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1.
Br J Haematol ; 169(6): 879-86, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25807974

RESUMO

Immunosuppressive therapy has been employed as the initial treatment for acquired chronic pure red cell aplasia (PRCA), such as idiopathic, thymoma-associated, or large granular lymphocyte (LGL) leukaemia-associated PRCA, which is thought to be immune-mediated. To explore the overall long-term outcome following immunosuppression and to identify the risk factors for death in these disorders, we conducted nationwide surveys in Japan 2004 and 2006, and identified a total of 185 patients with acquired chronic PRCA, including 72 idiopathic, 41 thymoma-associated and 14 LGL leukaemia-associated cases of PRCA for whom data was available. The present study evaluated 127 patients with these three subsets of PRCA. The median overall survival has not yet been reached in idiopathic PRCA. The estimated median overall survival times in patients with thymoma-associated and LGL leukaemia-associated PRCA were 142·1 and 147·8 months, respectively. Twenty-two deaths were reported, and the response to induction therapy and relapse of anaemia were found to be associated with death. The major causes of death were infection in seven patients and organ failure in another seven patients. The results suggest that maintenance therapy and the management of infectious complications are crucial for improving the prognosis of chronic PRCA.


Assuntos
Imunossupressores/uso terapêutico , Aplasia Pura de Série Vermelha/tratamento farmacológico , Aplasia Pura de Série Vermelha/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Doença Crônica , Estudos de Coortes , Comorbidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Aplasia Pura de Série Vermelha/epidemiologia , Aplasia Pura de Série Vermelha/mortalidade , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
2.
Br J Haematol ; 166(5): 720-8, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24862795

RESUMO

Myelodysplastic syndromes (MDS) are heterogeneous clonal disorders characterized by cytopenias that arise due to ineffective haematopoiesis and morphological dysplasia and carry an increased risk of incident acute myeloid leukaemia. The pathogenesis of marrow dysfunction in MDS is multifactorial and consistent with a multistep model and may lead to heterogeneity of MDS. We investigated the proteome profile of circulating neutrophils purified from patients with refractory cytopenia with multilineage dysplasia (RCMD) to identify proteins that have a role in the pathogenesis. Using 2-dimensional difference gel electrophoresis and protein identification by matrix-assisted laser desorption ionization time-of-flight mass spectrometry, we found that peroxiredoxin 2 (PRDX2), a member of the peroxiredoxin family that regulates reactive oxygen species, was markedly upregulated in neutrophils of RCMD patients compared to healthy donors. Increased PRDX2 expression in the neutrophils of RCMD patients was confirmed using quantitative reverse transcription polymerase chain reaction, immunoblotting and immunocytochemical analysis. In addition, white blood cell and neutrophil counts in RCMD patients correlated inversely with the PRDX2 expression of. Oxidative stress is a known factor involved in the pathogenesis of MDS, and PRDX2 is associated with tumourigenesis of several solid tumours. Accordingly, our results suggest that PRDX2 may perform an important function in the pathogeneis of RCMD.


Assuntos
Anemia Refratária/sangue , Neutrófilos/metabolismo , Peroxirredoxinas/biossíntese , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Refratária/genética , Anemia Refratária/patologia , Linhagem da Célula , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neutrófilos/patologia , Peroxirredoxinas/genética , Prognóstico , Proteômica
3.
Acta Haematol ; 127(3): 170-2, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22301942

RESUMO

A 69-year-old woman with essential thrombocythemia (ET) developed giant ecchymosis, and she was admitted to hospital. Marked anemia (Hb 8.1 g/dl) accompanied by a prolonged activated partial thromboplastin time (89.6 s) was observed, and she received red blood cells (RBC) and fresh frozen plasma (FFP). On day 2 after admission, consciousness disturbance suddenly occurred, whereas computed tomography of the brain showed no evidence of bleeding. As the ecchymosis progressed, she developed shock. Although RBC and FFP transfusions were administered, she developed multi-organ failure and died 48 h after admission. Low factor VIII activity (<1%) accompanied by factor VIII inhibitor (17 Bethesda units) was found after her death. An autopsy revealed cerebral infarction without cerebral herniation. To date, acquired hemophilia A accompanying ET has been described in only one other patient. Although acquired factor VIII inhibitor is a rare disease, it should be tested for in ET patients with marked hemorrhagic tendency.


Assuntos
Hemofilia A/complicações , Hemofilia A/diagnóstico , Trombocitemia Essencial/complicações , Trombocitemia Essencial/diagnóstico , Idoso , Infarto Cerebral/patologia , Equimose/etiologia , Fator VIII/antagonistas & inibidores , Feminino , Humanos , Tomografia Computadorizada por Raios X
4.
Rinsho Ketsueki ; 53(12): 1991-6, 2012 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-23318964

RESUMO

Intracranial involvement of relapsed Hodgkin lymphoma (HL) is quite rare and its prognosis is very poor. We report a patient with relapsed HL with central nervous system (CNS) involvement after autologous stem cell transplantation successfully treated with allogeneic bone marrow transplantation with reduced intensity conditioning regimen. A standard therapy for relapsed CNS HL has not yet established. To the best of our knowledge, this is the first case report describing allogeneic stem cell transplantation for relapsed CNS HL in an elderly patient. Our results in this case suggest that allogeneic stem cell transplantation could be a useful therapeutic option in relapsed CNS HL patients, if their CNS lesions are controlled before stem cell transplantation.


Assuntos
Sistema Nervoso Central/patologia , Doença de Hodgkin/patologia , Prevenção Secundária , Transplante de Medula Óssea/métodos , Feminino , Doença de Hodgkin/terapia , Humanos , Pessoa de Meia-Idade , Invasividade Neoplásica , Fatores de Tempo , Transplante Homólogo/métodos , Resultado do Tratamento
5.
Rinsho Ketsueki ; 53(12): 2013-7, 2012 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-23318968

RESUMO

Many patients with POEMS syndrome have osteosclerotic plasmacytoma. Radiation therapy is useful for patients who have localized lesions, although chemotherapy is necessary for patients who have widespread lesions. Thus, evaluation of these lesions is important to determine the therapeutic strategy. We evaluated the activities of lesions in two patients with POEMS syndrome by (18)F-FDG positron emission tomography (PET)/computed tomography (CT) scan. In the first patient, PET/CT scan revealed osteosclerotic lesions, which were not detected by Ga-scintigraphy or plain X-ray. It also detected residual disease activity and relapse. In the second patient, lymph node involvement was suggested by (18)F-FDG uptake, and plasmacytoma was confirmed by subsequent biopsy. In the extramedullary lesions of this case, FDG uptake was as marked as in myeloma, whereas bone lesion was only detectable by CT scan. In POEMS syndrome, the PET and CT are complementary, and the combined PET/CT scan is considered to be very useful for evaluation of involved lesions.


Assuntos
Fluordesoxiglucose F18 , Imagem Multimodal , Síndrome POEMS/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Tomografia Computadorizada por Raios X , Adulto , Biópsia/métodos , Humanos , Masculino , Síndrome POEMS/complicações , Síndrome POEMS/patologia , Plasmocitoma/etiologia , Adulto Jovem
6.
Mycopathologia ; 172(3): 227-32, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21472389

RESUMO

Rituximab-related late-onset neutropenia (R-LON) is an adverse event associated with rituximab. A 65-year-old woman presented with diffuse large B-cell lymphoma of the kidney without bone marrow involvement. She was treated with 4 cycles of CHOP chemotherapy consisting of doxorubicin, cyclophosphamide, vincristine, and prednisolone at 4-week intervals. Rituximab was also administrated of the second, third, fourth CHOP cycles. She developed a high fever of 38°C, nausea, and severe neutropenia following the four cycles of R-CHOP chemotherapy. Her leukocyte count was 160/µl without neutrophils. Initially, a blood and pleural fluid and cerebrospinal fluid cultures were positive for Cryptococcus neoformans. Once she became asymptomatic following treatment with fluconazole and neutropenia was recovered with lenograstim, she had neck stiffness and admitted soon. Cerebro-spinal fluid (CSF) culture was positive for Cryptococcus neoformans. Treatment with amphotericin B(AMPH-B) and flucytosine(5-FC) was initiated as diagnosis of cryptococcus meningitis. Lenograstim was administrated for 9 months, and amount of dose was 9,750 µg. Cryptococcosis with malignant lymphoma is rare disease, and previously 17 cases were reported. Of note, mortality of disseminated cryptococcosis with malignant lymphoma is 54%. The more and more rituximab is widely used; the cases of severe infection in R-LON may increase.


Assuntos
Anticorpos Monoclonais Murinos/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Criptococose/diagnóstico , Cryptococcus neoformans/isolamento & purificação , Fatores Imunológicos/efeitos adversos , Linfoma não Hodgkin/complicações , Neutropenia/induzido quimicamente , Idoso , Anfotericina B/administração & dosagem , Anticorpos Monoclonais Murinos/administração & dosagem , Antifúngicos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Criptococose/tratamento farmacológico , Ciclofosfamida/administração & dosagem , Ciclofosfamida/efeitos adversos , Doxorrubicina/administração & dosagem , Doxorrubicina/efeitos adversos , Feminino , Fluconazol/administração & dosagem , Flucitosina/administração & dosagem , Humanos , Fatores Imunológicos/administração & dosagem , Linfoma não Hodgkin/tratamento farmacológico , Prednisolona/administração & dosagem , Prednisolona/efeitos adversos , Rituximab , Resultado do Tratamento , Vincristina/administração & dosagem , Vincristina/efeitos adversos
7.
Rinsho Ketsueki ; 52(7): 563-9, 2011 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-21821991

RESUMO

A 70-year-old man was admitted to our hospital with fever, generalized lymphadenopathy and hypoxia in October 2009. Blood examination demonstrated leukocytosis, anemia, thrombocytopenia and hyper γ-globulinemia. Peripheral blood and bone marrow smear showed marked plasma cell proliferation mimicking plasma cell leukemia. However, flow cytometric analysis showed that plasma cells were of polyclonal origin and M-protein was not detected by immunofixation of serum protein. Elevations of soluble interleukin 2 receptor and serum IL-6 were observed. A heavy Epstein-Barr viral load was detected in serum by real-time PCR. Biopsy was obtained from the right inguinal lymph node. The pathological diagnosis was angioimmunoblastic T-cell lymphoma (AITL) and rearrangement of the T-cell receptor Cß1 gene was detected. The patient was treated with CHOP therapy, and all clinical manifestations, including fever, lymphadenopathy, anemia, thrombocytopenia, hyper γ-globulinemia, plasmacytosis and hypoxia, were improved. Only a few reported cases have demonstrated AITL with marked polyclonal plasmacytosis. Although pathological mechanisms of plasmacytosis in AITL patients have not been fully elucidated, it is suggested that IL-6 and IL-10 were involved in its pathogenesis in the present case.


Assuntos
Medula Óssea/patologia , Linfadenopatia Imunoblástica/sangue , Linfadenopatia Imunoblástica/patologia , Linfoma de Células T/sangue , Linfoma de Células T/patologia , Plasmócitos/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Humanos , Linfadenopatia Imunoblástica/tratamento farmacológico , Linfadenopatia Imunoblástica/etiologia , Interleucina-10 , Interleucina-6 , Leucemia Plasmocitária , Linfoma de Células T/tratamento farmacológico , Linfoma de Células T/etiologia , Masculino , Prednisolona/administração & dosagem , Resultado do Tratamento , Vincristina/administração & dosagem
8.
Rinsho Ketsueki ; 51(3): 189-95, 2010 Mar.
Artigo em Japonês | MEDLINE | ID: mdl-20379113

RESUMO

Factors that affect the response of multiple myeloma patients to thalidomide were evaluated in 40 patients who were not eligible for chemotherapy (untreated: 14, relapse/refractory: 26). The complete response (CR) rate was 2.5%; partial response (PR) 50.0%; minimal response (MR) 25.0%; no change (NC) 12.5%; and progressive disease 10.0%. The response to thalidomide could be evaluated after four weeks of treatment. Significantly higher responses were associated with untreated patients, patients with combined use of thalidomide plus dexamethasone, and patients with kappa light chain. Patients who responded well to thalidomide showed a significantly higher progression-free survival (PFS) rate. In patients with kappa light chain, PFS and overall survival rates were significantly higher than those with lambda light chain. Frequent adverse reactions were numbness (47.5%), constipation (32.5%), and eruption (30.0%). In patients previously treated with vincristine, numbness occurred in a significantly higher percentage of patients.


Assuntos
Mieloma Múltiplo/tratamento farmacológico , Talidomida/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Constipação Intestinal/induzido quimicamente , Dexametasona/administração & dosagem , Intervalo Livre de Doença , Toxidermias/etiologia , Quimioterapia Combinada , Feminino , Humanos , Hipestesia/induzido quimicamente , Cadeias kappa de Imunoglobulina , Cadeias lambda de Imunoglobulina , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/mortalidade , Talidomida/efeitos adversos , Resultado do Tratamento
9.
Haematologica ; 93(10): 1555-9, 2008 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-18641028

RESUMO

Large granular lymphocyte leukemia-associated pure red cell aplasia accounts for a significant portion of secondary pure red cell aplasia cases. However, because of its rarity, long-term responses and relapse rates after immunosuppressive therapy are largely unknown. We conducted a nationwide survey in Japan and collected 185 evaluable patients. Fourteen patients with large granular lymphocyte leukemia-associated pure red cell aplasia were evaluated. Cyclophosphamide, cyclosporine A and prednisolone produced remissions in 6/8, 1/4 and 0/2 patients respectively. Seven and 5 patients were maintained on cyclophosphamide or cyclosporine A respectively. Two patients relapsed after stopping cyclophosphamide, and 2 patients relapsed during maintenance therapy with cyclosporine A. The median relapse-free survival in the cyclophosphamide - and the cyclosporine A groups was 53 and 123 months respectively. Large granular lymphocyte leukemia-associated pure red cell aplasia showed a good response to either cyclophosphamide or cyclosporine A. Most patients continued to receive maintenance therapy and it remains uncertain whether cyclophosphamide or cyclosporine A can induce a maintenance-free hematologic response in large granular lymphocyte leukemia-associated pure red cell aplasia.


Assuntos
Imunossupressores/farmacologia , Leucemia Linfocítica Granular Grande/epidemiologia , Leucemia Linfocítica Granular Grande/imunologia , Aplasia Pura de Série Vermelha/epidemiologia , Aplasia Pura de Série Vermelha/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Comportamento Cooperativo , Feminino , Seguimentos , Humanos , Japão/epidemiologia , Leucemia Linfocítica Granular Grande/etiologia , Masculino , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/complicações , Indução de Remissão , Terapia de Salvação , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento
11.
Int J Hematol ; 87(4): 410-413, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-18365139

RESUMO

Isolated primary granulocytic sarcoma is a rare disease that presents as an extramedullary tumor of myeloid lineage cells. Most patients subsequently develop acute myelogenous leukemia (AML) within a short period, and their prognosis is poor. Herein, we report the case of a 33-year-old woman with a primary isolated granulocytic sarcoma which originated in the small intestine. After she recovered from surgery, she received intensive chemotherapy equivalent to that for AML, followed by allogeneic bone marrow transplantation from an HLA-matched, unrelated donor. Four years after the transplantation, she remains in complete remission without graft-versus-host disease or any other symptoms. This case illustrates the effectiveness of our therapeutic strategy for isolated granulocytic sarcoma, not only with surgical resection of the tumor and intensive chemotherapy equivalent to that for AML, but also with allogeneic bone marrow transplantation, performed while no sign of AML is observed.


Assuntos
Transplante de Medula Óssea , Neoplasias Intestinais/tratamento farmacológico , Intestino Delgado/patologia , Sarcoma Mieloide/tratamento farmacológico , Adulto , Antineoplásicos/uso terapêutico , Feminino , Humanos , Neoplasias Intestinais/diagnóstico por imagem , Neoplasias Intestinais/patologia , Neoplasias Intestinais/cirurgia , Intestino Delgado/cirurgia , Radiografia , Sarcoma Mieloide/diagnóstico por imagem , Sarcoma Mieloide/patologia , Sarcoma Mieloide/cirurgia
12.
Exp Hematol ; 35(1): 13-20, 2007 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-17198869

RESUMO

OBJECTIVE: Although a number of reports have documented a significantly increased incidence of HLA-DR15 in aplastic anemia (AA), the exact role of HLA-DR15 in the immune mechanisms of AA remains unclear. We herein clarify the difference between DRB1( *)1501 and DRB1( *)1502, the two DRB1 alleles that determine the presentation of HLA-DR15, in the pathophysiology of AA. MATERIALS AND METHODS: We investigated the relationships of the patients( *) HLA-DRB1 allele with both the presence of a small population of CD55(-)CD59(-) (PNH-type) blood cells and the response to antithymocyte globulin (ATG) plus cyclosporin (CsA) therapy in 140 Japanese AA patients. RESULTS: Of the 30 different DRB1 alleles, only DRB1( *)1501 (33.6% vs 12.8%, p(c) < 0.01) and DRB1( *)1502 (43.6% vs 24.4%, p(c) < 0.01) displayed significantly higher frequencies among the AA patients than among a control. AA patients possessing HLA-DR15 tended to be old, and especially, the frequency of DRB1( *)1502 in patients 40 years of age and older (52.4%) was markedly higher than that in those younger than 40 years old (16.2%, p(c) < 0.01). Only DRB1( *)1501 was significantly associated with the presence of a small population of PNH-type cells and it also showed a good response to ATG plus CsA therapy in a univariate analysis. A multivariate analysis showed only the presence of a small population of PNH-type cells to be a significant factor associated with a good response to the immunosuppressive therapy (p < 0.01). CONCLUSIONS: Although both DRB1( *)1501 and DRB1( *)1502 contribute to the development of AA, the methods of contribution differ between the two alleles.


Assuntos
Anemia Aplástica/etiologia , Anemia Aplástica/imunologia , Antígenos HLA-DR/fisiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Anemia Aplástica/terapia , Soro Antilinfocitário/administração & dosagem , Células Sanguíneas , Criança , Ciclosporina/administração & dosagem , Quimioterapia Combinada , Feminino , Antígenos HLA-DR/imunologia , Subtipos Sorológicos de HLA-DR , Cadeias HLA-DRB1 , Hemoglobinúria Paroxística/sangue , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
14.
Haematologica ; 92(8): 1021-8, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17640861

RESUMO

BACKGROUND AND OBJECTIVES: Cyclosporine A (CsA) has become one of the leading agents for the treatment of pure red cell aplasia (PRCA). However, further studies are necessary to determine the relapse-free survival (RFS) and overall survival (OS) of patients treated with this drug, the minimum duration of therapy for induction of remission, and whether or not there is need for maintenance treatment. DESIGN AND METHODS: We conducted a nationwide survey in Japan. From a total of 185 patients (with 73 primary idiopathic PRCA and 112 with secondary PRCA), we evaluated 62 patients with primary idiopathic PRCA for this report. RESULTS: The remission induction therapy for these patients included CsA (n=31), corticosteroids (CS) (n=20) or other drugs (n=11). CsA and CS produced remissions in 23 (74%) and 12 (60%) patients, respectively. The salvage treatment produced remissions in 58 patients (94%). Forty-one and 15 patients were maintained on CsA+/-CS (CsA-containing group) or CS alone (CS group), respectively. The median RFS in the CsA-containing group was 103 months, longer than that seen in the CS group (33 months) (p<0.01). Of 14 patients whose CsA was discontinued, 12 patients (86%) relapsed after a median of 3 months (range 1.5 to 40 months), while only 3 of 27 patients (11%) relapsed during CsA-containing maintenance therapy. Thus, the discontinuance of maintenance therapy was strongly correlated with relapse (p<0.001). Four patients in the CsA-containing group died; however, the OS of this group was not significantly different from that of the CS-groups (p=0.104). INTERPRETATION AND CONCLUSIONS: CsA-containing regimens sustain prolonged RFS more effectively than CS in primary idiopathic PRCA and seem to be important to prevent relapse.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Ciclosporina/uso terapêutico , Imunossupressores/uso terapêutico , Aplasia Pura de Série Vermelha/tratamento farmacológico , Adolescente , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/mortalidade , Estudos de Coortes , Ciclofosfamida/uso terapêutico , Intervalo Livre de Doença , Avaliação de Medicamentos , Feminino , Seguimentos , Inquéritos Epidemiológicos , Neoplasias Hematológicas/complicações , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/complicações , Pré-Leucemia/complicações , Recidiva , Aplasia Pura de Série Vermelha/etiologia , Aplasia Pura de Série Vermelha/mortalidade , Indução de Remissão , Terapia de Salvação , Análise de Sobrevida , Resultado do Tratamento
15.
Int J Hematol ; 86(2): 150-7, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17875530

RESUMO

Although immunosuppressive therapy using antithymocyte globulin or cyclosporine A (CSA) is effective in selected patients with low-risk myelodysplastic syndrome, the response rates reported so far are inconsistent, and the indication of immunosuppressive therapy for myelodysplastic syndrome has not been clearly defined. We treated 20 myelodysplastic syndrome patients (17 refractory anemia cases [RA], 2 RA with excess blasts, and one RA with ringed sideroblasts) with 4 mg/kg per day of CSA for 24 weeks. Among the 19 patients evaluated, 10 showed hematologic improvement; 8 patients showed an erythroid response, 6 showed a platelet response, and one showed a neutrophil response. Most patients with hematologic improvement continued CSA thereafter, and the progressive response was observed until the latest follow-up (median, 30 months). Most toxicities associated with CSA usage were manageable, and no patient had developed acute leukemia up to this point. Short duration of illness, refractory anemia with minimal dysplasia determined by bone marrow morphology, and the presence of paroxysmal nocturnal hemoglobinuria-type cells were significantly associated with the platelet response. A minority of RA patients who did not possess such predictive variables achieved an isolated erythroid response. In conclusion, CSA may be a therapeutic option for patients with RA who do not have adverse prognostic factors.


Assuntos
Plaquetas/efeitos dos fármacos , Ciclosporina/administração & dosagem , Síndromes Mielodisplásicas/tratamento farmacológico , Valor Preditivo dos Testes , Adolescente , Adulto , Idoso , Antígenos CD55/análise , Antígenos CD59/análise , Ciclosporina/toxicidade , Contagem de Eritrócitos , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/complicações , Neutrófilos/citologia , Contagem de Plaquetas , Prognóstico , Estudos Prospectivos , Medição de Risco , Resultado do Tratamento
17.
Rinsho Ketsueki ; 48(7): 581-5, 2007 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-17695309

RESUMO

A 78-year-old man and an 81-year-old woman without hemophilia suddenly developed giant ecchymoses and intramuscular hemorrhage. Prolonged activated partial thromboplastin time and low factor VIII activity were observed, and the patients were subsequently diagnosed as having acquired factor VIII inhibitors. The patients were infused with one dose of recombinant factor VIII, 50 to 100 U/kg body weight, followed by cyclophosphamide, 500 mg on day 1 and 200 mg/day on days 2 to 5; vincristine, 2 mg on day 1; and prednisolone, 100 mg/day on days 1 to 5 (recombinant factor VIII-CVP therapy). Both patients responded after one course of therapy. Disappearance of inhibitors was achieved after three to four courses of therapy without subsequent recurrence. Recombinant factor VIII-CVP therapy is safe and effective in the eradication of factor VIII inhibitors.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Transtornos da Coagulação Sanguínea/terapia , Fator VIII/antagonistas & inibidores , Transtornos Hemorrágicos/terapia , Idoso , Transtornos da Coagulação Sanguínea/etiologia , Ciclofosfamida/administração & dosagem , Esquema de Medicação , Quimioterapia Combinada , Fator VIII/administração & dosagem , Transtornos Hemorrágicos/etiologia , Humanos , Masculino , Prednisona/administração & dosagem , Vincristina/administração & dosagem
19.
Int J Hematol ; 83(2): 147-51, 2006 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-16513533

RESUMO

Eradication of Helicobacter pylori leads to platelet recovery in some patients with idiopathic thrombocytopenic purpura (ITP). Therefore, the pathogenesis of a subgroup of ITP is probably associated with H pylori infection (H pylori-related ITP). If H pylori-related ITP is a definite subgroup of ITP, specific oligoclonal T-cells might accumulate in the peripheral blood (PB). To address this issue, we performed single-strand conformation polymorphism analysis of complementarity-determining region 3 (CDR3) of the T-cell receptor beta-chain genes of PB T-cells. Fourteen ITP patients with H pylori infection and 12 ageadjusted healthy volunteers were studied. Of the 14 patients, 8 patients (responders) exhibited a platelet response after successful H pylori eradication therapy, but 6 patients (nonresponders) did not. Vbeta5.2, Vbeta15, and Vbeta19 gene usage by clonally expanded T-cells in PB obtained before H pylori eradication therapy was significantly higher in responders than in nonresponders or healthy volunteers (Vbeta5.2, P = .023; Vbeta15, P = .004; Vbeta19, P = .036). Furthermore, an abrogation of clonally expanded T-cells was observed after therapy in some responders. These findings suggest that specific T-cell clones accumulate in H pylori-related ITP and that such clones may be associated with immune-mediated destruction of platelets.


Assuntos
Infecções por Helicobacter/complicações , Helicobacter pylori , Púrpura Trombocitopênica Idiopática/imunologia , Púrpura Trombocitopênica Idiopática/microbiologia , Linfócitos T/patologia , Adulto , Idoso , Plaquetas/microbiologia , Plaquetas/patologia , Estudos de Casos e Controles , Células Clonais/imunologia , Células Clonais/patologia , Regiões Determinantes de Complementaridade/genética , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polimorfismo Conformacional de Fita Simples
20.
Int J Hematol ; 81(2): 162-8, 2005 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-15765787

RESUMO

A retrospective study was performed to determine the prevalence of Helicobacter pylori (H pylori) infection, the effect of H pylori eradication on platelet counts, and the characteristic clinical features of chronic immune or idiopathic thrombocytopenic purpura (ITP) with H pylori infection. H pylori infection was found in 300 patients, a group that was significantly older (P < .005) and had more cases of hyperplastic megakaryocytes in the bone marrow (P = .01) than patients without H pylori infection. H pylori eradication therapy was performed in 207 H pylori-positive ITP cases, and the platelet count response was observed in 63% of the successful eradication group and in 33% of the unsuccessful eradication group (P < .005). In the successful group, the complete remission and partial remission rates were 23% and 42%, respectively, 12 months after eradication. In the majority of responders, the platelet count response occurred 1 month after eradication therapy, and the increased platelet count continued without ITP treatment for more than 12 months. H pylori eradication therapy was effective even in refractory cases, which were unresponsive to splenectomy. In conclusion, H pylori infection was involved in most ITP patients older than 40 years in Japan, and eradication therapy should be the first line of treatment in H pylori-positive ITP patients.


Assuntos
Antibacterianos/uso terapêutico , Infecções por Helicobacter/tratamento farmacológico , Helicobacter pylori , Púrpura Trombocitopênica Idiopática/microbiologia , Adulto , Distribuição por Idade , Feminino , Seguimentos , Infecções por Helicobacter/complicações , Infecções por Helicobacter/epidemiologia , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Prevalência , Púrpura Trombocitopênica Idiopática/etiologia , Indução de Remissão , Estudos Retrospectivos
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