isolated form of this complication in bone is extremely rare and, so far, has not been described in a patient treated with cladribine (2-CdA). We report a case of CLL treated successfully with 2-CdA, where isolated diffuse large B-cell lymphoma (LBCL) developed 2 years after the diagnosis of CLL Rai II and one year after the completion of 2-CdA treatment. RS was first manifested as a pathologic fracture of the left femur. The LBCL was clonally distinct from the original CLL cells. The patient was successfully treated with CHOP and radiotherapy and obtained complete response of the LBCL.


Assuntos
Cladribina/efeitos adversos , Fraturas do Fêmur/etiologia , Leucemia Linfocítica Crônica de Células B/patologia , Linfoma de Células B/patologia , Linfoma não Hodgkin/induzido quimicamente , Idoso , Medula Óssea/patologia , Transformação Celular Neoplásica/induzido quimicamente , Cladribina/administração & dosagem , Fraturas do Fêmur/diagnóstico por imagem , Humanos , Cariotipagem , Linfonodos/patologia , Linfoma não Hodgkin/etiologia , Linfoma não Hodgkin/patologia , Masculino , Segunda Neoplasia Primária/induzido quimicamente , Segunda Neoplasia Primária/etiologia , Segunda Neoplasia Primária/patologia , Cintilografia , Síndrome
7.
Leuk Lymphoma ; 41(5-6): 659-67, 2001 May.
Artigo em Inglês | MEDLINE | ID: mdl-11378584

RESUMO

Coexistence of systemic lupus erythematosus (SLE) with low-grade non-Hodgkin's lymphoma (LGNHL) has been described occasionally in the literature with the potential pathogenetic role of monoclonal B CD5+/CD19+ cells. We report a case of LGNHL which developed 18 months after diagnosis of SLE. The monoclonal population of lymphocytes in the peripheral blood and bone marrow was CD5/CD19 negative but CD19/CD22 positive. The SLE responded well to treatment with prednisone and the course of the LGNHL was stable and cytotoxic treatment was not required.


Assuntos
Moléculas de Adesão Celular , Lectinas , Lúpus Eritematoso Sistêmico/complicações , Linfoma não Hodgkin/etiologia , Antígenos CD/análise , Antígenos CD19/análise , Antígenos de Diferenciação de Linfócitos B/análise , Linfócitos B/química , Linfócitos B/patologia , Medula Óssea/patologia , Antígenos CD5/análise , Células Clonais/química , Células Clonais/patologia , Feminino , Rearranjo Gênico , Genes Codificadores da Cadeia delta de Receptores de Linfócitos T , Humanos , Imunofenotipagem , Lúpus Eritematoso Sistêmico/patologia , Linfoma não Hodgkin/patologia , Pessoa de Meia-Idade , Lectina 2 Semelhante a Ig de Ligação ao Ácido Siálico , Pele/patologia , Linfócitos T/patologia
8.
Leuk Lymphoma ; 36(1-2): 77-84, 1999 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-10613452

RESUMO

Coagulation disorders are often the reason for fatal bleeding in acute promyelocytic leukemia. Their occurrence as well as pathogenesis and prognostic significance in other subtypes of acute myelogenous leukemia and acute lymphoblastic leukemia is less known. Tests were carried out in 70 patients including 49 with AML and 21 with ALL. In all patients thrombin-antithrombin complexes (TAT), D-dimer (DD) and plasmin-antiplasmin complexes (PAP), antithrombin III activity, fibrinogen/fibrin degradation products, APTT and PT were determined. The tests were performed on diagnosis and after cytostatic treatment. The level of TAT, DD and PAP was elevated in 83% of the patients on diagnosis and in 90% after treatment. The highest values were observed in AML M3 patients. Among leukemic patients with normal levels of TAT, DD and PAP at diagnosis, cytostatic treatment had a negligible effect on the level of these markers. During remission the levels of these markers returned to the normal values while in patients without remission they were either elevated or returned to normal values. No correlation between the levels of activation markers and remission rate was reported. DIC was diagnosed in 13 patients including three after chemotherapy. The DIC was acute or subacute in AML and chronic in ALL patients. In the majority of acute leukemia patients there were already changes on diagnosis indicating coagulation activation. Except for AML M3, these usually had a subclinical course. The TAT, DD and PAP tests are not reliable markers of remission in acute leukemias.


Assuntos
Transtornos da Coagulação Sanguínea/etiologia , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Adulto , Antitrombina III/análise , Coagulação Sanguínea , Coagulação Intravascular Disseminada/etiologia , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Humanos , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/complicações , Masculino , Pessoa de Meia-Idade , Peptídeo Hidrolases/análise , Leucemia-Linfoma Linfoblástico de Células Precursoras/sangue , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações
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