RESUMO
We aimed to investigate the possible causes of metamorphopsia for rhegmatogenous retinal detachment (RRD) based on findings of enface optical coherence tomography (OCT). The study was a retrospective, consecutive case series of 33 eyes with macula-off RRD. Metamorphopsia was measured by M-CHARTS and enface OCT images covering a macular area of 6 × 6 mm square obtained at 1, 3, and 6 months postoperatively. Within the outer retinal slabs of enface OCT, multiple lines that looked like folds were delineated in all eyes at month 1, and we succeeded in extracting images of folds by subtracting retinal vessels. We calculated their density by an image-processing technique. The mean M-CHARTS scores were 0.62 ± 0.47 at month 1 and 0.30 ± 0.29 at month 6 (P < 0.001). The fold density was 8.3 ± 4.2 at month 1 and 6.1 ± 3.1 at month 6 (P = 0.0044). The M-CHARTS scores at 6 months were significantly associated with the fold density at 1 month (r = 0.515, P = 0.002). In conclusion, enface OCT visualized the outer retinal folds in eyes that had undergone successful RRD surgery, and a larger number of folds was related to the remaining metamorphopsia.
Assuntos
Retina/diagnóstico por imagem , Retina/cirurgia , Descolamento Retiniano/diagnóstico por imagem , Descolamento Retiniano/cirurgia , Tomografia de Coerência Óptica , Vitrectomia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Retina/patologia , Retina/fisiopatologia , Descolamento Retiniano/fisiopatologia , Fatores de Tempo , Transtornos da Visão/etiologia , Transtornos da Visão/fisiopatologia , Acuidade Visual , Vitrectomia/efeitos adversosRESUMO
A young female patient in a second remission of acute lymphoblastic leukemia underwent bone marrow transplantation after total body irradiation and high-dose cytarabine from her HLA-matched brother. Following successful engraftment, mixed chimerism was seen 75 days post transplant. The karyotype contained numerous abnormalities in residual recipient cells. Chromosomes 1, 7, 13, and X were significantly more affected than other chromosomes. The high-frequency breakpoints identified were 1p22.2, 5q31.2, and 13q14.2. Some karyotypes specific for leukemia, such as t(9;22)(q34.1;q11.2) and t(8;21)(q22.2;q22.2), not seen with the original disease, were also present. As the frequency of aberrant chromosomes increased markedly with time, donor leukocytes were infused 14 months after BMT, which effectively eradicated the abnormal karyotypes.
Assuntos
Células da Medula Óssea/patologia , Transplante de Medula Óssea/efeitos adversos , Aberrações Cromossômicas , Pré-Escolar , Células Clonais/patologia , Terapia Combinada , Feminino , Humanos , Cariotipagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Transplante HomólogoRESUMO
We report a 14-year-old boy who had successfully received allogeneic BMT for chronic granulomatous disease and 3 years later was treated with donor lymphocyte infusion (DLI, 3.3 x 10(8) cells/kg) at unstable mixed chimerism in association with reduced neutrophil function. Following DLI, the patient developed transient acute hepatic GVHD, which was confirmed by liver biopsy and was manageable with cyclosporin A and prednisolone. The patient eventually attained complete chimerism with improved neutrophil function. At the time of writing (2.5 years from the DLI), the patient is doing well, free from infectious episodes and chronic GVHD. Our experience suggests that DLI could be a safe and effective strategy for dissolution of unstable mixed chimerism in BMT recipients for inherited disorders.
Assuntos
Transplante de Medula Óssea , Doença Granulomatosa Crônica/terapia , Transfusão de Linfócitos , Adolescente , Quimera , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/fisiopatologia , Doença Granulomatosa Crônica/fisiopatologia , Humanos , Fígado/fisiopatologia , Hepatopatias/etiologia , Hepatopatias/fisiopatologia , Transfusão de Linfócitos/efeitos adversos , Masculino , Neutrófilos/fisiologia , Doadores de Tecidos , Transplante HomólogoRESUMO
We report two cases of Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPD) after allogenic bone marrow transplantation which were conditioned with regimens including antithymocyte globulin (ATG). The first case was a 31 year-old man which severe aplastic anemia who was transplanted from HLA-matched unrelated donor conditioned with total lymphoid irradiation (TLI)/ cyclophosphamide/ATG and prophylactic administration of ganciclovir Grade I acute GVHD improved in response to cyclosporine (CsA). LPD as a polyclonal epipharyngeal mass developed at day +53 and spontaneously regressed along with the withdrawal of CsA. Second case was a 11 year-old boy with acute myelomonocytic leukemia (FAB:M4E). He was transplanted from HLA B locus mismatched mother conditioned with total body irradiation (TBI)/busulfan/L-PAM/ATG. He showed grade IV acute GVHD, which was controlled by steroids and FK-506. LPD as a monoclonal intestinal lymphoma was diagnosed at day +82, and he was unsuccessfully treated with ganciclovir, acyclovir, chemotherapy and transfusions of EBV-specific cytotoxic lymphocytes in addition to discontinuation of immunosuppressants, and died at day +18 due to sepsis and multiple cerebral infarction. Early detection and introduction of appropriate treatment for post bone marrow transplantation LPD is necessary.
Assuntos
Transplante de Medula Óssea/efeitos adversos , Infecções por Herpesviridae/complicações , Herpesvirus Humano 4 , Transtornos Linfoproliferativos/etiologia , Adulto , Anemia Aplástica/terapia , Criança , Infecções por Herpesviridae/transmissão , Humanos , Terapia de Imunossupressão/efeitos adversos , Leucemia Mielomonocítica Aguda/terapia , MasculinoAssuntos
Transplante de Medula Óssea/efeitos adversos , Infecções por Herpesviridae/etiologia , Herpesvirus Humano 4 , Transtornos Linfoproliferativos/etiologia , Infecções Tumorais por Vírus/etiologia , Adulto , Anemia Aplástica/terapia , Eletroforese das Proteínas Sanguíneas , Infecções por Herpesviridae/sangue , Herpesvirus Humano 4/imunologia , Humanos , Transtornos Linfoproliferativos/sangue , Masculino , Fatores de Tempo , Transplante Homólogo , Infecções Tumorais por Vírus/sangueRESUMO
We report the case of a 14-year-old boy with myelodysplastic syndrome (MDS/RAEB) which developed following Fanconi anemia. The patient received BMT from an HLA-identical sister. Based on the in vitro CY-sensitivity test, 100 mg/kg of CY was administered for conditioning combined with 6 Gy TBI. Mucosal symptoms such as stomatitis, diarrhea and hematuria were severe, but manageable, and engraftment was successful. The patient has maintained normal trilineage hematopoiesis with > 90% Karnofsky score for 30 months with disappearance of a clonal chromosomal abnormality (47,XY, +i(lq)) which was detected before BMT.
Assuntos
Transplante de Medula Óssea , Anemia de Fanconi/complicações , Síndromes Mielodisplásicas/terapia , Adolescente , Ciclofosfamida/uso terapêutico , Humanos , Masculino , Síndromes Mielodisplásicas/etiologia , Transplante HomólogoRESUMO
A 78-year-old man was admitted to the hospital with exertional dyspnea and fever. Chronic idiopathic interstitial pneumonia (IIP) had been diagnosed a year before. Follow-up chest X-ray examination showed diffuse reticular shadows and progressive shrinkage of both lower lobes. Chest CT scan revealed honeycomb appearance of both lower lung fields. Moderate hypoxemia was found by arterial blood gas analysis. The patient responded to an orally administered corticosteroid. Because tapering of the medicine caused exacerbation of the hypoxemia, methylprednisolone pulse therapy was given with poor response. Then high-dose (750 mg) of cyclophosphamide (CPM) were given intravenously 6 times, every 3 or 4 weeks. After the second administration of CPM, decreases in hypoxemia and in blood LDH levels were observed. This treatment allowed as to taper the dose of oral corticosteroid from 60 mg/day to 15 mg/day. This case suggests that intravenous administration of high-dose CPM may be effective against IIP.
Assuntos
Ciclofosfamida/administração & dosagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Idoso , Esquema de Medicação , Tolerância a Medicamentos , Humanos , Injeções Intravenosas , Masculino , Prednisolona/administração & dosagemRESUMO
A case of nemaline myopathy with rimmed vacuoles was presented. Muscle biopsy revealed type I fiber predominance with type grouping, thus suggesting that neurogenic factors played a pathologic role in the degenerative change.