RESUMEN
Plexiform neurofibroma is a benign proliferation that arises from the peripheral nerves and represents one of the diagnostic features of neurofibromatosis type I (NF-1). They are commonly found in the gastrointestinal tract, and intrathoracic neurofibroma is relatively uncommon. Ultimately, this tumor grows along the length of any single nerve and may involve multiple fascicles or branches of major nerves. Here, we report a case of multiple-site plexiform neurofibromas in a 40-year-old man previously diagnosed with NF-1. Although he had no perceptible symptoms, contrast-enhanced CT revealed massive diffuse neurofibromas that involved the mediastinum, periportal region, retroperitoneal space, and the mesentery. Histopathological studies of the neck, skin, and intra-abdominal soft tissue showed loose spindle cells and collagen bundles. The microscopic appearance was typical of a plexiform-type neurofibroma. It was decided that we would observe the patient without surgical management, because the neurofibromas were asymptomatic.
Asunto(s)
Adulto , Humanos , Abdomen , Colágeno , Tracto Gastrointestinal , Mediastino , Mesenterio , Cuello , Neurofibroma , Neurofibroma Plexiforme , Neurofibromatosis , Neurofibromatosis 1 , Nervios Periféricos , Espacio Retroperitoneal , PielRESUMEN
BACKGROUND: The purpose of this study was to evaluate the toxicity and efficacy of high-dose chemotherapy with busulfan, thiotepa and melphalan (BTM) as a myeloablative regimen in allogeneic bone marrow transplantation (allo-BMT) for patients with acute myelogenous leukemia (AML). METHODS: Twenty-seven patients with AML were enrolled; Sixteen patients had standard risk (SR) diseases (first complete remission (CR1) and de novo AML) and eleven patients had high risk (HR) diseases (second, or subsequent remission, secondary AML, relapsed, or refractory AML, CR marrow with persisting extramedullary manifestation (chloroma), or hypoplastic acute leukemia). The conditioning regimen included busulfan 4 mg/kg/day for a total dose of 12 mg/kg; thiotepa 250 mg/m2/day for a total dose of 500 mg/m2; and melphalan 50 mg/m2/day for a total dose of 100 mg/m2. Cyclosporine A and short-course methotrexate were used for graft-versus-host disease (GVHD) prophylaxis. RESULTS: The median time to recovery a granulocyte count of 0.5 x 109/L was 14 days (range 10~25 days) and platelet transfusion independence was 30 days (range 12~49 days). The major regimen-related toxicities were gastrointestinal-related symptoms including oral mucositis, nausea, vomiting, and diarrhea. All patients experienced oral mucositis (> or = grade 1) and the patients with oral mucositis of equal and greater than grade 3 were 44% in SR and 45% in HR. The toxicities associated with lung, skin, heart and brain were minimal. Three (11%) patients had severe or fatal veno-occlusive disease (VOD). There were five treatment-related death (19%) (hepatic VOD with multiorgan failure (n=3), pneumonia and ARDS (n=2)) within the first 100 days after allo-BMT. There was not a significant difference between SR and HR group (p=0.167). The incidence of acute GVHD equal or greater than grade II was less than 10%. The actual survival at 2 year was 70.4%(95% confidence interval (CI), 54.7%~86.1%)(SR; 81.3% (95% CI; 63.4~99.1%) vs HR; 54.6% (95% CI; 28.7~80.4%), p=0.154). After a median follow-up of 630 days, 18 of 27 (67%, 355~1062 days) patients are alive without evidence of disease. Three of the 27 patients relapsed (SR; 0% vs HR; 55.6% (95% CI; 19.6~71.3%), p=0.004). CONCLUSION: The BTM regimen followed by allo-BMT is associated with acceptable toxicity and appears to have significant activity in patients with AML. It should be used with caution in patients with prior hepatopathy or refractory state who have an increased risk of severe VOD. Busulfan, thiotepa, and melphalan is an effective and alternative myeloablative regimen for patients with AML.
Asunto(s)
Humanos , Trasplante de Médula Ósea , Médula Ósea , Encéfalo , Busulfano , Ciclosporina , Diarrea , Quimioterapia , Estudios de Seguimiento , Enfermedad Injerto contra Huésped , Granulocitos , Corazón , Incidencia , Leucemia Mieloide Aguda , Pulmón , Melfalán , Metotrexato , Náusea , Transfusión de Plaquetas , Neumonía , Piel , Estomatitis , Tiotepa , Trasplante Homólogo , VómitosRESUMEN
A patient presenting paroxysmal nocturnal hemoglobinuria (PNH) cloned cells in the course of myelodysplastic syndrome (MDS) with reticulocytosis is described. The bone marrow biopsy demonstrated erythroid hyperplasia and moderate dysplasia. Mild hemoglobinuria was detected but the Ham test was negative. The reticulocyte survival test revealed sustained survival curve indicating delayed reticulocyte maturation regarded as the characteristic of MDS cloned erythroid cells. The glycosylphosphatidylinositol-linked protein deficient neutrophils and erythrocytes population regarded as PNH clones were identified by flow cytometric analysis using monoclonal antibody. From these results, we concluded that MDS and PNH cloned cells were coexisited in this patient. In this patient, long-term follow-up observation could clarify whether MDS and PNH were arising from the same clone or from two distinct clones.
Asunto(s)
Humanos , Biopsia , Médula Ósea , Células Clonales , Eritrocitos , Células Eritroides , Estudios de Seguimiento , Glicosilfosfatidilinositoles , Hemoglobinuria , Hemoglobinuria Paroxística , Hiperplasia , Síndromes Mielodisplásicos , Neutrófilos , Reticulocitos , ReticulocitosisRESUMEN
No abstract available.
Asunto(s)
Glomerulonefritis Membranosa , Carcinoma Pulmonar de Células PequeñasRESUMEN
No abstract available.