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1.
Viruses ; 13(1)2021 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-33466993

RESUMO

BACKGROUND: Type-1 cryoglobulinemia (CG) is a rare disease associated with B-cell lymphoproliferative disorder. Some viral infections, such as Epstein-Barr Virus infections, are known to cause malignant lymphoproliferation, like certain B-cell lymphomas. However, their role in the pathogenesis of chronic lymphocytic leukemia (CLL) is still debatable. Here, we report a unique case of Type-1 CG associated to a CLL transformation diagnosed in the course of a human metapneumovirus (hMPV) infection. CASE PRESENTATION: A 91-year-old man was initially hospitalized for delirium. In a context of febrile rhinorrhea, the diagnosis of hMPV infection was made by molecular assay (RT-PCR) on nasopharyngeal swab. Owing to hyperlymphocytosis that developed during the course of the infection and unexplained peripheral neuropathy, a type-1 IgG Kappa CG secondary to a CLL was diagnosed. The patient was not treated for the CLL because of Binet A stage classification and his poor physical condition. CONCLUSIONS: We report the unique observation in the literature of CLL transformation and hMPV infection. We provide a mini review on the pivotal role of viruses in CLL pathophysiology.


Assuntos
Transformação Celular Viral , Suscetibilidade a Doenças , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/etiologia , Metapneumovirus/fisiologia , Infecções por Paramyxoviridae/complicações , Infecções por Paramyxoviridae/virologia , Idoso de 80 Anos ou mais , Biomarcadores , Evolução Clonal , Crioglobulinemia/diagnóstico , Crioglobulinemia/etiologia , Humanos , Imunoglobulina G/sangue , Cadeias kappa de Imunoglobulina/sangue , Imunofenotipagem , Masculino
2.
J Healthc Eng ; 2020: 6648574, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33343851

RESUMO

For the last few years, computer-aided diagnosis (CAD) has been increasing rapidly. Numerous machine learning algorithms have been developed to identify different diseases, e.g., leukemia. Leukemia is a white blood cells- (WBC-) related illness affecting the bone marrow and/or blood. A quick, safe, and accurate early-stage diagnosis of leukemia plays a key role in curing and saving patients' lives. Based on developments, leukemia consists of two primary forms, i.e., acute and chronic leukemia. Each form can be subcategorized as myeloid and lymphoid. There are, therefore, four leukemia subtypes. Various approaches have been developed to identify leukemia with respect to its subtypes. However, in terms of effectiveness, learning process, and performance, these methods require improvements. This study provides an Internet of Medical Things- (IoMT-) based framework to enhance and provide a quick and safe identification of leukemia. In the proposed IoMT system, with the help of cloud computing, clinical gadgets are linked to network resources. The system allows real-time coordination for testing, diagnosis, and treatment of leukemia among patients and healthcare professionals, which may save both time and efforts of patients and clinicians. Moreover, the presented framework is also helpful for resolving the problems of patients with critical condition in pandemics such as COVID-19. The methods used for the identification of leukemia subtypes in the suggested framework are Dense Convolutional Neural Network (DenseNet-121) and Residual Convolutional Neural Network (ResNet-34). Two publicly available datasets for leukemia, i.e., ALL-IDB and ASH image bank, are used in this study. The results demonstrated that the suggested models supersede the other well-known machine learning algorithms used for healthy-versus-leukemia-subtypes identification.


Assuntos
Aprendizado Profundo , Diagnóstico por Computador , Internet das Coisas , Leucemia/classificação , Leucemia/diagnóstico , Reconhecimento Automatizado de Padrão , Algoritmos , Computação em Nuvem , Bases de Dados Factuais , Diagnóstico por Imagem , Humanos , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnóstico , Leucemia Mieloide Aguda/diagnóstico , Aprendizado de Máquina , Redes Neurais de Computação , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Telemedicina
3.
BMJ Case Rep ; 13(12)2020 Dec 22.
Artigo em Inglês | MEDLINE | ID: mdl-33370993

RESUMO

We report a novel case of a patient who presented with new diagnoses of both cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCAs) positive vasculitis and chronic lymphocytic leukaemia (CLL). The patient was a 79-year-old man who presented with melena, haemoptysis, acute hypoxia and acute kidney injury. In the current literature, there are rare associations of c-ANCA vasculitis and malignancy, but very few, if any, relating c-ANCA vasculitis and CLL. This case is unique due to the presence of both pathologies and an uncommon presentation of the vasculitis. He presented with renal and pulmonary findings, unlike the dermal manifestations commonly seen with vasculitis. We think that this could be an easily overlooked combination of diseases and, therefore, the purpose of this case is to prevent delays in care that could affect patient outcomes and also to encourage further research into the relationship between these diseases.


Assuntos
Lesão Renal Aguda/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Leucemia Linfocítica Crônica de Células B/diagnóstico , Pneumopatias/diagnóstico , Melena/imunologia , Lesão Renal Aguda/imunologia , Lesão Renal Aguda/patologia , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Biópsia , Medula Óssea/patologia , Quimioterapia Combinada/métodos , Humanos , Rim/patologia , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/imunologia , Pulmão/diagnóstico por imagem , Pneumopatias/imunologia , Masculino , Melena/diagnóstico , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Tomografia Computadorizada por Raios X , Resultado do Tratamento
4.
Medicine (Baltimore) ; 99(45): e22787, 2020 Nov 06.
Artigo em Inglês | MEDLINE | ID: mdl-33157924

RESUMO

INTRODUCTION: Non-HIV-related visceral leishmaniasis (VL) is becoming increasingly prevalent in nontropical countries because of the increasing number of patients with chronic diseases and the development of immune-modulating drugs. PATIENT CONCERNS: Case 1 is a 60-year-old male patient of Senegalese origin presented with weight loss, lymphadenopathy, anemia, and elevated lactate dehydrogenases. Case 2 is a 46-year-old male patient of Algerian origin, with a negative HIV serology presented with cutaneous lesions. DIAGNOSIS: Patient 1: The diagnosis of stage IV lymphocytic lymphoma (LL) was confirmed by an inguinal nodal biopsy in 2013. Patient 2: The diagnosis of T-cell lymphoma was made in 2003. INTERVENTIONS: Patient 1 received 5 cycles of bendamustine and rituximab followed by a complete remission. Patient 2 was initially treated with >10 different treatments followed by 8 different chemotherapy regimens due to the disease progression. OUTCOMES: Patient 1: In 2017, after a follow-up of 4 years, the patient presented with fever, lymphadenopathy, splenomegaly, and pancytopenia in the setting of hemophagocytic syndrome. The initial diagnosis was a relapse of lymphoma and the patient was treated with ibrutinib. His status worsened, and Leishmania DNA was detected by polymerase chain reaction (PCR) on the blood and bone marrow aspirates. Ibrutinib was stopped. Amphotericin B treatment induced a complete clinical remission and clearance of Leishmania DNA from the blood.Patient 2: In 2017, after a follow-up of 14 years, the patient presented with fever, lymphadenopathy, hepatosplenomegaly, pancytopenia with hemophagocytic syndrome, and an increase in the tumor skin lesions. A skin biopsy was taken from the face and the patient. A careful reexamination of the skin biopsy revealed the presence of Leishmania bodies. He was treated with 40 mg/kg liposomal amphotericin B leading to a regression of the clinical symptoms and negativation of the blood PCR. CONCLUSIONS: This case study shows that VL may be a diagnostic challenge in patients with lymphoma. Reactivation or primary infection should be considered in the differential diagnosis. The purpose of this study is to remind clinicians to think of VL in patients with systemic symptoms that could be misdiagnosed as a progression of the underlying lymphoma.


Assuntos
Leishmaniose Visceral/complicações , Leishmaniose Visceral/diagnóstico , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Diagnóstico Diferencial , Progressão da Doença , Humanos , Leishmaniose Visceral/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/diagnóstico , Masculino , Pessoa de Meia-Idade , Indução de Remissão
5.
Am J Case Rep ; 21: e926062, 2020 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-33098641

RESUMO

BACKGROUND COVID-19 is a newly emerging disease that is not yet fully understood. It is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel virus that is easily transmitted from human to human through the respiratory route. Usually, it presents with fever, headache, fatigue accompanied by respiratory symptoms like cough and dyspnea, and other systemic involvements. Chronic lymphocytic leukemia (CLL) is a common lymphoproliferative neoplasm characterized by absolute lymphocytosis and demonstration of clonality unlike other causes of lymphocytosis. Patients with CLL are considered immunocompromised because of impaired humoral immunity (mainly) and cellular immunity. Therefore, they are vulnerable to various infections including COVID-19. Little is known about the COVID-19 infection when it unmasks CLL. CASE REPORT A 49-year-old man with no significant previous illnesses, and an unremarkable family history, presented with a moderate COVID-19 infection. He initially presented to the emergency department with fever and mild shortness of breath. A complete blood count showed a high white blood cell count with absolute lymphocytosis. Flow cytometry revealed the clonality of the lymphocytes confirming the diagnosis of CLL. Despite having CLL, he developed a moderate COVID-19 infection and recovered in a few days. To the best of our knowledge, this is the first report of CLL, which presented with a COVID-19 infection as the initial presentation. CONCLUSIONS Lymphocytosis is an unexpected finding in patients diagnosed with COVID-19 infection and the elevated lymphocytes may be indicative of other conditions. Secondary causes of lymphocytosis like malignancy or other infections should be considered in these cases.


Assuntos
Betacoronavirus , Infecções por Coronavirus/epidemiologia , Hospedeiro Imunocomprometido , Leucemia Linfocítica Crônica de Células B/epidemiologia , Pneumonia Viral/epidemiologia , Comorbidade , Humanos , Leucemia Linfocítica Crônica de Células B/diagnóstico , Masculino , Pessoa de Meia-Idade , Pandemias
6.
Pan Afr Med J ; 36: 286, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33117480

RESUMO

With the major spread of SARS-COV-2 around the world, its association with various pathologies has been reported. However, hemopathy has rarely been revealed during a coronavirus infection. The authors of this article aim to emphasize the diagnostic and therapeutic challenges faced while treating COVID/hemopathy patients.


Assuntos
Betacoronavirus , Infecções por Coronavirus/diagnóstico , Leucemia Linfocítica Crônica de Células B/diagnóstico , Pneumonia Viral/diagnóstico , Idoso , Infecções por Coronavirus/sangue , Infecções por Coronavirus/complicações , Evolução Fatal , Humanos , Achados Incidentais , Masculino , Pandemias , Pneumonia Viral/sangue , Pneumonia Viral/complicações , Avaliação de Sintomas
7.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 28(5): 1474-1479, 2020 Oct.
Artigo em Chinês | MEDLINE | ID: mdl-33067940

RESUMO

OBJECTIVE: To investigate the value of fluorescence in situ hybridization (FISH) in the diagnosis and prognosis evaluation of patients with chronic lymphocytic leukemia (CLL). METHODS: Ninty-three patients with newly diagnosed CLL were tested by five probes including RB1 (13q14.1), D13S25 (13q14.3), p53(17p13.1), ATM( 11q22.3) and CSP12, while conventional cytogenetics (CC) was used for karyotype analysis. Then the correlation of the molecular cytogenetic abnormalities with the clinical Binet stages, Rai stages and the other related laboratory examinations was analyzed. RESULTS: The detection rate of chromosome abnormality in 93 patients was 79.6%, out of which detection rate of 13q (13q- was the highest and accounted for 45.2%), followed by trisomy 12 (+12) 26.9%, p53 deletion (17p-) 19.4% and ATM deletion (11p-) 17.2%. There were 27 cases (29.0%) with 2 or more abnormalities, including 13 cases with 13q-/17q-, 5 with 13q-/11q-, and 4 with 13q-/+12. Compared with CC test results, the positive rate of FISH detection was significantly higher (χ2=32.127, P<0.01). There was no significant correlation between FISH results and Rai stages (P>0.05), meanwhile 17p- highly correlated with later stage of the Binet stages (P=0.012). The molecular cytogenetic abnormalities significantly correlated with age, absolute value of peripheral lymphocyte count and CD38 expression level (P>0.05). The incidence of 13q- in female (65.4%) was statistically significantly higher than that in male (37.3%) (P=0.015). The unmutated IGHV rate of CLL patients with a 17p- was significantly higher than that in patients without this genetic abnormality (P=0.013). The expression of CD38 was detected among 29.0% of the patients, which significantly correlated with Binet stages (P=0.027) and unmutated IGHV (P=0.006). CONCLUSION: FISH can greatly increase the detection rate of molecular cytogenetic abnormalities in CLL patients, which, as a powerful supplement to the conventional cytogenetics, can be applied for the clinical staging and prognosis evaluation of CLL patients.


Assuntos
Leucemia Linfocítica Crônica de Células B , Aberrações Cromossômicas , Citogenética , Feminino , Humanos , Hibridização in Situ Fluorescente , Cariotipagem , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/genética , Masculino
9.
Ann Afr Med ; 19(3): 203-206, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32820734

RESUMO

Background: Chronic lymphocytic leukemia (CLL) is a heterogeneous group of monoclonal forms of lymphoproliferative disorder, which is usually common among older adults. There is an increasing trend in the number of patients presenting with the disease. Aim: This study aims to determine the epidemiology pattern of CLL in Cross River state. Methodology: A retrospective study with 10-years data (2010-2019) obtained from the register of the Department of Haematology and Blood Transfusion, University of Calabar Teaching Hospital, Calabar. The data collected include the date of presentation, the age, gender, location of residence, and occupations of the patients. Results: A total of 47 cases were seen during the 10-year period, with a male: female ratio of 1:1. The mean age at presentation was 59 years. The majority of the patients were in their fifth and sixth decades of life. Most patients (44.68%) practice farming as their profession. Conclusion: The study has reawaken our consciousness on the increasing trend on the epidemiological burden of CLL in our environment and will help to enhance further investigation into the relationship between the rising trend and available possible risk factors in our environment.


Assuntos
Leucemia Linfocítica Crônica de Células B/epidemiologia , Idoso , Feminino , Hospitais de Ensino , Humanos , Incidência , Leucemia Linfocítica Crônica de Células B/diagnóstico , Masculino , Pessoa de Meia-Idade , Nigéria/epidemiologia , Estudos Retrospectivos , Fatores de Risco
11.
Am Surg ; 86(9): 1208-1211, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32683914

RESUMO

Atypical spindle cell lipomatous neoplasm, also known as well-differentiated spindle cell liposarcoma, represents a newly discovered entity of adipocytic tumors. Recent research has shown this tumor variant to be more related to spindle cell lipoma, rather than the originally hypothesized atypical lipomatous tumor spectrum. Here we present a case of a 58-year-old man with a history of chronic lymphocytic leukemia with an enlarging mass on the posterior left shoulder, initially hypothesized to be a benign lipoma. However, magnetic resonance imaging showed a large, multiseptated, heterogeneous mass concerning for soft tissue sarcoma. After resection, pathologic analysis showed cells closely resembling spindle cell lipoma, with additional cellular and fascicular zones containing lipoblasts and mitotic figures. Molecular analysis showed no MDM2 amplification. This lack of amplification indicates this tumor is distinctly different from an atypical lipomatous tumor, which characteristically displays MDM2 amplification. However, tumor expression of RB1 was normal. The majority of atypical spindle cell lipomatous neoplasms are associated with RB1 deletions. We conclude that we have a unique example of an atypical spindle cell lipomatous tumor.


Assuntos
Procedimentos Cirúrgicos Dermatológicos/métodos , Leucemia Linfocítica Crônica de Células B/complicações , Lipossarcoma/cirurgia , Neoplasias Cutâneas/cirurgia , Biópsia , Diagnóstico Diferencial , Humanos , Leucemia Linfocítica Crônica de Células B/diagnóstico , Lipossarcoma/diagnóstico , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/diagnóstico
12.
Leukemia ; 34(11): 2934-2950, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32404973

RESUMO

Drug combinations that target critical pathways are a mainstay of cancer care. To improve current approaches to combination treatment of chronic lymphocytic leukemia (CLL) and gain insights into the underlying biology, we studied the effect of 352 drug combination pairs in multiple concentrations by analysing ex vivo drug response of 52 primary CLL samples, which were characterized by "omics" profiling. Known synergistic interactions were confirmed for B-cell receptor (BCR) inhibitors with Bcl-2 inhibitors and with chemotherapeutic drugs, suggesting that this approach can identify clinically useful combinations. Moreover, we uncovered synergistic interactions between BCR inhibitors and afatinib, which we attribute to BCR activation by afatinib through BLK upstream of BTK and PI3K. Combinations of multiple inhibitors of BCR components (e.g., BTK, PI3K, SYK) had effects similar to the single agents. While PI3K and BTK inhibitors produced overall similar effects in combinations with other drugs, we uncovered a larger response heterogeneity of combinations including PI3K inhibitors, predominantly in CLL with mutated IGHV, which we attribute to the target's position within the BCR-signaling pathway. Taken together, our study shows that drug combination effects can be effectively queried in primary cancer cells, which could aid discovery, triage and clinical development of drug combinations.


Assuntos
Antineoplásicos/farmacologia , Avaliação Pré-Clínica de Medicamentos , Resistencia a Medicamentos Antineoplásicos/genética , Leucemia Linfocítica Crônica de Células B/genética , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais , Linhagem Celular Tumoral , Relação Dose-Resposta a Droga , Avaliação Pré-Clínica de Medicamentos/métodos , Avaliação Pré-Clínica de Medicamentos/normas , Sinergismo Farmacológico , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Sequenciamento de Nucleotídeos em Larga Escala/normas , Humanos , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Cultura Primária de Células , Inibidores de Proteínas Quinases/farmacologia , Inibidores de Proteínas Quinases/uso terapêutico , Proteínas Proto-Oncogênicas c-bcl-2/antagonistas & inibidores , Proteínas Proto-Oncogênicas c-bcl-2/genética , Proteínas Proto-Oncogênicas c-bcl-2/metabolismo , Receptores de Antígenos de Linfócitos B/antagonistas & inibidores , Receptores de Antígenos de Linfócitos B/genética , Receptores de Antígenos de Linfócitos B/metabolismo , Reprodutibilidade dos Testes
13.
Medicine (Baltimore) ; 99(19): e20115, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32384487

RESUMO

B lymphocytic leukemia (B-ALL) is a hematopoietic malignant disease characterized by an accumulation of early B cells. This study aimed to construct a children B-ALL Nomogram prediction model based on Therapeutically Applicable Research to Generate Effective Treatments database, so as to further guide clinical diagnose and treatment.Clinical data related to children B-ALL were collected from the TARGET database, among which, the stage II clinical data were used as the prediction model, while the stage I clinical data were utilized as the external verification model. The stage II clinical factors were analyzed through Lasso regression analysis to screen the risk factors for the construction of Nomogram prediction model. In addition, the model prediction capacity and accuracy were verified internally and externally using the ROC curve, C-index and calibration curve, respectively.A total of 1316 B-ALL children were enrolled in this study. Lasso regression analysis revealed that, Age, Gender, WBC, CNSL, MRD29, BMR, CNS R, BCR-ABL1, BMA29, DS, and DI were the important prognostic risk factors. The C-index values of internal and external verification models were 0.870 and 0.827, respectively, revealing the ideal model discriminating capacity. Besides, the calibration curve had high contact ratio, which suggested favorable consistency between the incidence predicted by the model and the actual incidence. Moreover, the AUC values of the ROC curve were 0.858, 0.787, 0.898, and 0.867, respectively, indicating high model prediction accuracy in predicting the 3- and 5-year survival rates of children with B-ALL.The Nomogram prediction model plotted in this study exhibits favorable prediction capacity and clinical practicability for the survival rate of B-ALL children, which contributes to patients screening and clinical intervention.


Assuntos
Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/mortalidade , Leucemia Linfocítica Crônica de Células B/patologia , Nomogramas , Adolescente , Fatores Etários , Criança , Feminino , Humanos , Masculino , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Curva ROC , Análise de Regressão , Fatores de Risco , Fatores Sexuais , Taxa de Sobrevida
14.
Leuk Res ; 91: 106335, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32114372

RESUMO

We performed a retrospective study comparing treatment patterns and overall survival (OS) in chronic lymphocytic leukemia (CLL) patients with the advent of ibrutinib to provide current real-world data. METHODS: Using a provincial population-based database, we analyzed CLL patients who received upfront treatment in British Columbia before ibrutinib availability (1984-2014), during ibrutinib access for: relapse only (2014-2015) and for upfront treatment of patients (with 17p deletion or unfit for chemotherapy) (2015-2016). Analysis included up to third-line treatment. RESULTS: Of 1729 patients meeting inclusion criteria (median age, 66 years; 1466, period 1; 140, period 2; 123, period 3), FR was the most common first-line therapy (35.8 %, 54.3 % and 40.7 %, periods 1-3, respectively) and 18.7 % received ibrutinib upfront in period 3. The most common therapies in relapse were chemoimmunotherapy (36.1 % and 55.6 %, periods 1 and 2, second-line; 29.2 %, period 1, third-line) and ibrutinib (69.8 %, period 3, second-line; 46.4 % and 70.3 %, periods 2 and 3, third-line). OS improved for patients treated in periods 2-3 over period 1 (median OS not reached vs. 11.9 years, p < 0.001; no difference in OS for periods 2-3, p = 0.385). CONCLUSION: Ibrutinib has replaced chemoimmunotherapy as the preferred therapy in relapse. Overall survival has improved over time with access to ibrutinib.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Leucemia Linfocítica Crônica de Células B/terapia , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Síndrome de Smith-Magenis/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Colúmbia Britânica , Deleção Cromossômica , Cromossomos Humanos Par 17/genética , Ciclofosfamida/uso terapêutico , Gerenciamento Clínico , Doxorrubicina/uso terapêutico , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/genética , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Pessoa de Meia-Idade , Neoplasia Residual , Prednisona/uso terapêutico , Prognóstico , Recidiva , Indução de Remissão , Estudos Retrospectivos , Rituximab/uso terapêutico , Síndrome de Smith-Magenis/diagnóstico , Síndrome de Smith-Magenis/genética , Síndrome de Smith-Magenis/mortalidade , Análise de Sobrevida , Transplante Homólogo , Resultado do Tratamento , Vidarabina/análogos & derivados , Vidarabina/uso terapêutico , Vincristina/uso terapêutico
15.
Ann Agric Environ Med ; 27(1): 160-164, 2020 Mar 17.
Artigo em Inglês | MEDLINE | ID: mdl-32208597

RESUMO

Richter's syndrome (RS) is a rare complication in which chronic lymphocytic leukaemia (CLL) or small lymphocytic lymphoma (SLL) transforms into a more aggressive type of lymphoma - diffuse large B cell lymphoma (DLBCL), or Hodgkin's lymphoma (HL). The review describes the clinical case of a patient with CLL and RS diagnosis. A computed tomography (CT) scan of the abdominal cavity detected numerous normodense areas in the liver. Simultaneously, ultrasound examination (USG) of the thyroid revealed the presence of a solid hypoechogenic lump. The material sampled from closed biopsies of liver and thyroid in both cases allowed the diagnosis of diffuse large B cell lymphoma (DLBCL). The liver and the thyroid are particularly rare locations of RS. However, those cases allowed the conclusion that RS may occur even in a very unexpected and less probable location.


Assuntos
Leucemia Linfocítica Crônica de Células B/diagnóstico , Neoplasias Hepáticas/patologia , Linfoma Difuso de Grandes Células B/diagnóstico , Neoplasias da Glândula Tireoide/patologia , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/patologia , Neoplasias Hepáticas/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/patologia , Pessoa de Meia-Idade , Neoplasias da Glândula Tireoide/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Ultrassonografia
18.
Am J Case Rep ; 21: e921131, 2020 Mar 09.
Artigo em Inglês | MEDLINE | ID: mdl-32150530

RESUMO

BACKGROUND Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and mantle cell lymphoma (MCL) both have a common origin arising from mature CD5+ B-lymphocytes. Their distinction is crucial since MCL is a considerably more aggressive disease. Composite lymphoma consisting of CLL/SLL and MCL has been rarely reported. This type of composite lymphoma may be under-diagnosed as the 2 neoplasms have many features in common, both morphologically and immunophenotypically. CASE REPORT We report the case of a 57-year-old male patient who presented with a 4-month history of recurrent abdominal pain and distention with hepatosplenomegaly. Peripheral blood showed a high leukocytes count (46.7×10³/uL) with marked lymphocytosis of 35.0×10³/uL, mostly small mature-looking, with some showing nuclear irregularities, with approximately 3% prolymphocytes. Immunophenotyping by flow cytometry and immunohistochemistry revealed 2 immunophenotypically distinct abnormal CD5+monotypic B-cell populations. Fluorescence in situ hybridization (FISH) on peripheral blood demonstrated IGH/CCND1 rearrangement consistent with t(11;14) in 65% of cells analyzed. Accordingly, based on compilation of findings from morphology, flow cytometry, immunohistochemistry, and FISH, A diagnosis of composite lymphoma consisting of MCL; small cell variant and CLL/SLL was concluded. CONCLUSIONS We describe a case of composite lymphoma of MCL (small cell variant) and CLL/SLL that emphasizes the crucial role of the multiparametric approach, including vigilant cyto-histopathologic examination, immunophenotyping by flow cytometry and immunohistochemistry, as well as genetic testing, to achieve the correct diagnosis.


Assuntos
Linfoma Composto/diagnóstico , Linfoma Composto/patologia , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/patologia , Linfoma de Célula do Manto/diagnóstico , Linfoma de Célula do Manto/patologia , Biomarcadores Tumorais , Diagnóstico Diferencial , Citometria de Fluxo , Rearranjo Gênico , Humanos , Imuno-Histoquímica , Imunofenotipagem , Hibridização in Situ Fluorescente , Masculino , Pessoa de Meia-Idade , Proteínas de Fusão Oncogênica/genética
19.
Blood ; 135(15): 1244-1254, 2020 04 09.
Artigo em Inglês | MEDLINE | ID: mdl-32006000

RESUMO

CD49d is a remarkable prognostic biomarker of chronic lymphocytic leukemia (CLL). The cutoff value for the extensively validated 30% of positive CLL cells is able to separate CLL patients into 2 subgroups with different prognoses, but it does not consider the pattern of CD49d expression. In the present study, we analyzed a cohort of 1630 CLL samples and identified the presence of ∼20% of CLL cases (n = 313) characterized by a bimodal expression of CD49d, that is, concomitant presence of a CD49d+ subpopulation and a CD49d- subpopulation. At variance with the highly stable CD49d expression observed in CLL patients with a homogeneous pattern of CD49d expression, CD49d bimodal CLL showed a higher level of variability in sequential samples, and an increase in the CD49d+ subpopulation over time after therapy. The CD49d+ subpopulation from CD49d bimodal CLL displayed higher levels of proliferation compared with the CD49d- cells; and was more highly represented in the bone marrow compared with peripheral blood (PB), and in PB CLL subsets expressing the CXCR4dim/CD5bright phenotype, known to be enriched in proliferative cells. From a clinical standpoint, CLL patients with CD49d bimodal expression, regardless of whether the CD49d+ subpopulation exceeded the 30% cutoff or not, experienced clinical behavior similar to CD49d+ CLL, both in chemoimmunotherapy (n = 1522) and in ibrutinib (n = 158) settings. Altogether, these results suggest that CD49d can drive disease progression in CLL, and that the pattern of CD49d expression should also be considered to improve the prognostic impact of this biomarker in CLL.


Assuntos
Integrina alfa4/análise , Leucemia Linfocítica Crônica de Células B/patologia , Proliferação de Células/efeitos dos fármacos , Progressão da Doença , Humanos , Imunoterapia , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/terapia , Prognóstico , Modelos de Riscos Proporcionais , Inibidores de Proteínas Quinases/uso terapêutico , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico
20.
Am J Clin Pathol ; 153(5): 646-655, 2020 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-31953940

RESUMO

OBJECTIVES: Lymphoid enhancer binding factor 1 (LEF1) is expressed in most cases of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and has shown utility in distinguishing CLL/SLL from other small B-cell lymphomas. LEF1 expression has not been systematically studied in CD5-positive marginal zone lymphomas (MZLs), lymphoplasmacytic lymphomas (LPLs), and follicular lymphomas (FLs). We evaluated whether these cases lacked LEF1, helping to distinguish them from CLL/SLL. METHODS: MZLs, LPLs, and FLs expressing CD5 were retrospectively studied for expression of LEF1 by immunohistochemistry. RESULTS: LEF1 was absent in 17 of 18 CD5-positive lymphomas including 13 MZLs (2 nodal, 3 splenic, and 8 mucosa-associated lymphoid tissue lymphomas), 3 LPLs, and 1 of 2 FLs. One grade 3A CD5-positive FL expressed LEF1 in a majority of tumor cells. CONCLUSIONS: LEF1 is not expressed in most CD5-positive MZLs and LPLs; therefore, it is a reliable marker for distinguishing them from CLL/SLL. LEF1 may be expressed in CD5-positive FLs.


Assuntos
Antígenos CD5/metabolismo , Leucemia Linfocítica Crônica de Células B/diagnóstico , Fator 1 de Ligação ao Facilitador Linfoide/metabolismo , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma Folicular/diagnóstico , Macroglobulinemia de Waldenstrom/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Leucemia Linfocítica Crônica de Células B/metabolismo , Leucemia Linfocítica Crônica de Células B/patologia , Linfoma de Zona Marginal Tipo Células B/metabolismo , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma Folicular/metabolismo , Linfoma Folicular/patologia , Masculino , Pessoa de Meia-Idade , Macroglobulinemia de Waldenstrom/metabolismo , Macroglobulinemia de Waldenstrom/patologia
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