RESUMEN
Total skin electron beam therapy (TSEBT) is a treatment option for mycosis fungoides (MF). In Japan, it has been rarely performed because of the time required for each treatment, physical burden on patients, and difficulties in providing uniform dosimetry. In recent years, helical tomotherapy, an intensity-modulated radiation therapy that applies helical computed tomography technology, has been used to treat cancer. Total skin helical tomotherapy (TSHT) has been suggested as a promising alternative to TSEBT for patients with MF, but there are few reports from Japan. We used TSHT to treat a 28-year-old Japanese woman with tumor stage MF. She achieved complete remission with TSHT (12 Gy in six fractions over 6 days) and remained in remission for 32 months without additional treatment. Treatment-related grade 4 myelosuppression was observed, but resolved with blood transfusions and subcutaneous injection of granulocyte colony stimulating factor. Other adverse events were tolerable. Although careful attention should be paid to myelosuppression, TSHT might be a useful treatment option for MF.
Asunto(s)
Micosis Fungoide , Radioterapia de Intensidad Modulada , Neoplasias Cutáneas , Adulto , Femenino , Humanos , Micosis Fungoide/radioterapia , Inducción de Remisión , Piel , Neoplasias Cutáneas/radioterapiaRESUMEN
Cutaneous-type adult T-cell leukemia-lymphoma is treated with antiviral or skin-directed therapy. Medications that are used to treat skin lesions of cutaneous T-cell lymphomas are also used for the cutaneous-type adult T-cell leukemia-lymphoma. Etretinate, a synthetic retinoid, has been used for treating cutaneous T-cell lymphomas; however, its clinical effectiveness for the treatment of cutaneous-type adult T-cell leukemia-lymphoma has not been fully studied. We conducted a retrospective assessment of the efficacy and safety of etretinate in 9 patients with cutaneous-type adult T-cell leukemia-lymphoma. Complete and partial responses to etretinate were observed in 1 and 7 patients, respectively. Among the responders, remission was maintained for more than 6 years in 2 patients. These results suggest that etretinate is a promising treatment option for cutaneous-type adult T-cell leukemia-lymphoma.
Asunto(s)
Antineoplásicos/uso terapéutico , Etretinato/uso terapéutico , Leucemia-Linfoma de Células T del Adulto/tratamiento farmacológico , Linfoma Cutáneo de Células T/tratamiento farmacológico , Neoplasias Cutáneas/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Antineoplásicos/efectos adversos , Quimioterapia Adyuvante , Etretinato/efectos adversos , Femenino , Humanos , Leucemia-Linfoma de Células T del Adulto/patología , Linfoma Cutáneo de Células T/patología , Masculino , Persona de Mediana Edad , Terapia PUVA , Estudios Retrospectivos , Neoplasias Cutáneas/patología , Factores de Tiempo , Resultado del Tratamiento , Terapia UltravioletaAsunto(s)
Anticuerpos Monoclonales Humanizados/efectos adversos , Erupciones por Medicamentos/etiología , Leucemia-Linfoma de Células T del Adulto/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Erupciones por Medicamentos/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , PronósticoRESUMEN
Gorham's disease (GD) is a rare and intractable disease characterized by marked progression of osteolysis associated with lymphangioma and/or hemangioma. Here, we describe a case of GD of the proximal tibia occurring in a 10-year-old boy. Although we could not correctly diagnose it at first, we finally diagnosed him as having GD. Progression of osteolysis of the tibia stopped 3 months after the local administration of OK-432. Thereafter, the huge bone defect with varus and extension deformity was reconstructed successfully by distraction osteogenesis using the Ilizarov method. The present case suggests that local administration of OK-432, followed by distraction osteogenesis is a treatment option for GD.
Asunto(s)
Antineoplásicos/uso terapéutico , Osteogénesis por Distracción/métodos , Osteólisis Esencial/terapia , Picibanil/uso terapéutico , Tibia/cirugía , Niño , Humanos , Masculino , Resultado del TratamientoAsunto(s)
Enfermedad de Darier/complicaciones , Enfermedades del Esófago/etiología , Aciclovir/análogos & derivados , Aciclovir/uso terapéutico , Antivirales/uso terapéutico , Biopsia , Enfermedad de Darier/tratamiento farmacológico , Enfermedades del Esófago/tratamiento farmacológico , Esofagoscopía , Etretinato/uso terapéutico , Humanos , Queratolíticos/uso terapéutico , Masculino , Persona de Mediana Edad , Valaciclovir , Valina/análogos & derivados , Valina/uso terapéuticoRESUMEN
Adult T-cell leukemia-lymphoma (ATL), characterized by various clinicopathological features, is divided into four clinical subtypes, namely, acute, lymphoma, chronic and smoldering types, and the treatment strategy differs according to the clinical subtype. The designation cutaneous type ATL has been proposed to describe a peculiar subgroup of smoldering type ATL in which the skin is predominantly affected. However, diagnostic criteria and prognostic factors for cutaneous type ATL remain to be determined. Therefore, we performed a retrospective study to obtain a precise method for subtype classification and to clearly define cutaneous type ATL. A total of 87 ATL patients (acute, n = 31; lymphoma, n = 6; chronic, n = 24; smoldering, n = 26) were enrolled. The human T-lymphotropic virus type I (HTLV-1) proviral load in peripheral blood and the serum soluble interleukin-2 receptor (sIL-2R) level were evaluated with respect to the clinical features of the different types of ATL. The HTLV-1 proviral load was significantly increased in the acute and chronic type and the serum sIL-2R level was increased in the acute and lymphoma type. The HTLV-1 proviral load was significantly lower in cutaneous than other smoldering types of ATL without skin lesions. The clinical findings of cutaneous type ATL were also different from other subtypes. These results indicate that, in combination, determination of the HTLV-1 proviral load and the serum sIL-2R level is useful for distinguishing among the different types of ATL, and strongly suggest that cutaneous type ATL is a distinct clinical entity.
Asunto(s)
Leucemia-Linfoma de Células T del Adulto/virología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Humanos , Leucemia-Linfoma de Células T del Adulto/clasificación , Leucemia-Linfoma de Células T del Adulto/inmunología , Linfoma Cutáneo de Células T/clasificación , Linfoma Cutáneo de Células T/inmunología , Linfoma Cutáneo de Células T/virología , Masculino , Persona de Mediana Edad , Provirus/aislamiento & purificación , Receptores de Interleucina-2/sangre , Estudios Retrospectivos , Neoplasias Cutáneas/clasificación , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/virología , Carga Viral , Adulto JovenRESUMEN
Granulocyte and monocyte adsorption apheresis (GMA), an extracorporeal apheresis instrument whose column contains cellulose acetate (CA) beads, is designed to remove activated granulocytes and monocytes. We previously demonstrated that GMA was useful for treating neutrophilic dermatoses and associated arthropathy as it adsorbs Mac-1 (CD11b/CD18)-expressing neutrophils to the CA beads by the binding of complement component (iC3b) and CD11b expressed on activated neutrophils. The objective of this study is to further assess the clinical effectiveness of GMA in the treatment of neutrophilic dermatoses and associated arthropathy. The effect of GMA for skin lesions and joint lesions was assessed in 44 and 23 patients, respectively. Mac-1 expression on peripheral neutrophils was measured by flow cytometry. Skin lesions and arthropathy improved in 39 of 44 patients (88.6%) and 22 of 23 (95.6%), respectively. Mac-1 (CD11b/CD18) expression on the peripheral neutrophils, 27.1 ± 6.66 MFI (mean fluorescence intensity) before treatment, was reduced to 17.9 ± 3.02 MFI by GMA (P < 0.05). Clinical effectiveness of GMA for the treatment of intractable neutrophilic dermatoses and associated arthropathy was further confirmed.
Asunto(s)
Leucaféresis/métodos , Antígeno de Macrófago-1/metabolismo , Neutrófilos/metabolismo , Enfermedades de la Piel/terapia , Adolescente , Adsorción , Adulto , Anciano , Antígeno CD11b/metabolismo , Antígenos CD18/metabolismo , Femenino , Citometría de Flujo , Granulocitos/metabolismo , Humanos , Artropatías/patología , Artropatías/terapia , Masculino , Persona de Mediana Edad , Monocitos/metabolismo , Psoriasis/patología , Psoriasis/terapia , Enfermedades de la Piel/patología , Resultado del Tratamiento , Adulto JovenAsunto(s)
Células Dendríticas/patología , Infecciones por HTLV-I/complicaciones , Virus Linfotrópico T Tipo 1 Humano , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Portador Sano/virología , Resultado Fatal , Infecciones por HTLV-I/virología , Humanos , Masculino , Neoplasias Cutáneas/tratamiento farmacológicoRESUMEN
BACKGROUND: The use of random skin biopsy (RSB) to diagnose intravascular large B cell lymphoma (IVLBCL) has increased. OBJECTIVE: To explore the indication for RSB to diagnose IVLBCL. METHODS: We retrospectively evaluated the medical records of 18 Japanese adults who underwent RSB between January 2008 and December 2009. RESULTS: A final diagnosis of IVLBCL was returned in 2 patients based on RSB findings and in 1 based on brain biopsy findings. All 3 patients manifested neurological symptoms, hematocytopenia, elevated levels of LDH and soluble interleukin-2 receptor (sIL-2R), and the absence of lymphadenopathy. Malignant lymphoma other than IVLBCL was diagnosed in 6 patients, and in 5 of 6 patients who underwent nodal or parenchymal biopsy diagnostic findings were made. CONCLUSION: Although RSB is useful for the early diagnosis of IVLBCL, careful selection of patients is necessary. In patients with neurological symptoms, hematocytopenia, elevated LDH and sIL-2R and no nodal involvement, RSB may be useful.
Asunto(s)
Biopsia/métodos , Linfoma de Células B Grandes Difuso/patología , Adulto , Anciano , Anciano de 80 o más Años , Pueblo Asiatico , Biopsia/normas , Diagnóstico Diferencial , Femenino , Humanos , Enfermedades Linfáticas/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
BACKGROUND: Traditionally, dermatophytosis, a common disease affecting millions of people world-wide, has been diagnosed by direct microscopy and fungal culture. The immunochromatography (ICG) strip test was recently developed. METHODS: We compared the performance of the ICG strip test for the detection of dermatophytes in samples from human skin and nails with direct microscopy. The 160 samples, consisting of 88 skin and 72 nail specimens, were subjected to direct microscopy study using a 20% KOH solution and to examination with the ICG strip test. Of 160 samples, 18 were examined by fungal culture using Sabouraud dextrose agar medium. RESULTS: We found that the overall sensitivity and specificity of the ICG test were 83.5% and 66.7%; they were 82.1% and 76.2% for the 88 skin and 85.4% and 58.3% for the 72 nail specimens, respectively. CONCLUSIONS: Our findings indicate that the efficacy of the ICG test is comparable to direct microscopy for the detection of dermatophytes. Performance of the assay was easy, and results were available quickly. We suggest that it is an effective tool for dermatophytosis screening.
Asunto(s)
Cromatografía de Afinidad/métodos , Tiras Reactivas , Tiña/diagnóstico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Femenino , Humanos , Masculino , Microscopía , Persona de Mediana Edad , Onicomicosis/diagnóstico , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Lactoferrin (LF), an iron-binding glycoprotein, exhibits several biological activities, including anti-viral activity and immunomodulatory functions. LF has been reported to inhibit hepatitis C virus (HCV) infection in cultured human hepatocytes and HCV viremia in low pretreatment HCV RNA titers of patients with chronic hepatitis C (CHC). However, the combination of interferon (IFN) alpha-2b plus ribavirin with LF for CHC has not been previously investigated. Thirty-six CHC patients, who were positive for HCV RNA with high serum levels of HCV RNA or who did not respond to or relapsed after interferon monotherapy, were randomly assigned to two groups: IFN alpha-2b and ribavirin plus LF for 24 weeks (18 patients), and IFN alpha-2b and ribavirin plus placebo (18 patients). Treatment was discontinued in three patients (17%) in the LF group and eight patients (44%) in the placebo group. For the 25 patients who finished the 24 weeks of treatment, virological sustained response was seen in 6 (40%) patients in the LF group and in 5 (50%) patients in the placebo group and there was no statistically significant difference between the two groups (p=0.7). Serum alanine aminotransferase concentrations remained normal throughout the follow-up period in nine patients (60%) in the LF group as compared with five patients (50%) in the placebo group (p=0.7). The proportion of patients with a virological or biochemical response at the end of the treatment period did not differ between the two groups. Furthermore, there were no statistically significant differences between the two groups in hemoglobin concentration, serum iron, ferritin, Th1/Th2 ratio or ribavirin concentration throughout the treatment and follow-up periods. In conclusion, we could not demonstrate that LF in combination with IFN alpha-2b and ribavirin increases the virological and biochemical response rate for CHC patients with high serum levels of HCV RNA or for CHC patients who do not response to or relapse after IFN monotherapy.
RESUMEN
While enhanced expression of cyclooxygenase (COX)-2 has been observed in human skin epidermal cancer, the mechanisms underlying COX-2 expression have not been completely elucidated. Recently, a role for the phosphatidylinositol-3 (PI3) kinase pathway in COX-2 expression has attracted attention. We investigated COX-2 expression, PI3 kinase activity, and the phosphorylation level of Akt, a downstream effector of PI3 kinase, in the human skin cancer cell line HSC-5. Compared to the nontumorigenic keratinocyte HaCaT, in HSC-5 cells, COX-2 protein expression and PI3 kinase activity were increased. The PI3 kinase inhibitor LY294002 reduced COX-2 expression in HSC-5 cells and, contrary to our expectation, the phosphorylation of Akt was significantly decreased. The expression of Bcl-2, which is regulated by Akt, was reduced, and apoptosis was induced in HSC-5 cells compared to HaCaT cells. COX-2 inhibitor NS398 up-regulated Akt phosphorylation. These results imply that constitutively over-expressed COX-2 down-regulates the Akt phosphorylation through a negative feedback mechanism.
Asunto(s)
Biomarcadores de Tumor/metabolismo , Fragmentación del ADN , ADN de Neoplasias , Isoenzimas/metabolismo , Fosfatidilinositol 3-Quinasas/metabolismo , Prostaglandina-Endoperóxido Sintasas/metabolismo , Apoptosis/fisiología , Western Blotting , Supervivencia Celular , Ciclooxigenasa 2 , Regulación hacia Abajo , Células Epidérmicas , Retroalimentación , Humanos , Queratinocitos/fisiología , Proteínas de la Membrana , Sensibilidad y Especificidad , Transducción de Señal , Neoplasias Cutáneas/patología , Células Tumorales CultivadasRESUMEN
We report a 71-year-old woman with antiepiligrin cicatricial pemphigoid associated with nephrotic syndrome. She presented with a six-month history of pruritic blisters over her trunk and legs. She also had episodes of recurrent painful oral erosions. A skin biopsy showed a subepidermal bulla, and a direct immunofluorescence (DIF) study revealed linear deposition of IgG and C3 at the basement membrane zone (BMZ). Indirect immunofluorescence (IIF) staining of 1 M NaCl-split skin demonstrated circulating IgG autoantibodies reactive with the dermal side. Immunoprecipitation studies of the patient's serum disclosed IgG autoantibodies directed against a set of polypeptides that corresponded to laminin 5 (beta 3 gamma 2). Based upon the long-standing edema of her legs and her hypoproteinemia, she was diagnosed with nephrotic syndrome. To our knowledge, the association of antiepiligrin cicatricial pemphigoid with nephrotic syndrome has not been reported previously.