RESUMO
Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T) is a rare disease, which presents with features of myelodysplastic syndromes with ring sideroblasts and essential thrombocythemia, as well as anemia and marked thrombocytosis. SF3B1 and JAK2 mutations are often found in patients, and are associated with their specific clinical features. This study was a retrospective analysis of 34 Japanese patients with MDS/MPN-RS-T. Median age at diagnosis was 77 (range, 51-88) years, and patients had anemia (median hemoglobin: 9.0 g/dL) and thrombocytosis (median platelet count: 642 × 109/L). Median overall survival was 70 (95% confidence interval: 68-not applicable) months during the median follow-up period of 26 (range: 0-91) months. A JAK2V617F mutation was detected in 46.2% (n = 12) of analyzed patients (n = 26), while an SF3B1 mutation was detected in 87.5% (n = 7) of analyzed patients (n = 8). Like those with myelodysplastic syndromes or myeloproliferative neoplasms, patients often received erythropoiesis-stimulating agents and aspirin to improve anemia and prevent thrombosis. This study, which was the largest to describe the real-world characteristics of Japanese patients with MDS/MPN-RS-T, showed that the patients had similar characteristics to those in western countries.
Assuntos
Anemia Sideroblástica , Síndromes Mielodisplásicas , Doenças Mieloproliferativas-Mielodisplásicas , Neoplasias , Trombocitose , Humanos , Anemia Sideroblástica/genética , Estudos Retrospectivos , População do Leste Asiático , Síndromes Mielodisplásicas/genética , Doenças Mieloproliferativas-Mielodisplásicas/genética , Trombocitose/genética , Neoplasias/complicações , Mutação , Fatores de Processamento de RNA/genéticaRESUMO
Bone-marrow minimal residual disease (MRD) causes relapse after chemotherapy in patients with acute myelogenous leukemia (AML). We postulate that the drug resistance is induced by the attachment of very late antigen (VLA)-4 on leukemic cells to fibronectin on bone-marrow stromal cells. We found that VLA-4-positive cells acquired resistance to anoikis (loss of anchorage) or drug-induced apoptosis through the phosphatidylinositol-3-kinase (PI-3K)/AKT/Bcl-2 signaling pathway, which is activated by the interaction of VLA-4 and fibronectin. This resistance was negated by VLA-4-specific antibodies. In a mouse model of MRD, we achieved a 100% survival rate by combining VLA-4-specific antibodies and cytosine arabinoside (AraC), whereas AraC alone prolonged survival only slightly. In addition, overall survival at 5 years was 100% for 10 VLA-4-negative patients and 44.4% for 15 VLA-4-positive patients. Thus, the interaction between VLA-4 on leukemic cells and fibronectin on stromal cells may be crucial in bone marrow MRD and AML prognosis.
Assuntos
Fibronectinas/metabolismo , Integrina alfa4beta1/metabolismo , Leucemia Mieloide Aguda/metabolismo , Proteínas Serina-Treonina Quinases , Animais , Anticorpos/farmacologia , Antimetabólitos Antineoplásicos/uso terapêutico , Citarabina/uso terapêutico , Resistencia a Medicamentos Antineoplásicos , Humanos , Integrina alfa4beta1/efeitos dos fármacos , Integrina alfa4beta1/imunologia , Integrina alfa5beta1/metabolismo , Leucemia/tratamento farmacológico , Leucemia/metabolismo , Leucemia/patologia , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/mortalidade , Camundongos , Camundongos SCID , Neoplasia Residual , Fosfatidilinositol 3-Quinases/metabolismo , Valor Preditivo dos Testes , Proteínas Proto-Oncogênicas/metabolismo , Proteínas Proto-Oncogênicas c-akt , Proteínas Proto-Oncogênicas c-bcl-2/metabolismo , Transdução de Sinais , Células Estromais/metabolismo , Taxa de Sobrevida , Fatores de Tempo , Células Tumorais CultivadasRESUMO
PURPOSE: Long-standing ulcerative colitis (UC) predisposes patients to the development of colorectal cancer, but surveillance of colitis-associated cancer by detecting the precancerous lesion dysplasia is often difficult because of its rare occurrence and normal-looking appearance. In sporadic colorectal cancer, aberrant crypt foci (ACF) have been reported by many investigators to be precursor lesions of the adenoma-carcinoma sequence. In the present study, we analyzed the genetic background of ACF to determine whether they could be precursors for dysplasia, and we examined the usefulness of endoscopic examination of ACF as a surrogate marker for surveillance of colitis-associated cancer. EXPERIMENTAL DESIGN: ACF were examined in 28 UC patients (19 patients with UC alone and 9 patients with UC and dysplasia; 2 of those patients with dysplasia also had cancer) using magnifying endoscopy. K-ras, APC, and p53 mutations were analyzed by two-step PCR RFLP, in vitro--synthesized protein assay, and single-strand conformation polymorphism, respectively. Methylation of p16 was analyzed by methylation-specific PCR. RESULTS: ACF that appeared distinct endoscopically and histologically were identified in 27 out of 28 UC patients. They were negative for K-ras, APC, and p53 mutations but were frequently positive for p16 methylation (8 of 11; 73%). In dysplasia, K-ras and APC mutations were negative but p53 mutation (3 of 5; 60%) and p16 methylation (3 of 5; 60%) were positive. There was a significant stepwise increase in the number of ACF from patients with UC alone to patients with dysplasia and to patients with cancer. Univariate and multivariate analyses showed significant correlations between ACF and dysplasia. CONCLUSIONS: We have disclosed an ACF-dysplasia-cancer sequence in colitis-associated carcinogenesis similar to the ACF-adenoma-carcinoma sequence in sporadic colon carcinogenesis. This study suggests the use of ACF instead of dysplasia for the surveillance of colitis cancer and warrants further evaluation of ACF as a surveillance marker in large-scale studies.
Assuntos
Colite Ulcerativa/patologia , Neoplasias Colorretais/patologia , Lesões Pré-Cancerosas/patologia , Adulto , Colite Ulcerativa/genética , Neoplasias Colorretais/genética , Progressão da Doença , Endoscopia Gastrointestinal , Feminino , Genes APC , Genes p16 , Genes p53 , Genes ras , Humanos , Masculino , Mutação , Polimorfismo de Fragmento de Restrição , Polimorfismo Conformacional de Fita Simples , Lesões Pré-Cancerosas/genética , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
A 49-year-old man was admitted with high-grade fever, night sweating and cervical lymphadenopathy in September 2005. On examination, both neutrophilia and thrombocytosis were noted in the peripheral blood, a bone marrow examination revealed marked both myeloid and megakaryocytic hyperplasia. The sera obtained at initial presentation showed an elevated levels of granulocyte-colony stimulating factor (G-CSF) and interleukin-6 (IL-6). A pathologic diagnosis of T-cell rich B-cell lymphoma was made based on an inguinal lymph node biopsy. Following treatment with CHOP accompanied by rituximab (R-CHOP), both the neutrophilia and thrombocytosis subsided after 3 courses of R-CHOP, resulting in a complete remission after 4 courses of chemotherapy. Neutrophilia, thrombocytosis and T-cell rich B-cell lymphoma in this patient were considerably ameliorated with chemotherapy. We report here a patient with T-cell rich B-cell lymphoma associated with both neutrophilia and thrombocytosis, suggesting that the lymphoma triggered both myeloid and megakaryocytic hyperplasia.
Assuntos
Leucocitose/etiologia , Linfoma de Células B/complicações , Neutrófilos , Linfócitos T , Trombocitose/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Fator Estimulador de Colônias de Granulócitos/sangue , Humanos , Hiperplasia , Interleucina-6/sangue , Contagem de Linfócitos , Linfoma de Células B/diagnóstico , Linfoma de Células B/tratamento farmacológico , Linfoma de Células B/patologia , Masculino , Megacariócitos/patologia , Pessoa de Meia-Idade , Células Mieloides/patologia , Resultado do TratamentoRESUMO
A 75-year-old man was admitted to our hospital in October, 2005 for examination of pre-diagnosed pancytopenia. His bone marrow showed myeloid dysplasia, and 30.4% of the nucleated cells were blasts. Our diagnosis was acute myelogenous leukemia with multilineage myelodysplasia (AML with MLD; WHO classification). A direct Coombs test proved positive, and the platelet-associated IgG (PA-IgG) level was elevated. After treatment with CAG (Ara-C + ACR + G-CSF), complete remission was obtained, showing negative on the direct Coombs test with PA-IgG levels returned to normal. The patient subsequently relapsed, testing positive on the direct Coombs test and experiencing a re-elevation of PA-IgG levels. We report here a first case of AML with MLD, direct Coombs test and PA-IgG assay.
Assuntos
Plaquetas/imunologia , Teste de Coombs , Imunoglobulina G/sangue , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/diagnóstico , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/diagnóstico , Aclarubicina/administração & dosagem , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Citarabina/administração & dosagem , Fator Estimulador de Colônias de Granulócitos/administração & dosagem , Humanos , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/tratamento farmacológico , Masculino , Síndromes Mielodisplásicas/sangue , Síndromes Mielodisplásicas/tratamento farmacológico , Pancitopenia/etiologia , Prednisolona/administração & dosagem , Resultado do TratamentoRESUMO
According to the National Comprehensive Cancer Network (NCCN) clinical practice guideline for gastric cancer (2006, the first edition), 5-FU/Leucovorin (LV), 5-FU-based, cisplatin (CDDP)-based, oxaliplatin (L-OHP)-based, taxane-based, and irinotecan (CPT-11)-based, ECF are recommended. We used modified FOLFOX 6 (mFOLFOX 6) for pretreatment, that is oxaliplatin-based chemotherapy, for a patient who had received 5-FU-based, CDDPbased, taxane-based, and CPT-11-based treatment for an unresectable gastric cancer case responding to mFOLFOX 6. A 73-year-old male admitted to our hospital for treatment of advanced gastric cancer was diagnosed to be inoperable. A combination chemotherapy docetaxel and CDDP and S-1 as first-line treatment, CPT-11 and CDDP as second-line treatment, weekly paclitaxel treatment as third-line treatment, and MTX and 5-FU as fourth-line treatment were performed. He had progressed after 5-FU-based, CDDP-based, taxane-based, and CPT-11-based chemotherapy. There are no effective approved drugs for gastric cancer in Japan. Oxaliplatin was reportedly effective for metastatic gastric cancer, but it is still non-approved in Japan. After receiving an explanation of oxaliplatin-based therapy, he gave informed consent. Oxaliplatin-based therapy for this patient was then evaluated and approved under an institutional review board of Higashi Sapporo Hospital. mFOLFOX 6 used for the oxaliplatin-based therapy. After 2 courses of mFOLFOX 6, he showed a partial response. Oxaliplatin-based treatment was thought to be promising for previously CDDP-treated patients with unresectable gastric cancers.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Gástricas/tratamento farmacológico , Idoso , Fluoruracila/uso terapêutico , Humanos , Leucovorina/uso terapêutico , Masculino , Compostos Organoplatínicos/uso terapêutico , Resultado do TratamentoRESUMO
Appetite loss, nausea and vomiting are most common gastrointestinal distress in patients with advanced cancers. It is worthwhile to relieve these symptoms for improving their quality of life (QOL), an inadequate treatment, on the contrary, will increase their distressing symptoms. To avoid this, it is essential to clarify the cause of illness, especially to find out whether it is due to a complete mechanical gastrointestinal obstruction or not. If it is so, the use of metoclopramide is a contraindication. Practically, the combined use of octreotide acetate, steroids and morphine will be effective to manage a severe gastrointestinal distress such as an obstinately nausea and vomiting.
Assuntos
Anorexia/etiologia , Náusea/etiologia , Neoplasias/complicações , Vômito/etiologia , Anorexia/terapia , Humanos , Náusea/tratamento farmacológico , Vômito/tratamento farmacológicoRESUMO
A 51-year-old woman with severe aplastic anemia underwent allogeneic peripheral blood stem cell transplantation using blood stem cells from an HLA-identical sibling. Adenovirus type 11 hemorrhagic cystitis developed and progressed to nephritis and hemophagocytic syndrome. Oral ribavirin was effective not only for the hemorrhagic cystitis and nephritis but also for the hemophagocytic syndrome. Since therapeutic strategies for adenovirus infection after hematopoietic stem cell transplantation have not been established, we present our case and discuss the therapeutic approach.
Assuntos
Infecções por Adenoviridae , Antivirais/administração & dosagem , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/virologia , Infecções Oportunistas , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Ribavirina/administração & dosagem , Administração Oral , Anemia Aplástica/terapia , Feminino , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Imunossupressores/efeitos adversos , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
We report a case of chronic myelocytic leukemia (CML) with cyclic oscillation of platelet and leukocyte counts and attempt to elucidate the oscillatory mechanism from the standpoint of cytokine regulation of hematopoiesis. A 57-year-old woman with a diagnosis of CML exhibited platelet and white blood cell (WBC) count fluctuations of a cyclic nature. The average duration of the cycles was about 8 weeks. The patient suffered from headache, fatigue, and malaise at the peak of the cycle. The peak thrombopoietin concentration in peripheral blood coincided with a period of decrease in platelet numbers. The change in transforming growth factor beta (TGF-beta) level paralleled that of the platelet numbers. A progenitor cell assay revealed the suppression of trilineage colony formation in the presence of plasma from the blood cell peak point, and this suppression was completely blocked when the plasma was incubated with an anti-TGF-beta antibody. From these findings, we concluded that the cyclic oscillation of the platelet, WBC, and reticulocyte counts had been induced by excess negative feedback to megakaryopoiesis by TGF-beta.
Assuntos
Células Sanguíneas/citologia , Retroalimentação Fisiológica , Leucemia Mielogênica Crônica BCR-ABL Positiva/sangue , Periodicidade , Fator de Crescimento Transformador beta/sangue , Anticorpos Monoclonais/farmacologia , Células Sanguíneas/efeitos dos fármacos , Feminino , Hematopoese/efeitos dos fármacos , Humanos , Contagem de Leucócitos , Pessoa de Meia-Idade , Contagem de Plaquetas , Contagem de Reticulócitos , Fator de Crescimento Transformador beta/imunologiaRESUMO
Patients with chronic idiopathic neutropenia (CIN) usually show chronic inflammation in their bone marrow stromal cells, but the etiologies of this disorder are still unknown. We suspected viral infection of the bone marrow stromal cells and used polymerase chain reaction (PCR) analysis to test for cytomegalovirus (CMV) infection in 4 patients with CIN and in 6 healthy volunteers. Peripheral blood mononuclear cells did not yield signals in any of the patients or healthy volunteers, but PCR analysis for CMV DNA in bone marrow stromal cells showed positive signals in 2 of the patients with CIN. CMV DNA was not detected in the other 2 patients or in the healthy volunteers. The 2 patients who were positive for stromal CMV received 500 mg/day ganciclovir for 14 days. After this treatment, the PCR band showing the presence of stromal CMV disappeared, and the neutrophil numbers of the 2 patients who received this viral eradication therapy normalized 6 months after treatment. In conclusion, the etiology in some cases of CIN is speculated to be infection by CMV in bone marrow stromal cells, and treatment with ganciclovir may be effective in treating such patients.
Assuntos
Infecções por Citomegalovirus/complicações , Ganciclovir/administração & dosagem , Neutropenia/virologia , Células Estromais/virologia , Idoso , Células da Medula Óssea/virologia , Doença Crônica , Citomegalovirus/genética , Citomegalovirus/isolamento & purificação , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/tratamento farmacológico , DNA Viral/análise , Feminino , Ganciclovir/farmacologia , Humanos , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Neutropenia/tratamento farmacológico , Neutrófilos/efeitos dos fármacos , Reação em Cadeia da PolimeraseRESUMO
A 46-year-old woman suffering from agnogenic myeloid metaplasia (AMM) since 1994 was being followed as an outpatient. In November 1999, she exhibited respiratory failure caused by massive pulmonary effusion, which was speculated to have been produced by chest tumors. A biopsy specimen revealed findings compatible with fibrous hematopoietic tumor (FHT): prominent fibroblasts and fibrosis with scantv megakaryocytes. Serum concentrations of transforming growth factor (TGF)-beta and platelet-derived growth factor (PDGF) were significantly higher than those of other cases of AMM without FHT. The effusion did not respond to administrations of various chemotherapeutic agents, but after prednisolone administration, the effusion disappeared and the tumors also diminished. TGF-beta and PDGF were the possible causes of FHT formation, and for such fibrotic extramedullary regions, the administration of prednisolone should be considered.
Assuntos
Derrame Pleural/tratamento farmacológico , Derrame Pleural/etiologia , Prednisona/uso terapêutico , Mielofibrose Primária/complicações , Anti-Inflamatórios/uso terapêutico , Citocinas/sangue , Feminino , Humanos , Pessoa de Meia-Idade , Derrame Pleural/diagnóstico por imagem , Mielofibrose Primária/tratamento farmacológico , RadiografiaRESUMO
Death by bloodletting among patients with factitious anemia has never been reported to our knowledge. We report the first known case. A 25-year-old woman with severe iron deficiency anemia confessed her habit of bloodletting at her first visit to our hospital, in March 1998. We prescribed oral iron and referred her to a psychiatrist. The diagnosis was borderline personality disorder. The psychiatrist began counseling the patient and prescribed a major tranquilizer. The patient's method of bloodletting was to insert an 18-gauge needle without syringe into her vein after inducing congestion in her arm. This method was considered to involve risk of death, because once the patient fell into a faint caused by blood loss, the bloodletting could not be stopped. Although we attempted to persuade the patient to stop bloodletting by this method, she died after self-bloodletting in September 1999. It is not known whether the death was intentional suicide or an accident.
Assuntos
Transtorno da Personalidade Borderline/psicologia , Transtornos Autoinduzidos/psicologia , Flebotomia/psicologia , Comportamento Autodestrutivo/psicologia , Adulto , Evolução Fatal , Feminino , Humanos , Flebotomia/efeitos adversosRESUMO
In addition to hypersplenism, immunological destruction of platelets by elevated platelet associated IgG (PAIgG) and platelet surface IgG (PSIgG) has been proposed as a causative factor for thrombocytopenia in chronic liver disease (CLD), although the implication of PAIgG may be debatable since recent investigations on idiopathic thrombocytopenic purpura disclosed the fact that PAIgG largely relates to the intra-platelet IgG in alpha-granules and not to PSIgG. Further, with regard to the elevated PSIgG of CLD, characterization as to whether it mainly represents anti-platelet glycoprotein (GP) antibodies or IgG contained in the immune complex has not been elucidated. Thirty-seven patients with chronic viral liver disease (CVLD); 31 hepatitis C and 6 hepatitis B were included in this study. First we monitored the changes in levels of PAIgG, alpha-granule IgG, PSIgG and mean platelet volume (MPV) during the course of partial splenic arterial embolization (PSE). The elevated level of PAIgG decreased after PSE, paralleling that of alpha-granule IgG, while PSIgG showed no change; MPV decreased reciprocally with the increase of platelet count. These results indicate that the increment of PAIgG in CVLD may be caused by accelerated destruction of platelets; this generally evokes hyperproduction of large-sized thrombocytes, which have an increased capability to uptake circulating IgG. To characterize PSIgG, we then tested CVLD patients for antiplatelet GP antibodies and found only a 5.4% positivity. It was also found that circulating immune complex levels in CVLD patients were clearly elevated, correlating with the levels of PSIgG. Thus, it was surmised that immune complexes bound to the platelet surface, and not platelet specific GP antibodies, may be playing a crucial role in platelet destruction of CVLD, possibly through phagocytosis by macrophages.
RESUMO
A 70-year-old woman was admitted for anemia, elevated serum total protein and a right axillary mass. Laboratory data showed monoclonal x IgM with a decrease in serum IgG and IgA levels. An occipital punched-out lesion was detected on a cranial X-ray. A tumor lesion was detected on chest X-ray and computed tomography. Biopsy specimen revealed plasmacytoma with cytoplasmic IgM. Bone marrow aspiration revealed an elevated plasma cell count. An immunophenotype analysis of the plasma cells showed positivity of cytoplasmic IgM, x, CD5, CD38, CD11a (LFA-1), CD44 (HCAM), CD49d (VLA-4) and CD54 (ICAM-1). From the above results, we diagnosed the patient as having IgM myeloma associated with plasmacytoma. Melphalan and prednisolone therapy were prescribed, their effect on the myeloma was short term, so we changed the chemotherapy to VAD (vincristine, adriamycin and dexamethasone), but this treatment had little effect. The patient developed bacterial pneumonia and died. IgM myeloma is a rare disease and reports of immunophenotype analysis are also rare. There is no case report of plasmacytoma associated with IgM myeloma.
Assuntos
Moléculas de Adesão Celular/biossíntese , Imunoglobulina M/sangue , Mieloma Múltiplo/metabolismo , Idoso , Feminino , Humanos , Mieloma Múltiplo/imunologia , Mieloma Múltiplo/patologia , Plasmocitoma/complicaçõesRESUMO
The patient was a 68-year-old woman who was diagnosed as having Ph-positive acute lymphoblastic leukemia (ALL). Complete remission (CR) was not obtained with the induction therapy of the Japan Adult Leukemia Study Group Protocol. We then considered administration of imatinib (ST1571). The institutional review board of our hospital approved this therapy, and we initiated the administration of imatinib 400 mg/day after obtaining written informed consent from the patient. At day 10 of the regimen, CR was achieved, treatment had to be discontinued RT-PCR showed no induction of detectable minor bcr-abl mRNA after three courses of consolidation chemotherapy combined with imatinib. We changed the administration protocol of Imatinib to two weeks out of every in four, weeks, and conducted 9 courses of consolidation chemotherapy. The negative result of RT-PCR has been maintained 10 months after diagnosis. The adverse effects were body weight gain and retaining pleural effusion, and these were controlled by the diuretics. The negative result of RT-PCR in Ph positive ALL after chemotherapy has rarely been reported, so the combination of imatinib and chemotherapy may be considered to be effective for Ph positive ALL.
Assuntos
Inibidores Enzimáticos/administração & dosagem , Cromossomo Filadélfia , Piperazinas/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Proteínas Tirosina Quinases/antagonistas & inibidores , Pirimidinas/administração & dosagem , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Benzamidas , Quimioterapia Combinada , Feminino , Proteínas de Fusão bcr-abl , Humanos , Mesilato de Imatinib , Indução de RemissãoRESUMO
A 25-year-old previously healthy pregnant woman was admitted to our hospital because of severe anemia (Hb 6.7 g/dl), and diagnosed as having mixed-type autoimmune hemolytic anemia (AIHA) due to de novo cytomegalovirus (CMV) infection. After daily administration of prednisolone, the anemia gradually resolved and the patient delivered a healthy baby. This is the first report of a healthy person suffering from mixed-type AIHA due to de novo CMV infection.
Assuntos
Anemia Hemolítica Autoimune/etiologia , Infecções por Citomegalovirus/complicações , Complicações Hematológicas na Gravidez/etiologia , Complicações Infecciosas na Gravidez , Anemia Hemolítica Autoimune/imunologia , Temperatura Baixa , Feminino , Humanos , GravidezRESUMO
A 43-year-old woman was admitted for examination of fever, an elevated transaminase level, LDH, skin eruption, sore throat and bicytopenia. As bone marrow examination revealed an increased proportion of histiocytes and active phagocytosis, hemophagocytic syndrome (HPS) was diagnosed. After admission, the peripheral blood counts recovered spontaneously and the HPS subsided, but other symptoms persisted and the neutrophil count increased. At this time, we diagnosed the patient as having adult Still's disease. All the symptoms disappeared after administration of prednisolone. The markedly increased concentrations of TNF-alpha and IFN-gamma in the peripheral blood at the time of HPS declined gradually, and the IL-6 concentration increased at the time of diagnosis of Still's disease. However, all of these concentrations normalized after administration of prednisolone. As HPS and Still's disease have a common etiology, and each shows high concentrations of IFN-gamma, IL 6 and TNF-alpha, the symptoms are similar in both diseases. In particular, a relationship between HPS and high concentrations of TNF-alpha and IFN-gamma is suspected.
Assuntos
Histiocitose de Células não Langerhans/complicações , Doença de Still de Início Tardio/complicações , Adulto , Anti-Inflamatórios/administração & dosagem , Citocinas/sangue , Feminino , Histiocitose de Células não Langerhans/tratamento farmacológico , Humanos , Prednisolona/administração & dosagemRESUMO
We report here a patient who suffered from PCR-confirmed human herpesvirus type 6 (HHV-6) encephalitis following reduced intensity stem cell transplantation (RIST) from her HLA-matched sibling donor. A 66-year-old woman with MDS-RA underwent RIST from her HLA-matched brother. Engraftment was favorable and grade 2 GVHD (skin and intestine) was observed with good response to 60 mg of prednisolone. On day 162, she developed fever, headache, diplopia, disorientation and abnormal neurological findings including cervical stiffness and nystagmus. An analysis of cerebrospinal fluid (CSF) revealed 80 cells/microl, a glucose level of 50 mg/dl and a protein level of 97 mg/dl on day 162. Although computed tomography (CT) of the brain and electroencephalography (EEG) were nonspecific, HHV-6 was detected in the CSF using polymerase chain reaction (PCR) techniques and the patient was diagnosed as having encephalitis due to local reactivation of HHV-6. Administration of ganciclovir (GCV) and acyclovir (ACV) were started from day 162. Treatment with antiviral agents was effective, with total resolution of her symptoms and the DNA of this virus disappeared from the CSF after 23 days of treatment. This case shows that HHV-6 infection has to be considered in patients with neurological symptoms following stem cell transplantation, and suggests the necessity of PCR for HHV-6 virus from the CSF.
Assuntos
Aciclovir/uso terapêutico , Antivirais/uso terapêutico , Ganciclovir/uso terapêutico , Infecções por Roseolovirus/tratamento farmacológico , Infecções por Roseolovirus/etiologia , Transplante de Células-Tronco/efeitos adversos , Idoso , Feminino , Herpesvirus Humano 6 , Humanos , Síndromes Mielodisplásicas/terapiaRESUMO
A 63-year-old woman had previously been admitted to another hospital due to fever, abdominal pain and diarrhea. She was treated with fasting, antibiotics and G-CSF administration because of the coexistence of neutropenia, and the symptoms improved. However, discontinuation of G-CSF administration resulted in a recurrence of the neutropenia accompanied with enterocolitis. After admission to our hospital, a diagnosis for idiopathic AIN was performed as she tested positive in both granulocyte immunofluorescence and granulocyte agglutination tests. Administration of corticosteroid following G-CSF resulted in a continuous increase in the neutrophil count and the disappearance of anti-neutrophil autoantibodies.