RESUMO
BACKGROUND/AIMS: A high expression of Wilms tumor 1 (WT1) mRNA occurs in most cases of acute leukemia and myelodysplastic syndrome (MDS). Although there are many reports suggesting that acute myeloid leukemia patients with high expression levels of WT1 mRNA have a relatively poor long-term survival, there are few reports addressing the relationship between WT1 levels and prognosis in MDS. METHODS: We retrospectively analyzed 42 elderly patients with MDS whose WT1 levels at diagnosis were available, and we assessed the relationships between WT1 levels in peripheral blood and preexisting prognostic factors such as World Health Organization prognostic scores and Revised International Prognostic Scoring System risk categories, bone marrow blast percentages, and chromosomal abnormalities linked to a poor prognosis. We also evaluated the relationship between WT1 levels and prognosis. RESULTS: WT1 levels were significantly different between high- and low-risk MDS patients (p < 0.05). There was a trend towards a significant difference between those with and those without poor prognostic chromosomal rearrangements (p = 0.051). Moreover, the overall survival and progression-free survival were significantly worse in elderly patients with higher levels of WT1 (p = 0.00039 and p = 0.00077, respectively). CONCLUSIONS: The WT1 mRNA expression level at diagnosis may be a significant independent prognostic marker for elderly patients with MDS.
Assuntos
Células da Medula Óssea/metabolismo , Síndromes Mielodisplásicas/diagnóstico , RNA Mensageiro/genética , Proteínas WT1/genética , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Antimetabólitos Antineoplásicos/uso terapêutico , Azacitidina/uso terapêutico , Biomarcadores/metabolismo , Células da Medula Óssea/patologia , Aberrações Cromossômicas , Feminino , Expressão Gênica , Transplante de Células-Tronco Hematopoéticas , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/mortalidade , Síndromes Mielodisplásicas/terapia , Prognóstico , RNA Mensageiro/metabolismo , Projetos de Pesquisa , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Proteínas WT1/metabolismoRESUMO
We report the cases of 3 patients with hematological malignancies and complex karyotypes involving der(5; 17) (p10;q10), which results in the loss of 5q and 17p. Although deletions of 5q and 17p are recurrent abnormalities in hematological disease, only about 20 cases harboring der(5; 17) (p10;q10) have been reported. We address the tumorigenesis and morphological characteristics of hematological malignancies involving der(5; 17)(p10;q10), along with a review of the literature.
Assuntos
Cromossomos Humanos Par 17/ultraestrutura , Cromossomos Humanos Par 5/ultraestrutura , Neoplasias Hematológicas/genética , Translocação Genética , Idoso , Idoso de 80 Anos ou mais , Anemia Refratária com Excesso de Blastos/tratamento farmacológico , Anemia Refratária com Excesso de Blastos/genética , Anemia Refratária com Excesso de Blastos/patologia , Aneuploidia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Células da Medula Óssea/ultraestrutura , Transformação Celular Neoplásica/genética , Aberrações Cromossômicas , Bandeamento Cromossômico , Contraindicações , Evolução Fatal , Feminino , Neoplasias Hematológicas/patologia , Humanos , Cariótipo , Lenalidomida , Leucemia Mielomonocítica Crônica/genética , Leucemia Mielomonocítica Crônica/patologia , Linfoma de Células T Periférico/tratamento farmacológico , Masculino , Megacariócitos/ultraestrutura , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/patologia , Segunda Neoplasia Primária/genética , Segunda Neoplasia Primária/patologia , Recidiva , Indução de Remissão , Talidomida/análogos & derivadosRESUMO
Here we report a case of a 59-year-old man who developed neutropenic enterocolitis(NE)after autologous peripheral blood stem cell transplantation for non-Hodgkin's lymphoma in his second complete remission.Four days after transplantation, the patient suffered from diarrhea, abdominal pain, fever, and paralytic ileus.Abdominal computerized tomography scan revealed bowel wall thickening consistent with NE.Owing to his poor performance status, only medical management, including antibiotics and bowel rest, was administered, and the patient died 18 days after transplantation.Although NE after autologous peripheral blood stem cell transplantation is a relatively rare complication, it is important to be aware that this condition can occur as one of the early complications in stem cell transplantation.
Assuntos
Enterocolite Neutropênica/etiologia , Linfoma não Hodgkin/terapia , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Enterocolite Neutropênica/diagnóstico por imagem , Enterocolite Neutropênica/terapia , Evolução Fatal , Humanos , Masculino , Pessoa de Meia-Idade , Cintilografia , Tomografia Computadorizada por Raios X , Transplante Autólogo/efeitos adversosRESUMO
A 7 1-year-old man was admitted to our hospital with leukocytosis and anemia. Chronic myelomonocytic leukemia (CMML)harboring del(20q)was diagnosed by peripheral blood examination and bone marrow aspiration. The patient was subsequently treated with azacitidine, which resulted in rapid disappearance of monocytosis and resolved his dependency on red cell transfusion. With regard to the chromosomal abnormality, although del(20q)is estimated to be encountered in approximately 0.7-1.0% of all CMML cases, its significance in prognosis has not been fully analyzed. Hence, more such cases need to be evaluated to elucidate the therapeutic outcome of CMML involving del(20q). In addition, the Wilms tumor-1(WT 1)level in the patient gradually decreased after the initiation of azacitidine therapy. This phenomenon of WT1 decrease synchronizing with the patient's clinical improvement might reflect therapeutic efficacy with regard to the clinical course, as had been observed in acute myeloid leukemia and myelodysplastic syndrome.
Assuntos
Antimetabólitos Antineoplásicos/uso terapêutico , Azacitidina/uso terapêutico , Deleção Cromossômica , Cromossomos Humanos Par 20 , Leucemia Mielomonocítica Crônica/tratamento farmacológico , Idoso , Humanos , Leucemia Mielomonocítica Crônica/genética , Masculino , Resultado do TratamentoRESUMO
Eltrombopag, an oral thrombopoietin receptor agonist, is a novel drug that can be used in cases with previously-treated primary immune thrombocytopenia (ITP). In this study, we retrospectively analyzed 22 Japanese ITP patients treated in four hospitals. A responder was defined as a patient achieving a platelet count between 50,000/µl and 400,000/µl, at 75% or more of on-treatment assessments. Excluding 2 patients whose treatments were interrupted at their request, 13 of 20 patients (65%) were responders. Ten of the 13 responders had been taking more than 5 mg of a steroid preparation in the form of prednisolone or its equivalent. In 7 of these patients, the steroid dose could be tapered to 5 mg or less. Disappearance or amelioration of hemorrhagic symptoms was observed in 11 of 19 patients who had these symptoms prior to treatment (9 of 10 responders, 2 of 7 non-responders), and the improvement rate was greater in responders (p=0.018). No factors were identified as being related to efficacy. Reported adverse effects were fever (1), malaise (3), headache (2), and muscle pain (1). One severe adverse event, cerebral thromboembolism, was reported in 1 patient. Although eltrombopag is a useful therapeutic agent for refractory ITP, it is necessary to evaluate its position in the overall treatment strategy for ITP after assessing long-term complications as well as therapeutic effects.
Assuntos
Benzoatos/uso terapêutico , Hidrazinas/uso terapêutico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Pirazóis/uso terapêutico , Administração Oral , Adulto , Idoso , Idoso de 80 Anos ou mais , Benzoatos/administração & dosagem , Benzoatos/efeitos adversos , Benzoatos/farmacologia , Feminino , Humanos , Hidrazinas/administração & dosagem , Hidrazinas/efeitos adversos , Hidrazinas/farmacologia , Japão , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/sangue , Pirazóis/administração & dosagem , Pirazóis/efeitos adversos , Pirazóis/farmacologia , Receptores de Trombopoetina/agonistas , Estudos Retrospectivos , Resultado do TratamentoRESUMO
A 62-year-old man with chronic hepatitis C underwent interferon (IFN)-ß therapy. After treatment for a period comprising 29 months and 2 weeks, hematological results showed a decrease in white blood cell, hemoglobin, and platelet counts (WBC 2,300/µl, Hb 7.2 g/dl, PLT 4.7×10(4)/µl), and IFN therapy was stopped. Despite therapy discontinuation, the pancytopenia continued to progress with elevation of LDH (LDH 4,898 IU/l), and the patient was admitted to our hospital with suspected hematological disease. The patient underwent clinical screening, and pernicious anemia caused by vitamin B12 deficiency was diagnosed. The anemia rapidly improved with vitamin B12 treatment. Interferon is the mainstay of treatment for patients with viral hepatitis. While the adverse effects of interferon therapy are widely recognized, only a few reports have documented pernicious anemia developing during IFN-therapy. We recommend that particular attention be paid to such clinical and laboratory conditions as megaloblastic anemia when administering IFN. We also recommend checking the vitamin B12 level, as a deficiency of this vitamin may lead to the development of megaloblastic anemia.
Assuntos
Anemia Perniciosa/induzido quimicamente , Hepatite C Crônica/tratamento farmacológico , Interferon beta/efeitos adversos , Anemia Perniciosa/diagnóstico , Anemia Perniciosa/tratamento farmacológico , Diagnóstico Diferencial , Humanos , Interferon beta/uso terapêutico , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Vitamina B 12/administração & dosagemRESUMO
A 73-year-old male was admitted in January 1999 with hyperimmunoglobulinemia with a serum IgG level of 6530 mg/dl, bilateral eyelid tumors, bilateral submandibular swelling, and swelling of the superficial lymph nodes. A left submandibular gland biopsy showed severe chronic sialoadenitis with fibrosis. A left cervical lymph node biopsy showed invasion by many mature lymphocytes and plasma cells, but no lymphoma cells. The patient was diagnosed as having so-called Mikulicz disease associated with a disease similar to multicentric Castleman disease (MCD) characterized by multicentric lymphadenopathy and polyclonal hyperimmunoglobulinemia. Steroid therapy (prednisolone, 20 mg/day) was effective in reducing the symptoms, the bilateral eyelid tumors and the swelling of the bilateral submandibular glands and superficial lymph nodes. The lack of any increase of serum IL-6 suggested that this case had a hitherto unknown etiology, other than MCD.
Assuntos
Hipergamaglobulinemia/etiologia , Imunoglobulina G/sangue , Doenças Linfáticas/etiologia , Doença de Mikulicz/complicações , Doença de Mikulicz/diagnóstico , Idoso , Hiperplasia do Linfonodo Gigante , Diagnóstico Diferencial , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Interleucina-6/sangue , Doenças Linfáticas/tratamento farmacológico , Masculino , Doença de Mikulicz/tratamento farmacológico , Prednisolona/uso terapêutico , Resultado do TratamentoRESUMO
We report a patient with severe thrombotic thrombocytopenic purpura (TTP) refractory to plasmapheresis who was successfully treated with rituximab. A 57-year-old male patient was referred to our department for further differential diagnosis and treatment of anemia and severe thrombocytopenia. Progressive psychoneurotic symptoms, hemolytic anemia, thrombocytopenia, renal function insufficiency and fever led us to the diagnosis of TTP. ADAMTS13 activity was below 3% and an inhibitor for ADAMTS13 was detected. Treatment with plasmapheresis and high-dose steroid was initiated but without clinical benefit. Two weeks following the initiation of plasmapheresis, we decided to treat the patient with 7 cycles of rituximab. No severe rituximab-related adverse effects were observed. After treatment with rituximab, the disease remitted, and the ADAMTS13 activity level increased. The patient has remained in complete remission for more than 1 year. Our data suggest that rituximab may be the optimal immunosuppressive therapy for refractory thrombotic thrombocytopenic purpura caused by an anti-ADAMTS 13 inhibitor.
Assuntos
Anticorpos Monoclonais/administração & dosagem , Imunossupressores/administração & dosagem , Púrpura Trombocitopênica Trombótica/terapia , Proteínas ADAM/antagonistas & inibidores , Proteínas ADAM/deficiência , Proteína ADAMTS13 , Anticorpos Monoclonais Murinos , Humanos , Masculino , Pessoa de Meia-Idade , Plasmaferese , Púrpura Trombocitopênica Trombótica/etiologia , Indução de Remissão , Rituximab , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do TratamentoRESUMO
The etiologies of secondary idiopathic thrombocytopenic purpura (ITP) include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/µL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/µL) after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/µL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP) eradication or corticosteroid treatment, all of the patients' platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations.
RESUMO
We experienced the VNCOP-B (etoposide, mitoxantrone, cyclophosphamide, vincristine, predonisolone, bleomycin) combination regimen for the treatment of elderly patients with aggressive non-Hodgkin lymphoma (NHL) in a multicenter study by 6 collaborative institutions. Patients were previously untreated > or = 60 years of age and received prophylactic G-CSF. Twenty patients entered this trial, and all of them were evaluated for feasibility, toxicity, and efficacy. The complete remission rate was 75.0%, with a 100% overall response rate; overall survival (OS) rate at 3 years was 79.1% (median follow up 761.5 days), with a 60.7% progression-free 3-year survival (PFS) rate (median follow-up 600.0 days). Our trial was promising and well-tolerated. According to IPI, high/high-intermediate risk was associated with significantly worse OS and PFS than low/low-intermediate risk (2-year OS: 51.8% versus 100.0%, p=0.0118; 2-year PFS: 33.3% versus 80.0%, p=0.0125). Grade 3/4 infections occurred in 3 patients, but no patients experienced it with predonisolone reduced.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Bleomicina/administração & dosagem , Bleomicina/efeitos adversos , Ciclofosfamida/administração & dosagem , Ciclofosfamida/efeitos adversos , Esquema de Medicação , Etoposídeo/administração & dosagem , Etoposídeo/efeitos adversos , Estudos de Viabilidade , Feminino , Fator Estimulador de Colônias de Granulócitos/administração & dosagem , Humanos , Linfoma não Hodgkin/mortalidade , Masculino , Pessoa de Meia-Idade , Mitoxantrona/administração & dosagem , Mitoxantrona/efeitos adversos , Neutropenia/induzido quimicamente , Prednisona/administração & dosagem , Prednisona/efeitos adversos , Indução de Remissão , Taxa de Sobrevida , Resultado do Tratamento , Vincristina/administração & dosagem , Vincristina/efeitos adversosRESUMO
We describe an XXX female patient accompanied with essential thrombocythemia. To our knowledge this is the first case ever to have been reported. The patient was asymptomatic, but her platelet count had increased to 111.2 x 10(4)/microliter, and she was diagnosed as having essential thrombocythemia based on the diagnostic criteria of the Polycythemia Vera Study Group. At the same time, chromosome analysis of bone marrow cells revealed that she was an XXX female. The patient remained asymptomatic throughout the course of treatment.
Assuntos
Cromossomos Humanos X , Aberrações dos Cromossomos Sexuais , Trombocitemia Essencial/genética , Humanos , Pessoa de Meia-IdadeRESUMO
We have treated 29 patients (19 men and 10 women) with relapsed or refractory non-Hodgkin's lymphoma with a combination of cytarabine (Ara-C), carboplatin (CBDCA) and prednisolone (PSL). The regimen, on days 1 to 3, included Ara-C, 400 mg/m2 i.v.; CBDCA; 250 mg/m2 i.v.; and PSL, 40 mg/m2. Since complete response was achieved in 10 patients (34.5%) and partial response in 9 (31.0%), the total response rate was 65.5%. The 50% survival duration of all patients after the initiation of this therapy was 8 months. The overall 5-year survival rate was 66.7% for those who achieved CR or PR with the Ara-C/CBDCA regimen and received high-dose chemotherapy and autologous hematopoietic stem cell transplantation. Myelosuppression was the major toxicity. Total WBC counts under 1,000/microliter were seen in 67.7% of the courses, and thrombocytopenia under 50,000/microliter was seen in 96.8%. Ara-C/CBDCA has proven to be an effective salvage regimen for patients with relapsed or refractory lymphoma. High-dose chemotherapy and autologous hematopoietic stem cell transplantation should be considered for salvageable patients.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Terapia de Salvação , Adulto , Carboplatina/administração & dosagem , Citarabina/administração & dosagem , Esquema de Medicação , Feminino , Humanos , Linfoma não Hodgkin/patologia , Masculino , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Recidiva , Resultado do TratamentoRESUMO
A previous study of cases of myelodysplastic syndrome harboring der(5;19)(p10;q10) found that they displayed common characteristics including predominance in elderly men, dysplasia involving three hematopoietic lineages and CD7 expression in blasts. However, the whole-arm translocation der(5;19)(p10;q10) has not been fully analyzed because of its rarity. In this study we used flow cytometry to evaluate the immunophenotype of two patients' bone marrow mononuclear cells. Both patients had involved der(5;19)(p10;q10) in their karyotype analyzed by standard G-banding technique. Both patients had the CD7+ and CD41+ phenotype, and the CD41 positivity suggested that the myeloid neoplasms involving der(5;19)(p10;q10) were of megakaryoblastic origin. The der(5;19)(p10;q10) abnormality is associated with unique characteristics of the immunophenotype. We address the clinical, immunophenotypic and morphological aspects of hematological malignancy involving der(5;19)(p10;q10), along with a review of the literature.
Assuntos
Cromossomos Humanos Par 19 , Cromossomos Humanos Par 5 , Leucemia Mieloide Aguda/genética , Translocação Genética , Idoso , Análise Citogenética , Humanos , Imunofenotipagem , Leucemia Mieloide Aguda/patologia , Masculino , Pessoa de Meia-IdadeRESUMO
We report a case in which chronic myelogenous leukemia (CML) developed after postoperative adjuvant S-1 therapy for rectal cancer. A 56-year-old man was diagnosed with rectal adenocarcinoma, which was treated with abdominoperineal resection followed by a year of adjuvant S-1 therapy. At 39 postoperative months, he was diagnosed with CML. Although it remains unclear that CML that develops after treatment involving cytotoxic agents is treatment-related, clinicians should be aware of the possibility of CML developing after S-1 therapy.
RESUMO
We describe a case of post-polycythemic myelofibrosis harboring der(Y)t(Y;1)(q12;q12). The patient was a 69-year-old man and was initially diagnosed with polycythemia vera. During the clinical course of his condition, the polycythemia developed into myelofibrosis. Chromosome analysis detected der(Y)t(Y;1)(q12;q12). We discuss the association between der(Y)t(Y;1)(q11~12;q12~21) and tumorigenesis along with a review of literature.