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1.
Intern Med ; 2020 Jan 17.
Artículo en Inglés | MEDLINE | ID: mdl-31956206

RESUMEN

A 69-year-old man with palpitations and decreased blood pressure was referred. Echocardiography showed a mass in the right atrium and cardiac septum. The serum IgG4 level was 1450 mg/dl. A biopsy of the cardiac mass showed fibrosis with inflammatory cells and increased IgG4-positive plasma cells and lymphocytes. Flow cytometry and polymerase chain reaction of the immunoglobulin heavy chain did not demonstrate monoclonality. He was diagnosed with IgG4-related disease (IgG4-RD). IgG4-RD with a cardiac mass is rare and it is difficult to distinguish it from malignant lymphoma by a pathological examination alone. We therefore performed a biopsy and analyzed the clonality in order to make an accurate diagnosis of IgG4-RD.

3.
Br J Haematol ; 184(4): 570-577, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30417943

RESUMEN

The chromosomal abnormalities associated with follicular lymphoma (FL) prognosis are not fully elucidated. Here, we evaluated the pattern of chromosomal abnormalities in FL, and clarified the correlations between the cytogenetic features and clinical outcome. Cytogenetic analysis was performed using standard methods of Giemsa-banding at diagnosis for 201 FL patients admitted to our hospitals between 2001 and 2013. The identified chromosomal abnormalities were: t(14;18)(q32;q21) (59·2%), +X (17·9%), del(6)(q)/-6 (16·9%), +7 (14·4%), abnormality of 1q12-21/1q (12·9%), del(13)(q)/-13 (11·9%), abnormality of 3q27 (10·4%), abnormality of 10q22-24 (10·0%), +12/dup(12)(q) (10·0%), abnormality of 1p21-22/1p (9·0%), +18 (9·0%), del(17)(p)/-17 (5·0%), and a complex karyotype (54·7%). Patients with trisomy 21 had a significantly shorter progression-free survival (P = 0·00171) and overall survival (OS) (P < 0·001) than those without trisomy 21; additionally, patients with trisomy 21 in the rituximab-treated cohort also had a significantly shorter OS (P = 0·000428). Multivariate analysis identified trisomy 21 as an independent risk factor in our cohorts with or without t(14;18) (P = 0·015). In conclusion, the presence of trisomy 21 was an independent risk factor for in FL. Chromosomal analysis of FL patients at diagnosis can provide useful information about their expected survival.


Asunto(s)
Cromosomas Humanos Par 21/genética , Linfoma Folicular/genética , Linfoma Folicular/microbiología , Trisomía , Adulto , Anciano , Anciano de 80 o más Años , Supervivencia sin Enfermedad , Femenino , Humanos , Linfoma Folicular/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Factores de Riesgo , Rituximab/administración & dosificación , Tasa de Supervivencia
4.
Int J Hematol ; 109(1): 91-97, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30203253

RESUMEN

90Y-ibritumomab tiuxetan (90Y-IT) is widely used, but the factors responsible for its optimal treatment effects are unknown. We enrolled 34 patients with relapsed indolent lymphoma treated with 90Y-IT monotherapy at Gunma University Hospital between 2003 and 2014 in the present study. Clinical data including computed tomography and 18-Fluoro-deoxyglucose positron emission tomography were retrospectively analyzed. The overall response rate and complete response rate were 91% and 82%, respectively. The median progression-free survival (PFS) and overall survival were 32 months and not reached, respectively. In univariate analysis, tumor long-axis diameter ≤ 2.5 cm, maximum standardized uptake value (SUVmax) ≤ 6.5, localized disease, normal levels of serum soluble interleukin-2 receptor, and the number of involved nodal sites ≤ 3 immediately prior to 90Y-IT were associated with median PFS greater than 6 years. However, in multivariate analysis, only tumor long-axis diameter ≤ 2.5 cm and SUVmax ≤ 6.5 affected PFS [hazard ratio (HR) 0.130, P = 0.0021 and HR 0.283, P = 0.0311, respectively]. Patients with only one prior regimen needed less granulocyte colony-stimulating factor and platelet transfusion. Thus, 90Y-IT treatment should be considered for patients with indolent lymphoma in first relapse who have tumor long-axis diameter ≤ 2.5 cm and SUVmax ≤ 6.5.


Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Linfoma no Hodgkin/radioterapia , Diagnóstico por Imagen , Linfoma no Hodgkin/diagnóstico por imagen , Linfoma no Hodgkin/mortalidad , Linfoma no Hodgkin/patología , Tomografía de Emisión de Positrones , Valor Predictivo de las Pruebas , Recurrencia , Inducción de Remisión , Estudios Retrospectivos , Análisis de Supervivencia , Factores de Tiempo , Tomografía Computarizada por Rayos X , Radioisótopos de Itrio/uso terapéutico
5.
J Pediatr Hematol Oncol ; 40(3): e171-e175, 2018 04.
Artículo en Inglés | MEDLINE | ID: mdl-29200172

RESUMEN

We describe a rare case of chronic active Epstein-Barr virus (CAEBV) infection, with infiltration of the skeletal muscle. A 19-year-old woman with swollen cervical lymph nodes and a fever was referred to our hospital. Swelling of the trapezium muscle and elevation of creatinine kinase level were observed. Biopsy results of the brachialis muscle revealed infiltration of Epstein-Barr virus (EBV)-encoded RNA-positive CD8 T lymphocytes. The EBV virus load in the peripheral blood was high, and EBV monoclonality was determined by Southern blot analysis. Owing to the rarity of CAEBV with skeletal muscle infiltration, this case alerts physicians to the potential diagnostic pitfalls of CAEBV.


Asunto(s)
Linfocitos T CD8-positivos/virología , Infecciones por Virus de Epstein-Barr/patología , Músculo Esquelético/patología , Enfermedad Crónica , Femenino , Herpesvirus Humano 4 , Humanos , Miositis/patología , Miositis/virología , Adulto Joven
6.
Intern Med ; 56(20): 2753-2757, 2017 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-28924112

RESUMEN

A 41-year-old woman treated with isoniazid (INH) for latent tuberculosis infection and an oral corticosteroid for sarcoidosis developed severe anemia two months after initiating INH. A bone marrow examination showed erythroblastopenia, and a diagnosis of INH-induced pure red cell aplasia (PRCA) was made. Her reticulocyte count and hemoglobin levels improved two weeks after discontinuation of INH. A literature review of INH-induced PRCA shows that it occurs very rarely in the context of autoimmune disorders. This report describes a case of INH-induced PRCA occurring in a patient with sarcoidosis.


Asunto(s)
Antituberculosos/administración & dosificación , Isoniazida/efectos adversos , Tuberculosis Latente/tratamiento farmacológico , Aplasia Pura de Células Rojas/inducido químicamente , Corticoesteroides/uso terapéutico , Adulto , Antituberculosos/uso terapéutico , Femenino , Humanos , Isoniazida/uso terapéutico , Tuberculosis Latente/complicaciones , Sarcoidosis/complicaciones , Sarcoidosis/tratamiento farmacológico
7.
Br J Haematol ; 179(3): 449-460, 2017 11.
Artículo en Inglés | MEDLINE | ID: mdl-28770558

RESUMEN

Extramedullary myeloma (EMM) occurs when myeloma develops outside the bone marrow; it often develops after chemotherapy and is associated with the acquisition of chemo-resistance and a fatal course. The mechanisms underlying extramedullary spread have not yet been fully elucidated. MALAT1 is a highly abundantly and ubiquitously expressed long non-coding RNA that plays important roles in cancer metastasis. The aims of this study were to clarify the association of MALAT1 with EMM and to elucidate the underlying mechanism of EMM formation under chemotherapeutic pressure. MALAT1 expression was significantly higher in multiple myeloma (MM) than in monoclonal gammopathy of undetermined significance. Furthermore, MALAT1 expression was markedly higher in EMM compared with that in corresponding intramedullary myeloma cells. A higher MALAT1 level was associated with shorter overall and progression-free survival. MALAT1 expression level was positively correlated with expression of HSP90AA1, HSP90AB1 and HSP90B1 but not with TP53 expression. MALAT1 was significantly upregulated by bortezomib and doxorubicin. Considering the known functions of MALAT1, our results suggest that it acts as a stress response gene that is upregulated by chemotherapy, thereby linking chemotherapy to EMM formation. Elucidating the biological implication of long non-coding RNA contributes to deeper understanding concerning the pathogenesis and investigation of novel therapeutic targets for MM.


Asunto(s)
Biomarcadores de Tumor/genética , Mieloma Múltiple/patología , ARN Largo no Codificante/genética , Antineoplásicos/farmacología , Biomarcadores de Tumor/metabolismo , Bortezomib/farmacología , Progresión de la Enfermedad , Doxorrubicina/farmacología , Femenino , Regulación Neoplásica de la Expresión Génica/efectos de los fármacos , Humanos , Masculino , Gammopatía Monoclonal de Relevancia Indeterminada/genética , Gammopatía Monoclonal de Relevancia Indeterminada/metabolismo , Mieloma Múltiple/genética , Pronóstico , ARN Largo no Codificante/biosíntesis , ARN Neoplásico/biosíntesis , ARN Neoplásico/genética , Estrés Fisiológico/genética , Análisis de Supervivencia , Células Tumorales Cultivadas
8.
Ann Hematol ; 95(9): 1465-72, 2016 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-27358178

RESUMEN

There are some reports regarding hepatitis B virus (HBV) reactivation in patients with myeloma who are HBV carriers or who have had a resolved HBV infection, and there is no standard prophylaxis strategy for these patients. We performed a retrospective multicenter study to determine the incidence and characteristics of HBV reactivation in patients with multiple myeloma. We identified 641 patients with multiple myeloma who had been treated using novel agents and/or autologous stem cell transplantation with high-dose chemotherapy between January 2006 and June 2014 at nine Japanese hospitals. The patients' characteristics, laboratory data, and clinical courses were retrieved and statistically analyzed. During a median follow-up of 101 weeks, one of eight (12.5 %) HBV carriers developed hepatitis and 9 of 99 (9.1 %) patients with resolved HBV infection experienced HBV reactivation; the cumulative incidences of HBV reactivation at 2 years (104 weeks) and 5 years (260 weeks) were 8 and 14 %, respectively. The nine cases of reactivation after resolved HBV infection had received entecavir as preemptive therapy or were carefully observed by monitoring their HBV DNA levels, and none of these cases developed hepatitis. Among patients with multiple myeloma, HBV reactivation was not rare. Therefore, long-term monitoring of HBV DNA levels is needed to prevent hepatitis that is related to HBV reactivation in these patients.


Asunto(s)
Virus de la Hepatitis B/fisiología , Hepatitis B/virología , Mieloma Múltiple/terapia , Activación Viral/fisiología , Anciano , Anciano de 80 o más Años , Antineoplásicos/uso terapéutico , Terapia Combinada , ADN Viral/análisis , ADN Viral/genética , Femenino , Hepatitis B/complicaciones , Hepatitis B/epidemiología , Virus de la Hepatitis B/genética , Humanos , Incidencia , Japón/epidemiología , Masculino , Mieloma Múltiple/complicaciones , Mieloma Múltiple/epidemiología , Reacción en Cadena en Tiempo Real de la Polimerasa , Estudios Retrospectivos , Trasplante de Células Madre/métodos , Trasplante Autólogo
9.
Int J Hematol ; 103(2): 219-26, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26588928

RESUMEN

The incidence of chronic lymphocytic leukemia (CLL) is low in Japan. The clinical course ranges from very indolent to rapidly progressive. Recently, several reports have indicated that mutation of the splicing factor 3b, subunit 1 (SF3B1) gene in CLL is predictive of a poor prognosis. Here, we investigated the SF3B1 mutational status of Japanese CLL patients and clarified the association between SF3B1 mutational status and prognostic factors. One hundred and two patients that were referred to our institutions between 1999 and 2013 were enrolled. Mutation analysis of SF3B1 (n = 87) and of the immunoglobulin heavy chain gene (IGHV) (n = 102) was performed at diagnosis. FISH analysis of del(11)(q22) was performed for 17 patients. Seven patients have SF3B1 mutation (8.0 %: K700E, 5/7; G742D, 1/7 and Y623C, 1/7). The median survival times for patients with mutated and non-mutated SF3B1 were 53 and 130 months, respectively. Overall survival of the mutated SF3B1 group was significantly lower than that of the non-mutated group (p = 0.0187). No relationship was observed between IGHV mutational status and SF3B1 mutation. There was no patient with SF3B1 mutation in the IGHV1-69 population (0/2). In conclusion, mutation of SF3B1 at diagnosis in Japanese CLL patients is predictive of a poor prognosis.


Asunto(s)
Cadenas Pesadas de Inmunoglobulina/genética , Leucemia Linfocítica Crónica de Células B/genética , Mutación , Fosfoproteínas/genética , Factores de Empalme de ARN/genética , Adulto , Anciano , Anciano de 80 o más Años , Grupo de Ascendencia Continental Asiática , Progresión de la Enfermedad , Femenino , Humanos , Leucemia Linfocítica Crónica de Células B/diagnóstico , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico
10.
Rinsho Ketsueki ; 56(12): 2441-6, 2015 12.
Artículo en Japonés | MEDLINE | ID: mdl-26725352

RESUMEN

An 80-year-old man, presenting with gait disturbance and memory loss, had findings of normal pressure hydrocephalus. Primary leptomeningeal lymphoma (PLML) was diagnosed based on cytology and flow cytometry of cerebrospinal fluid obtained by examination. Gadolinium-enhanced MRI showed enhancement of the brain and spinal cord but FDG-PET/CT revealed no lymph node swelling. With intrathecal chemotherapy, meningeal lesions disappeared and the gait disturbance and memory loss improved. However, the disease recurred three months later, manifesting as left facial nerve palsy, but the symptoms disappeared in response to intrathecal chemotherapy and systemic rituximab administration. Although a tumor lesion in the spinal canal was suggested by MRI examination, the patient has maintained a good clinical course for four years with intrathecal chemotherapy every three months. PLML is a very rare disease and its diagnosis is difficult. Repeated intrathecal chemotherapy appeared to be effective against PLML in this case.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Hidrocéfalo Normotenso/tratamiento farmacológico , Linfoma de Células B/diagnóstico , Linfoma de Células B/tratamiento farmacológico , Carcinomatosis Meníngea/diagnóstico , Carcinomatosis Meníngea/tratamiento farmacológico , Recurrencia Local de Neoplasia/tratamiento farmacológico , Anciano de 80 o más Años , Dexametasona/administración & dosificación , Humanos , Hidrocéfalo Normotenso/complicaciones , Hidrocéfalo Normotenso/etiología , Linfoma de Células B/complicaciones , Masculino , Carcinomatosis Meníngea/complicaciones , Recurrencia Local de Neoplasia/complicaciones , Recurrencia Local de Neoplasia/diagnóstico , Rituximab/administración & dosificación , Resultado del Tratamiento
11.
Rinsho Ketsueki ; 56(12): 2447-51, 2015 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-26725353

RESUMEN

Testicular lymphoma is a rare disease, accounting for 1-2% of non-Hodgkin lymphoma and 5-9% of all testicular tumors, and has a high relapse rate with a poor prognosis. We report a patient with testicular diffuse large B-cell lymphoma (DLBCL) who relapsed after being in remission for 16 years. He had undergone orchiectomy of the right testis and was diagnosed as having DLBCL (stage IAE) at 49 years of age. After 3 cycles of CHOP, he achieved a complete remission. Orchiectomy was performed because of a left testicular tumor, and he was again diagnosed with DLBCL at the age of 65. VH3-21 was detected in lymphoma cells at the times of both the first diagnosis and the relapse based on analysis of the variable region of the immunoglobulin heavy chain. Accordingly, the lymphoma cells at relapse were confirmed to be the same clone as that which had been documented at the first diagnosis.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Linfoma de Células B Grandes Difuso/terapia , Neoplasias Testiculares/tratamiento farmacológico , Anciano , Humanos , Linfoma de Células B Grandes Difuso/diagnóstico , Masculino , Recurrencia , Inducción de Remisión , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/patología , Factores de Tiempo
12.
Acta Haematol ; 133(1): 83-8, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25170761

RESUMEN

INTRODUCTION: In some previous studies, vitamin B12 treatment showed immunomodulatory effects and restored the immunological abnormalities in patients with pernicious anemia (PA). In the present study, peripheral blood T cell subsets, including regulatory T cells (T(reg)s), were examined before and after vitamin B12 treatment in PA patients. PATIENTS AND METHODS: The percentages of CD4, CD8, Th1, Th2 and T(reg)s were examined in 23 PA patients before vitamin B12 treatment, in 23 other PA patients after vitamin B12 treatment and in 28 healthy controls. RESULTS: The mean percentage of CD8+ T cells was significantly higher in the control group (23.0%; 95% CI, 20.4-25.6%) than in the pre- (16.0%; 95% CI, 12.1-20.0%) and posttreatment groups (15.2%; 95% CI, 11.8-18.6%; p < 0.05). The CD4/CD8 ratio was significantly lower in the control group (2.01; 95% CI, 1.66-2.34) than in the pre- (3.45; 95% CI, 2.55-7.80) and posttreatment groups (2.97; 95% CI, 2.22-3.72; p < 0.05). There was no significant difference in the mean Th1/Th2 ratio among these groups. There were significant increases in the mean percentage of T(reg)s in the pre- (6.29%; 95% CI, 5.04-7.54%) and posttreatment groups (7.77%; 95% CI, 6.34-9.20%) compared with the control group (4.18%; 95% CI, 3.92-4.47%; p < 0.05). CONCLUSIONS: The percentage of T(reg)s was significantly higher in PA patients than in normal subjects, and this high T(reg) percentage was not different before and after vitamin B12 treatment. Other immunological alterations also did not recover after vitamin B12 treatment, so that these immunological changes appear to be the cause of PA and are not induced by vitamin B12 deficiency.


Asunto(s)
Anemia Perniciosa/sangre , Anemia Perniciosa/tratamiento farmacológico , Linfocitos T Reguladores , Vitamina B 12/uso terapéutico , Adulto , Anciano , Anciano de 80 o más Años , Anemia Perniciosa/inmunología , Antígenos de Superficie/metabolismo , Recuento de Linfocito CD4 , Relación CD4-CD8 , Estudios de Casos y Controles , Femenino , Humanos , Inmunofenotipificación , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Subgrupos de Linfocitos T/inmunología , Subgrupos de Linfocitos T/metabolismo , Linfocitos T Reguladores/inmunología , Linfocitos T Reguladores/metabolismo , Resultado del Tratamiento , Adulto Joven
13.
Rinsho Ketsueki ; 55(6): 687-91, 2014 Jun.
Artículo en Japonés | MEDLINE | ID: mdl-24975338

RESUMEN

Herein, we report a patient with polycythemia vera (PV) who exhibited Philadelphia chromosome (Ph) positive CML-like clinical features after 13 years of hydroxycarbamide administration and successful treatment with a tyrosine kinase inhibitor (TKI). She was 64 years old when initially diagnosed with PV and was confirmed to be negative for BCR-ABL translocation. Thirteen years later, with increasing white blood cell and platelet counts, a BCR-ABL positive clone emerged and the JAK2V617F mutation disappeared. After TKI treatment, the BCR-ABL copy number decreased and the JAK2V617F mutation was again detected. Furthermore, MPN clinical features were observed. This case provides insights into the clonal divergence and growth advantage of the Ph positive clone over the MPN clone. Whether JAK2V617F is an MPN initiating event or a secondary mutation has been a point of discussion for the past several years. This issue is also considered in the present report.


Asunto(s)
Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Policitemia Vera/tratamiento farmacológico , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirimidinas/uso terapéutico , Tiazoles/uso terapéutico , Dasatinib , Femenino , Proteínas de Fusión bcr-abl/genética , Humanos , Janus Quinasa 2/genética , Leucemia Mielógena Crónica BCR-ABL Positiva/complicaciones , Leucemia Mielógena Crónica BCR-ABL Positiva/genética , Persona de Mediana Edad , Mutación , Policitemia Vera/complicaciones , Policitemia Vera/genética , Factores de Tiempo
14.
J Clin Exp Hematop ; 53(3): 197-205, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24369221

RESUMEN

We evaluated the prognostic significance of the serum level of the soluble form of interleukin-2 receptorα (sIL-2Rα) and investigated its association with CD25 expression on tumor cells in diffuse large B-cell lymphoma (DLBCL). Three hundred and thirty-eight adult patients with newly diagnosed DLBCL were eligible for this retrospective study. 32.2% of patients were treated with CHOP-like regimen and 67.8% with R-CHOP-like regimen. CD25 expression on the surface of tumor cells was evaluated in 143 cases and its relationship with sIL-2Rα level was also investigated. Both overall survival (OS) and progression-free survival (PFS) were poorer in patients with higher sIL-2Rα, in both R-CHOP and CHOP groups. sIL-2Rα > 1,000 U/mL and performance status (PS) ≥ 2 were independently associated with poorer OS, and sIL-2Rα > 1,000 U/mL, age > 60 years, and ≥ 2 extranodal sites were independently associated with poorer PFS in the R-CHOP group. The sIL-2Rα level was higher in the CD25-positive group than in the CD25-negative group in stage 3 or 4 disease (p = 0.010). Multiple linear regression analysis showed CD25 expression to be independently correlated with sIL-2Rα levels. High sIL-2Rα is an important risk factor for survival in DLBCL treated with not only CHOP-like, but also R-CHOP-like regimens, regardless of the tumor's expression of CD25.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Subunidad alfa del Receptor de Interleucina-2/metabolismo , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/metabolismo , Adulto , Anciano , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Femenino , Humanos , Inmunofenotipificación , Subunidad alfa del Receptor de Interleucina-2/sangre , Linfoma de Células B Grandes Difuso/mortalidad , Linfoma de Células B Grandes Difuso/patología , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Prednisona/uso terapéutico , Pronóstico , Estudios Retrospectivos , Rituximab , Resultado del Tratamiento , Vincristina/uso terapéutico
15.
PLoS One ; 8(11): e81722, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24312342

RESUMEN

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy. Plasmacytoid DCs (pDCs), which are defined as lineage marker (Lin)(-)HLA-DR(+)CD56(-)CD123(+)CD11c(-) cells, are considered to be the normal counterpart of BPDCNs. However, BPDCN can be distinguished from pDCs by uniform expression of CD56. In this study, to identify a normal counterpart of BPDCN, we searched for a Lin(-)HLA-DR(+)CD56(+) population and focused on a minor subpopulation of Lin(-)DR(+)CD56(+)CD123(+)CD11c(-) cells that we designated as pDC-like cells (pDLCs). pDLC constituted 0.03% of peripheral blood mononuclear cells (PBMCs), and the pDLC/pDC ratio was higher in bone marrow cells than in PBMCs. pDLC clearly expressed BDCA2, BDCA4, and myeloid antigens, which are frequently expressed by BPDCN. pDLCs exhibited modest expression of Toll-like receptors and produced less interferon-α after CpG stimulation, but presented very low endocytic ability unlike mDCs. These functional differences were attributed to the expression profile of transcriptional factors. After in vitro culture with Flt3-ligand and GM-CSF, pDLCs expressed CD11c and BDCA1. These data suggested that pDLCs are a distinct subpopulation, with an immunophenotype similar to BPDCNs. Moreover, our results indicate that pDLCs might be immature DCs and might contribute to the immunophenotypical diversity of BPDCNs.


Asunto(s)
Antígeno CD56/metabolismo , Células Dendríticas/citología , Células Dendríticas/metabolismo , Neoplasias Hematológicas/inmunología , Diferenciación Celular/efectos de los fármacos , Linaje de la Célula/efectos de los fármacos , Citocinas/biosíntesis , Células Dendríticas/efectos de los fármacos , Células Dendríticas/inmunología , Regulación de la Expresión Génica/efectos de los fármacos , Factor Estimulante de Colonias de Granulocitos y Macrófagos/farmacología , Antígenos HLA-DR/metabolismo , Humanos , Proteínas de la Membrana/farmacología , Fenotipo , Receptores Toll-Like/metabolismo , Factores de Transcripción/metabolismo
16.
Leuk Res ; 37(12): 1662-7, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24094886

RESUMEN

DNA methyltransferase (DNMT) 3B7 is the most expressed DNMT3B splice variant. It was reported that the loss of DNMT3B function led to overexpression of the MEthylated in Normal Thymocyes (MENT) and accelerated mouse lymphomagenesis. We investigated the DNMT3B7 expression and its relationship to MENT expression and promoter methylation in human lymphomas. DNMT3B7 and MENT expression were significantly (p<0.0001, p<0.01) higher in lymphomas than in non-malignant. Expression of DNMT3B7 and MENT were associated with MENT promoter hypomethylation. DNMT3B7 overexpression might interfere with the normal DNA methylation mechanism required for silencing the MENT proto-oncogene, and may accelerate human lymphomagenesis.


Asunto(s)
ADN (Citosina-5-)-Metiltransferasas/genética , Metilación de ADN/fisiología , Linfoma/genética , Proteínas de Neoplasias/genética , Regiones Promotoras Genéticas , Proto-Oncogenes/genética , Empalme Alternativo/genética , ADN (Citosina-5-)-Metiltransferasa 1 , Regulación Enzimológica de la Expresión Génica/fisiología , Regulación Neoplásica de la Expresión Génica , Humanos , Isoenzimas/genética , Leucocitos Mononucleares/metabolismo , Leucocitos Mononucleares/patología , Linfoma/patología
17.
Int J Hematol ; 98(1): 122-8, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23666666

RESUMEN

Reported is a rare case IgG4-related disease that developed 10 years after combination chemotherapy for non-Hodgkin lymphoma. A 59-year-old Japanese man with longstanding bronchial asthma was referred to our hospital for bilateral hilar lymph node enlargement. The initial diagnosis was diffuse large B cell lymphoma (DLBCL) by supraclavicular lymph node biopsy. Serum IgG was high (4550 mg/dL) at diagnosis. The patient achieved complete response following six cycles of combination chemotherapy. Ten years later, bilateral submaxillary gland swelling was observed. Serum IgG and IgG4 were 2909 and 1470 mg/dL, respectively. The patient was diagnosed with IgG4-related disease by submandibular lymph node biopsy. Due to the difficulty in distinguishing IgG4-related disease from DLBCL through imaging findings alone, pathological confirmation of such lesions by biopsy is mandatory before proceeding to treatment.


Asunto(s)
Asma/complicaciones , Hipergammaglobulinemia/diagnóstico , Inmunoglobulina G/análisis , Linfoma de Células B Grandes Difuso/complicaciones , Asma/tratamiento farmacológico , Diagnóstico Diferencial , Humanos , Hipergammaglobulinemia/sangre , Hipergammaglobulinemia/complicaciones , Hipergammaglobulinemia/inmunología , Inmunoglobulina G/biosíntesis , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Masculino , Persona de Mediana Edad
18.
Rinsho Ketsueki ; 54(2): 214-8, 2013 Feb.
Artículo en Japonés | MEDLINE | ID: mdl-23470830

RESUMEN

A 45-year-old woman with acute myelogenous leukemia developed platelet transfusion refractoriness (PTR) after the engraftment of an allogeneic peripheral blood stem cell transplantation (PBSCT) from her multiparous sister, which was attributed to HLA antibodies that could not be detected in the patient's serum before transplantation. She achieved neutrophil engraftment by day 18 and megakaryocytopoiesis and complete donor chimerism was confirmed in the bone marrow on day 21. IgG-class HLA antibodies were detected in her serum on day 24 after PBSCT; however, on day 15, no HLA antibodies were detected. The specificity of the antibodies that emerged in the patient closely resembled that of the antibodies found in the donor. The donor had probably been immunized during pregnancy by their partner's HLA-antigens expressed by the fetus. Consequently, transplanted donor-derived cells provoked HLA antibodies in the recipient early after PBSCT, and those HLA antibodies induced PTR. The presence of HLA antibodies should be examined at least in pregnant female donors whose recipients developed PTR attributable to HLA antibodies after SCT.


Asunto(s)
Antígenos HLA/inmunología , Trasplante de Células Madre Hematopoyéticas , Isoanticuerpos/inmunología , Leucemia Mieloide Aguda/terapia , Transfusión de Plaquetas , Trombocitopenia/etiología , Femenino , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Leucemia Mieloide Aguda/inmunología , Persona de Mediana Edad , Transfusión de Plaquetas/métodos , Hermanos , Trombocitopenia/inmunología , Donantes de Tejidos , Trasplante Homólogo
19.
Clin J Gastroenterol ; 6(2): 150-5, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26181453

RESUMEN

Primary hepatic mucosa-associated lymphoid tissue (MALT) lymphoma is extremely rare, and the etiology of disease is not fully understood. We present herein the case of a primary hepatic MALT lymphoma with Helicobacter pylori and hepatitis C virus infection. A 71-year-old male was admitted to our institution to undergo a precise evaluation of a hepatic tumor incidentally detected during a computed tomography (CT) scan for chest examination. Dynamic CT showed faint enhancement during the arterial phase. The gadoxetate disodium-enhanced magnetic resonance imaging showed a hyper-intensity on the arterial phase and low intensity during the late and hepatocyte phases. Liver biopsy specimen showed small to intermediate size atypical lymphocytes with positive CD20 immunohistochemical staining. It was finally diagnosed as primary hepatic MALT lymphoma. FDG-PET/CT showed faintly increased uptake with a maximum standardized uptake value of 4.6, and did not show other pathological uptake. We present the rare case of primary hepatic MALT lymphoma and discussed the etiology of this disease.

20.
J Clin Exp Hematop ; 52(3): 205-9, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-23269081

RESUMEN

We report a rare case of age-related Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disorder (aEBVBLPD) primarily involving the orbit and maxillary sinus. Lesions in the left orbit and maxillary sinus were observed in a 59-year-old man presenting with pain in the left orbit and maxilla. Owing to the presence of Reed-Sternberg-like cells, the initial diagnosis was nodular sclerosis-type Hodgkin's lymphoma. Clinical stage was IIAE, and response to chemotherapy and radiotherapy was favorable. Further immunohistochemical and in situ hybridization analyses of the Reed-Sternberg-like giant cells revealed CD30, CD15, CD20, Bob-1, Oct-2, EBV-encoded RNAs (EBERs) and latent membrane protein-1 (LMP-1) expression. The characteristics of the present case, which included immunohistochemical findings, sites of primary lesions, absence of other lymph node lesions and relatively old age, suggested aEBVBLPD. Owing to the similarity in morphology, higher frequency at extranodal sites and poor prognosis, aEBVBLPD represents a differential diagnostic issue from classical Hodgkin's lymphoma when Reed-Sternberg cells are positive for EBV.


Asunto(s)
Infecciones por Virus de Epstein-Barr/diagnóstico , Herpesvirus Humano 4 , Enfermedad de Hodgkin/diagnóstico , Linfoma no Hodgkin/diagnóstico , Seno Maxilar/patología , Proteínas de Neoplasias/inmunología , Órbita/patología , Diagnóstico Diferencial , Infecciones por Virus de Epstein-Barr/inmunología , Infecciones por Virus de Epstein-Barr/patología , Expresión Génica , Enfermedad de Hodgkin/inmunología , Enfermedad de Hodgkin/patología , Humanos , Inmunohistoquímica , Linfoma no Hodgkin/inmunología , Linfoma no Hodgkin/patología , Masculino , Seno Maxilar/inmunología , Persona de Mediana Edad , Proteínas de Neoplasias/genética , Estadificación de Neoplasias , Órbita/inmunología , Células de Reed-Sternberg/inmunología , Células de Reed-Sternberg/patología
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