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1.
Am J Hematol ; 99(6): 1172-1174, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38436141

RESUMO

Probability of treatment-free remission (TFR) in CML patients with additional chromosomal abnormalities (ACA) in the Philadelphia-positive clone or variant Philadelphia translocations (ACA/Var-Ph group, blue panel), in those with no cytogenetic abnormality other than the classical Philadelphia translocation (c-Ph group, green panel) and in the subgroups of CML patients with high-risk ACA (HR-ACA, yellow panel) and Var-Ph (red panel).


Assuntos
Leucemia Mielogênica Crônica BCR-ABL Positiva , Cromossomo Filadélfia , Indução de Remissão , Translocação Genética , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Aberrações Cromossômicas , Idoso , Adolescente
2.
Ann Hematol ; 100(1): 1-10, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33009935

RESUMO

Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection with high mortality rate usually seen in the context of immunosuppression. Although cases have been reported largely in patients with HIV/AIDS, following the use of monoclonal antibodies and occasionally in haematological malignancies, there is no review to date of patients with smouldering or treated myeloma who developed PML. Here, we conducted a literature search of PML cases in patients with multiple myeloma (MM), analyse patient and disease characteristics and describe the possible mechanisms that could lead to the development of PML. The lack of data and case reports until 2010 may indicate that PML in MM is underdiagnosed. Simultaneously, with an expanding field of new therapeutic options, patients with MM live longer, albeit continually immunosuppressed, and at risk of opportunistic infections. Emerging new treatments for PML in the horizon render the need to look out for this complication mandatory, and more case reports are needed to enrich our knowledge in this field.


Assuntos
Hospedeiro Imunocomprometido/efeitos dos fármacos , Leucoencefalopatia Multifocal Progressiva/tratamento farmacológico , Leucoencefalopatia Multifocal Progressiva/imunologia , Mieloma Múltiplo/tratamento farmacológico , Mieloma Múltiplo/imunologia , Anticorpos Monoclonais/metabolismo , Anticorpos Monoclonais/farmacologia , Anticorpos Monoclonais/uso terapêutico , Humanos , Hospedeiro Imunocomprometido/fisiologia , Fatores Imunológicos/metabolismo , Fatores Imunológicos/farmacologia , Fatores Imunológicos/uso terapêutico , Leucoencefalopatia Multifocal Progressiva/metabolismo , Mieloma Múltiplo/metabolismo
3.
J Clin Oncol ; 41(19): 3534-3544, 2023 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-37126762

RESUMO

PURPOSE: Polycythemia vera (PV) is characterized by JAK/STAT activation, thrombotic/hemorrhagic events, systemic symptoms, and disease transformation. In high-risk PV, ruxolitinib controls blood counts and improves symptoms. PATIENTS AND METHODS: MAJIC-PV is a randomized phase II trial of ruxolitinib versus best available therapy (BAT) in patients resistant/intolerant to hydroxycarbamide (HC-INT/RES). Primary outcome was complete response (CR) within 1 year. Secondary outcomes included duration of response, event-free survival (EFS), symptom, and molecular response. RESULTS: One hundred eighty patients were randomly assigned. CR was achieved in 40 (43%) patients on ruxolitinib versus 23 (26%) on BAT (odds ratio, 2.12; 90% CI, 1.25 to 3.60; P = .02). Duration of CR was superior for ruxolitinib (hazard ratio [HR], 0.38; 95% CI, 0.24 to 0.61; P < .001). Symptom responses were better with ruxolitinib and durable. EFS (major thrombosis, hemorrhage, transformation, and death) was superior for patients attaining CR within 1 year (HR, 0.41; 95% CI, 0.21 to 0.78; P = .01); and those on ruxolitinib (HR, 0.58; 95% CI, 0.35 to 0.94; P = .03). Serial analysis of JAK2V617F variant allele fraction revealed molecular response was more frequent with ruxolitinib and was associated with improved outcomes (progression-free survival [PFS] P = .001, EFS P = .001, overall survival P = .01) and clearance of JAK2V617F stem/progenitor cells. ASXL1 mutations predicted for adverse EFS (HR, 3.02; 95% CI, 1.47 to 6.17; P = .003). The safety profile of ruxolitinib was as previously reported. CONCLUSION: The MAJIC-PV study demonstrates ruxolitinib treatment benefits HC-INT/RES PV patients with superior CR, and EFS as well as molecular response; importantly also demonstrating for the first time, to our knowledge, that molecular response is linked to EFS, PFS, and OS.


Assuntos
Policitemia Vera , Humanos , Policitemia Vera/tratamento farmacológico , Policitemia Vera/genética , Policitemia Vera/complicações , Resultado do Tratamento , Hidroxiureia/efeitos adversos , Nitrilas/uso terapêutico , Hemorragia/complicações , Hemorragia/tratamento farmacológico
4.
Blood Adv ; 7(9): 1672-1681, 2023 05 09.
Artigo em Inglês | MEDLINE | ID: mdl-36375042

RESUMO

Chronic neutrophilic leukemia (CNL) and atypical chronic myeloid leukemia (aCML) are rare myeloid disorders that are challenging with regard to diagnosis and clinical management. To study the similarities and differences between these disorders, we undertook a multicenter international study of one of the largest case series (CNL, n = 24; aCML, n = 37 cases, respectively), focusing on the clinical and mutational profiles (n = 53 with molecular data) of these diseases. We found no differences in clinical presentations or outcomes of both entities. As previously described, both CNL and aCML share a complex mutational profile with mutations in genes involved in epigenetic regulation, splicing, and signaling pathways. Apart from CSF3R, only EZH2 and TET2 were differentially mutated between them. The molecular profiles support the notion of CNL and aCML being a continuum of the same disease that may fit best within the myelodysplastic/myeloproliferative neoplasms. We identified 4 high-risk mutated genes, specifically CEBPA (ß = 2.26, hazard ratio [HR] = 9.54, P = .003), EZH2 (ß = 1.12, HR = 3.062, P = .009), NRAS (ß = 1.29, HR = 3.63, P = .048), and U2AF1 (ß = 1.75, HR = 5.74, P = .013) using multivariate analysis. Our findings underscore the relevance of molecular-risk classification in CNL/aCML as well as the importance of CSF3R mutations in these diseases.


Assuntos
Leucemia Mieloide Crônica Atípica BCR-ABL Negativa , Leucemia Neutrofílica Crônica , Doenças Mieloproliferativas-Mielodisplásicas , Humanos , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/diagnóstico , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/genética , Leucemia Neutrofílica Crônica/diagnóstico , Leucemia Neutrofílica Crônica/genética , Epigênese Genética , Doenças Mieloproliferativas-Mielodisplásicas/genética , Mutação
5.
Clin Case Rep ; 8(12): 3549-3550, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33363972

RESUMO

Blood films are an easy tool to aid the diagnosis and management of unwell patients and should be prepared in this setting. Green-blue neutrophil inclusion bodies could be an ominous sign of poor prognosis in critically ill patients.

6.
BMJ Case Rep ; 13(3)2020 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-32205382

RESUMO

A 59-year-old man, with a background of multiply relapsed myeloma, presented with a 3-week history of confusion, short-term memory impairment and behavioural changes. CT head showed bilateral white matter changes and numerous, large lytic lesions of the skull vault. MRI brain revealed multiple areas of hyperintensity on T2-weighted sequences which did not enhance (many of which showed diffusion restriction) unexpectedly bringing progressive multifocal leukoencephalopathy (PML) into the differential. Initial cerebrospinal fluid studies were largely unremarkable, aside from a mildly elevated protein; cultures were negative. PCR for the John Cunningham (JC) virus was positive. Considering the patient's medical history and rapidily progressive symptoms, a palliative approach was adopted, with the patient dying 14 days later. We present this case as an example of PML in a patient with multiple myeloma, highlighting the need to consider this diagnosis in an enlarging population of heavily treated, severely immunocompromised, patients.


Assuntos
Antineoplásicos/uso terapêutico , Transplante de Medula Óssea , Hospedeiro Imunocomprometido , Leucoencefalopatia Multifocal Progressiva/etiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/terapia , Evolução Fatal , Humanos , Masculino , Pessoa de Meia-Idade , Transplante Autólogo
7.
Anemia ; 2016: 8494738, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27195147

RESUMO

Hemoglobin thresholds and triggers for blood transfusions have changed over the years moving from a higher to a lower level. This review article summarizes the current evidence of transfusion thresholds in the hospitalized as well as in the outpatient setting and particularly in myelodysplasia. Fatigue is the main reported symptom in this group of patients and current clinical trials are looking for a more liberal approach of red cell transfusion and the effect on quality of life as opposed to the restrictive strategy used in the critical care setting. Practical considerations, the cost effectiveness of this strategy in addition to the possible complications, and the use of quality of life questionnaires have also been reviewed.

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