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1.
Eur J Haematol ; 109(5): 590-592, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35871485

RESUMO

B-cell prolymphocytic leukaemia (B-PLL) is an aggressive B-cell lymphoproliferative disease with few clinically proven targeted treatments. Due to the rarity of the condition, there is a paucity of clinical trial data and none that involve targeted inhibitors. We present a unique case of relapsed refractory B-PLL treated to a sustained minimal residual disease-negative remission with venetoclax monotherapy, to add to the current evidence base and rationale for future studies using BCL-2 inhibitors in B-PLL.


Assuntos
Leucemia Linfocítica Crônica de Células B , Leucemia Prolinfocítica Tipo Células B , Compostos Bicíclicos Heterocíclicos com Pontes/uso terapêutico , Humanos , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Leucemia Prolinfocítica Tipo Células B/tratamento farmacológico , Leucemia Prolinfocítica Tipo Células B/etiologia , Neoplasia Residual , Proteínas Proto-Oncogênicas c-bcl-2/genética , Sulfonamidas , Proteína Supressora de Tumor p53
2.
Ann Diagn Pathol ; 54: 151790, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34293709

RESUMO

B-prolymphocytic leukemia (B-PLL) is included as a distinct entity in the current World Health Organization classification of hematolymphoid neoplasms. However, the diagnosis of B-PLL has presented several challenges since its conception, and over the past decades investigations of B-PLL have revealed substantial biologic and molecular heterogeneity. These data have shown that many B-PLL cases present many similarities with other types of small B-cell lymphomas, and that small B-cell lymphomas can undergo prolymphocytoid transformation. As a result, the frequency of B-PLL has markedly decreased, and currently B-PLL is a very rare entity. Most recent studies focused on B-PLL cases have been conducted on limited cohorts, precluding robust conclusions. In this article, we provide a concise historical review of B-PLL and describe the diagnostic and clinical challenges associated with establishing this diagnosis. We also argue that cases currently classified as B-PLL are unlikely to be a unique biologic entity, but rather represent a state of morphologic transformation characterized by many prolymphocytes that is shared by various types of small B-cell lymphoma.


Assuntos
Leucemia Prolinfocítica Tipo Células B/diagnóstico , Leucemia Prolinfocítica Tipo Células B/patologia , Linfócitos/patologia , Transformação Celular Neoplásica/patologia , Humanos , Imunofenotipagem/métodos , Leucemia Prolinfocítica Tipo Células B/epidemiologia , Linfoma de Células B/patologia
3.
Curr Oncol Rep ; 19(4): 29, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28324286

RESUMO

PURPOSE OF REVIEW: We aimed to produce a comprehensive update on clinical and biological data regarding two rare lymphoid neoplasms, B and T prolymphocytic leukemias, and assess therapeutic management in the light of new molecular insights and the advent of targeted therapies. RECENT FINDINGS: B cell prolymphocytic leukemia (B-PLL) diagnosis remains challenging in the absence of clear immunophenotypic or cytogenetic signature and overlap with mantle cell lymphoma. New molecular defects have been identified in T cell prolymphocytic leukemia (T-PLL), especially in the JAK STAT pathway. Like in chronic lymphocytic leukemia (CLL), B-PLL treatment depends on the presence of TP53 dysfunction. In T-PLL, alemtuzumab still remains the standard of care. Allogeneic transplantation is the only curable option. Thanks to reduced intensity conditioning regimens, it has become accessible to a larger number of patients. PLL prognosis remains poor with conventional therapies. However, great advances in the understanding of both T- and B-PLL pathogenesis lead to promising new therapeutic agents.


Assuntos
Leucemia Prolinfocítica Tipo Células B/diagnóstico , Leucemia Prolinfocítica Tipo Células B/genética , Leucemia Prolinfocítica Tipo Células B/terapia , Leucemia Prolinfocítica de Células T/diagnóstico , Alemtuzumab , Anticorpos Monoclonais Humanizados/uso terapêutico , Humanos , Imunofenotipagem/métodos , Leucemia Prolinfocítica de Células T/genética , Leucemia Prolinfocítica de Células T/terapia , Transplante Homólogo/métodos
4.
Ter Arkh ; 89(7): 10-17, 2017.
Artigo em Russo | MEDLINE | ID: mdl-28766535

RESUMO

AIM: To analyze the efficiency and reproducibility of the ALL-2009 protocol within the Russian prospective multicenter study based on different principles of cytostatic effects (non-intensive, but continuous cytotoxic treatment and a small number of allogeneic hematopoietic stem cells). SUBJECTS AND METHODS: The ALL-2009 (NCT01193933) study conducted in April 2009 to December 2016 included 194 patients (95 males and 99 females) aged 15 to 55 years (median age 28 years) with Ph-negative B-cell acute lymphoblastic leukemia (ALL). There was early pre-B-cell ALL in 54 patients, common ALL in 101, pre-B ALL in 39, initial leukocytosis in 9.4·109/l (0.4-899.0), lactate dehydrogenase in 901 IU (31-13 059), an initial central nervous system lesion in 17 (8.7%), mediastinal injury in 3 (1.5%), and splenomegaly in 111 (57.2%). The results of standard cytogenetic analysis are known in 113 (60.4%) patients. Normal karyotypes were detected in 49 (54.5%) out of the patients; t(4;11) in 9 (5.4%), t(1;19) in 2 (1.2%), and other karyotypic abnormalities in 53 (46.9%). Thirteen (7.8%) patients underwent allogeneic hematopoietic stem cell transplantation in first complete remission (CR); their proportion did not differ in the federal and regional centers. RESULTS: The frequency of CR achievement was the same in the federal and regional centers and generally amounted to 87.5%. Early (8.8%) and CR (9.6%) mortality rates remained high despite the low aggressiveness of cytotoxic action, necessitating the improvement of auxiliary treatment. The five-year overall survival (OS) rates vary considerably in the federal and regional centers (72.6 and 43.8%), the relapse-free survival (RFS) (70.2 and 53.4%) and recurrence risk (23.1 and 36.5%) are comparable. This suggests that the non-intensive, but continuous exposure principle built in the ALL-2009 protocol makes it possible to reproduce the envisaged treatment program and to achieve satisfactory results. CONCLUSION: The ALL-2009 protocol allows both the federal and regional centers to obtain the long-term results comparable with those of current foreign studies: OS (54.2%), RFS (56.5%); and relapse risk (35.4%). Multivariate analysis has identified age (over 30 years), initial leukocytosis (30·109/l and more) and t(4;11) among the main clinical prognostic factors. Gene mutation detection evaluated in a small number of patients (8/36) is not a poor prognostic sign. There is a need for further investigations with centralized evaluation of the mutation status of leukemic cells and the clearance of minimal residual disease.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Prolinfocítica Tipo Células B , Indução de Remissão/métodos , Doença Aguda , Adulto , Feminino , Humanos , Quimioterapia de Indução/métodos , Quimioterapia de Indução/estatística & dados numéricos , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Leucemia Prolinfocítica Tipo Células B/epidemiologia , Leucemia Prolinfocítica Tipo Células B/terapia , Masculino , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Federação Russa/epidemiologia , Prevenção Secundária/métodos , Prevenção Secundária/estatística & dados numéricos , Análise de Sobrevida
6.
Int J Hematol ; 120(2): 252-255, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38796826

RESUMO

B-cell prolymphocytic leukemia (B-PLL) was recognized as a distinct entity in the fourth edition of the World Health Organization (WHO) classification for hematolymphoid neoplasms (WHO-HAEM4); however, its de novo presentation has been removed from the upcoming 5th edition classification (WHO-HAEM5). We present a case of a 65-year-old man with leukocytosis, fatigue, and no organomegaly by imaging. Bone marrow examination showed a prolymphocytoid population comprising 78% of the marrow elements. After thorough exclusion of other entities by clinical parameters and ancillary methods, we concluded that this case represents a de novo case of B-PLL.


Assuntos
Leucemia Prolinfocítica Tipo Células B , Humanos , Masculino , Idoso , Leucemia Prolinfocítica Tipo Células B/patologia , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Medula Óssea/patologia , Leucocitose/diagnóstico , Leucocitose/etiologia , Leucocitose/patologia
9.
Rinsho Ketsueki ; 51(1): 80-2, 2010 Jan.
Artigo em Japonês | MEDLINE | ID: mdl-20134145

RESUMO

CD5 is a T-cell marker that is expressed in mature B cell malignancies and other B cell chronic lymphoproliferative disorders, but the biologic function of CD5 is unknown. We report a 68-year-old woman with B-cell prolymphocytic leukemia (B-PLL) expressing CD5 antigen. On admission, jaundice and hepatosplenomegaly were noted. Hematological examination demonstrated a platelet count of 2.8 x 10(4)/microl and a white blood cell count of 19,900/microl with 69% PLL cells. Surface marker analysis of the PLL cells was positive for CD5, CD19, CD20, sIgM, and was negative for CD23, and cyclin D1 was negative in immunostaining.


Assuntos
Biomarcadores Tumorais/sangue , Antígenos CD5/sangue , Leucemia Prolinfocítica Tipo Células B/sangue , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Idoso , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Contagem de Leucócitos , Contagem de Plaquetas
10.
Orv Hetil ; 151(31): 1261-3, 2010 Aug 01.
Artigo em Húngaro | MEDLINE | ID: mdl-20656663

RESUMO

B-cell prolymphocytic leukemia (B-PLL) is a rare disorder characterized by marked lymphocytosis in the peripheral blood, matured lymphocytic infiltration in the bone marrow and splenomegaly. It has a distinct immunophenotype (CD19, CD20, CD22, FMC7, intensive surface immunoglobulin positivity) which helps to differentiate from other lymphoproliferative malignancies. It has a poor prognosis and its treatment is unsettled. The authors present a case of a patient with typical B-PLL treated with FCR-Lite (fludarabine, cyclophosphamide, rituximab) protocol achieving complete hematological (and immunophenotypic) remission. The treatment was well tolerated. Neither major infective complication nor tumor lysis syndrome was observed. According to the author's experience the FCR-Lite protocol can not only be used in patients with CLL but it also can be effective in patients with B-PLL. No clinical experience has been reported yet in the literature with this protocol.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Prolinfocítica Tipo Células B/tratamento farmacológico , Idoso , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais Murinos , Ciclofosfamida/administração & dosagem , Diagnóstico Diferencial , Humanos , Imunofenotipagem , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Masculino , Prognóstico , Rituximab , Resultado do Tratamento , Vidarabina/administração & dosagem , Vidarabina/análogos & derivados
12.
Rinsho Ketsueki ; 49(12): 1619-22, 2008 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-19110524

RESUMO

We report a case of B-cell prolymphocytic leukemia (B-PLL) that was treated successfully with splenic irradiation (SI). An 86-year-old man underwent a medical examination for lumbago and general fatigue at another hospital in June 2007. A compressed lumbar fracture and splenomegaly were found using computed tomography (CT). Thereafter, the patient consulted our hospital because of leukocytosis. Peripheral blood showed hemoglobin level 9.8 g/dl and white blood cell count 38.1x10(9)/l with 91% atypical cells. Surface marker analysis demonstrated that atypical cells were positive for CD20, CD22, FMC7, surface IgM, surface IgD and kappa, but were negative for CD5, TdT and lambda. The morphology of these cells was compatible with prolymphocytes with prominent nucleoli and condensed nuclear chromatin. A diagnosis of B-PLL was made. SI (total dose 20 Gy) was chosen for the treatment and a single course of SI was very effective without causing any significant adverse events. This case demonstrates that SI may remain valuable for the treatment of B-PLL in an elderly patient.


Assuntos
Leucemia Prolinfocítica Tipo Células B/radioterapia , Baço , Idoso de 80 Anos ou mais , Humanos , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Masculino , Dosagem Radioterapêutica
13.
Epigenetics ; 11(6): 449-55, 2016 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-27128508

RESUMO

Based on the methylation status of 5 single CpG sites, a novel epigenetic classification of chronic lymphocytic leukemia (CLL) was recently proposed, classifying CLL patients into 3 clinico-biological subgroups with different outcome, termed memory like CLL (m-CLL), naïve like CLL (n-CLL), and a third intermediate CLL subgroup (i-CLL). While m-CLL and n-CLL patients at large corresponded to patients carrying mutated and unmutated IGHV genes, respectively, limited information exists regarding the less defined i-CLL group. Using pyrosequencing, we investigated the prognostic impact of the proposed 5 CpG signature in a well-characterized CLL cohort (135 cases), including IGHV-mutated and unmutated patients as well as clinically aggressive stereotyped subset #2 patients. Overall, we confirmed the signature's association with established prognostic markers. Moreover, in the presence of the IGHV mutational status, the epigenetic signature remained independently associated with both time-to-first-treatment and overall survival in multivariate analyses. As a prime finding, we observed that subset #2 patients were predominantly classified as i-CLL, probably reflecting their borderline IGHV mutational status (97-99% germline identity), though having a similarly poor prognosis as n-CLL patients. In summary, we validated the epigenetic classifier as an independent factor in CLL prognostication and provide further evidence that subset #2 is a member of the i-CLL group, hence supporting the existence of a third, intermediate epigenetic subgroup.


Assuntos
Biomarcadores Tumorais/normas , Metilação de DNA , Epigênese Genética , Leucemia Prolinfocítica Tipo Células B/classificação , Adulto , Idoso , Biomarcadores Tumorais/genética , Feminino , Humanos , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Leucemia Prolinfocítica Tipo Células B/genética , Masculino , Pessoa de Meia-Idade , Mutação , Prognóstico , Análise de Sobrevida
15.
Best Pract Res Clin Haematol ; 28(4): 180-92, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26614896

RESUMO

Significant advances in the diagnosis and treatment of hairy cell leukemia (HCL) have recently been made. Improved distinction of HCL from its mimics though clinical presentations, morphologic and immunophenotypic features, and more recently molecular biology, has highlighted marked differences in treatment response and overall prognosis between these disorders. As our understanding of the unique pathobiology of HCL has grown, exciting new avenues of treatment as well as insight into immune function have been obtained. This review provides an overview of the clinical features and diagnostic attributes of HCL, with contrast to other mature B cell lymphoproliferative disorders with overlapping features.


Assuntos
Leucemia de Células Pilosas/diagnóstico , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Macroglobulinemia de Waldenstrom/diagnóstico , Antineoplásicos/uso terapêutico , Linfócitos B/efeitos dos fármacos , Linfócitos B/patologia , Diagnóstico Diferencial , Fadiga/diagnóstico , Fadiga/patologia , Feminino , Humanos , Indóis/uso terapêutico , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/patologia , Leucemia de Células Pilosas/cirurgia , Leucemia Prolinfocítica Tipo Células B/tratamento farmacológico , Leucemia Prolinfocítica Tipo Células B/patologia , Leucemia Prolinfocítica Tipo Células B/cirurgia , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma de Zona Marginal Tipo Células B/cirurgia , Masculino , Mutação , Proteínas Proto-Oncogênicas B-raf/antagonistas & inibidores , Proteínas Proto-Oncogênicas B-raf/genética , Fatores Sexuais , Esplenectomia , Esplenomegalia/diagnóstico , Esplenomegalia/patologia , Esplenomegalia/cirurgia , Sulfonamidas/uso terapêutico , Vemurafenib , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Macroglobulinemia de Waldenstrom/patologia , Macroglobulinemia de Waldenstrom/cirurgia
16.
BMJ Case Rep ; 20132013 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-24000206

RESUMO

B-cell prolymphocytic leukaemia (BPLL) is a haematological malignancy defined as lymphocytosis and splenomegaly with >55% circulating cells being clonal prolymphocytes of B-cell origin. The evolution of this disease is more aggressive than chronic lymphocytic leukaemia. We reported a case of a 62-year-old man with BPLL who, on treatment, attained cytological, immunophenotypic and complete cytogenetic remission. He subsequently developed an asymmetric sensorimotor neurological disorder, suggestive of lymphomatous infiltration (neurolymphocytosis). Repetition of the MRI and the electromyography was essential for diagnosis. Progressive mononeuritis multiplex in B-cell leukaemias/lymphomas is rare and may be the only presenting symptom of relapsed or progressive disease. Repeat imaging studies based on judicious evaluation of the clinical scenario for exclusion of other causes of neurological symptoms is necessary. This can be challenging in patients with long-standing malignancies who have received multiple courses of chemotherapy and/or radiotherapy.


Assuntos
Leucemia Prolinfocítica Tipo Células B , Mononeuropatias , Eletromiografia , Humanos , Leucemia Prolinfocítica Tipo Células B/complicações , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Leucemia Prolinfocítica Tipo Células B/patologia , Masculino , Pessoa de Meia-Idade , Mononeuropatias/diagnóstico , Mononeuropatias/etiologia , Mononeuropatias/patologia , Neoplasias do Sistema Nervoso/complicações , Neoplasias do Sistema Nervoso/diagnóstico
17.
Intern Med ; 51(15): 1977-81, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22864121

RESUMO

OBJECTIVE: Japanese chronic lymphocytic leukemia (CLL) provides a diagnostic dilemma due to the low incidence and the heterogeneity shown in its morphology and immunophenotype. We clarified the diagnostic problems in Japanese CLL through our retrospective observation. METHODS: Between 2006 and 2011, we found a total of 48 cases with CLL and other indolent B-cell leukemias. We made a diagnosis of true CLL based on clinical, laboratory, immunophenotypic and cytogenetic data. RESULTS: Among the 48 cases, only 28 cases (58.3%) were diagnosed with true CLL. Morphologic evaluation using a forced-air dried preparation alone is not helpful to distinguish CLL from other indolent B-cell leukemias, including hairy cell leukemia, mantle cell lymphoma, lymphoplasmacytic lymphoma, and splenic marginal zone lymphoma. CLL immunophenotypic score should be more strictly applied in Japan than in Western countries. CONCLUSION: Fluorescence in situ hybridization for CCND1/IGH, the presence of leukocytosis and lymphadenopathy at diagnosis, and the morphological evaluation using naturally air dried preparations are important clues to make a correct diagnosis of Japanese CLL.


Assuntos
Leucemia de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/diagnóstico , Povo Asiático , Antígenos CD5/metabolismo , Análise Citogenética , Humanos , Imunofenotipagem , Hibridização in Situ Fluorescente , Japão , Leucemia de Células B/genética , Leucemia de Células B/imunologia , Leucemia de Células Pilosas/diagnóstico , Leucemia Linfocítica Crônica de Células B/genética , Leucemia Linfocítica Crônica de Células B/imunologia , Leucemia Prolinfocítica Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma Folicular/diagnóstico , Linfoma de Célula do Manto/diagnóstico , Proteínas de Fusão Oncogênica/genética , Estudos Retrospectivos
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