RESUMEN
OBJECTIVES: Optimal selection of pretransplant conditioning is crucially vital for improving survival and quality-of-life of patients who receive allogeneic hematopoietic cell transplantation (allo-HCT), particularly in those with high-risk diseases. In this study, we evaluated the efficacy and safety of recently-developed reduced-toxicity myeloablative regimen that combines fludarabine, intravenous busulfan, and melphalan (FBM). METHODS: We conducted a single-center retrospective analysis of 39 patients (23 with myeloid neoplasms and 16 with lymphoid neoplasms), with a median age of 50 (range, 17-68) years, who underwent their first allo-HCT using the FBM regimen. Graft types were bone marrow in 11, peripheral blood in 11, and cord blood in 17 patients. Cyclosporine- or tacrolimus-based graft-versus-host disease (GVHD) prophylaxis was administered. The primary end point of the study was the overall survival rate at 2-year after transplantation. RESULTS: After a median follow-up of 910 days for the surviving patients, 2-year overall survival was 62% for the entire cohort; 73% in the low-to-intermediate-risk group and 44% in the high-to-very high-risk group classified by the refined CIBMTR Disease Risk Index. Cumulative incidences of engraftment, grade II-IV acute GVHD, chronic GVHD, relapse, and non-relapse mortality were 95%, 56%, 56%, 31%, and 17%, respectively. CONCLUSION: These results suggest that our FBM regimen can be applied to allo-HCT using various graft types and yields acceptable outcomes with relatively low non-relapse mortality in both myeloid and lymphoid neoplasms. Also, we observed a promising survival in the group of patients with high-risk diseases, warranting more accumulation of patients and longer follow-up.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Hematológicas/terapia , Trasplante de Células Madre Hematopoyéticas , Acondicionamiento Pretrasplante , Adolescente , Adulto , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Busulfano/administración & dosificación , Terapia Combinada , Femenino , Enfermedad Injerto contra Huésped/diagnóstico , Enfermedad Injerto contra Huésped/etiología , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/mortalidad , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Masculino , Melfalán/administración & dosificación , Persona de Mediana Edad , Agonistas Mieloablativos/administración & dosificación , Recurrencia , Inducción de Remisión , Estudios Retrospectivos , Análisis de Supervivencia , Acondicionamiento Pretrasplante/efectos adversos , Acondicionamiento Pretrasplante/métodos , Trasplante Homólogo , Resultado del Tratamiento , Vidarabina/administración & dosificación , Vidarabina/análogos & derivados , Adulto JovenRESUMEN
Immunoglobulin G4-related disease (IgG4-RD) is defined as an inflammatory lymphoproliferative disorder. The relationship between malignancies and IgG4-RD remains unclear. We herein present a case of IgG4-RD that occurred during chemotherapy for advanced breast cancer. In this case, it was challenging to determine which of these diseases was responsible for the patient's mediastinal lymphadenopathy. Lymphadenopathy with IgG4-RD was diagnosed by assessing the reactivity to corticosteroids, which were used as premedication in chemotherapy, over time. The administration of prednisolone, which was initiated to treat active IgG4-RD, led to stable systemic therapy for malignancy. It is imperative to assess the disease activity and consider each treatment.
Asunto(s)
Antineoplásicos Inmunológicos/efectos adversos , Neoplasias de la Mama/tratamiento farmacológico , Enfermedad Relacionada con Inmunoglobulina G4/etiología , Linfadenopatía/etiología , Anciano , Antineoplásicos Inmunológicos/uso terapéutico , Femenino , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Prednisolona/uso terapéuticoRESUMEN
Trastuzumab emtansine (T-DM1) is an antibody-drug conjugate approved for use in the treatment of human epidermal growth factor receptor 2-positive metastatic breast cancer. Here, we present the cases of two patients with metastatic breast cancer who received T-DM1 monotherapy and developed noncirrhotic portal hypertension (NCPH). Patient 1 presented with ruptured gastric varices at 2 years and 5 months after T-DM1 treatment. Patient 2 presented with intrahepatic portal-hepatic venous shunt at 2 years and 6 months and portal-systemic shunt encephalopathy at 4 years and 11 months after T-DM1 treatment. In both the patients, liver biopsies revealed sinusoidal obstruction syndrome (SOS). T-DM1-induced hepatotoxicity can result from SOS. In long-term administration of T-DM1 the unfavorable events associated with chronic liver circulatory disorder due to SOS, such as NCPH, are concerning.
RESUMEN
Acute chest syndrome (ACS), characterized by fever, respiratory symptoms, and new pulmonary infiltration, is a serious complication of sickle cell disease (SCD). Regardless of the etiology, the conventional treatment options for ACS include empirical antibiotic therapy, the administration of analgesics, and red cell transfusion. The indications and methods of red cell transfusion are critical. We herein report the case of a 26-year-old African-American man with SCD who developed ACS and who was successfully treated with manual exchange transfusion. Despite increasing globalization, SCD remains extremely rare in Japan. Manual exchange transfusion can be performed easily anywhere and should be considered for treating SCD patients presenting with ACS.
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Síndrome Torácico Agudo/terapia , Anemia de Células Falciformes/complicaciones , Transfusión de Eritrocitos/métodos , Talasemia beta/complicaciones , Síndrome Torácico Agudo/diagnóstico por imagen , Síndrome Torácico Agudo/etiología , Adulto , Humanos , Masculino , Radiografía Torácica , Tomografía Computarizada por Rayos XRESUMEN
We herein report two cases of AIHA (autoimmune hemolytic anemia), a 25-year-old woman and a 77-year-old man, who presented with normal serum LDH values. Though in these two cases, low hemoglobin and haptoglobin, high total bilirubin and positive direct Coombs' test results led to the diagnosis of AIHA, both patients had normal LDH levels (218 and 187 IU/l). Both cases were successfully treated with prednisone. In the diagnosis of AIHA, elevated LDH is usually used as a marker of hemolysis. However, medical records of 24 AIHA patients collected in our institute from January 2001 to August 2012 revealed LDH levels to have been normal in 25% of these cases. This report indicates the importance of obtaining complete information about the blood testing of patients and taking these data into account when considering the diagnosis of AIHA.
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Anemia Hemolítica Autoinmune/diagnóstico , Lactato Deshidrogenasas/sangre , Adulto , Anciano , Femenino , Humanos , MasculinoRESUMEN
Recently, 5-azacitidine has been reported to improve the survival of patients with high-risk myelodysplastic syndrome (MDS) and was approved for the treatment of MDS in Japan. We herein report a case of high-risk MDS in which the patient exhibited a hematological improvement three months after the first cycle of 5-azacitidine therapy. The second cycle of 5-azacitidine was not administered due to a severe pulmonary infection. Bone marrow aspiration revealed a decrease in the level of blast cells from 7.0% to 0.7%, and the subclassification of MDS improved from refractory anemia with excess blasts (RAEB)-1 to refractory cytopenia with unilineage dysplasia. This case demonstrates a possible late effect of 5-azacitidine treatment.
Asunto(s)
Azacitidina/uso terapéutico , Hemoglobinas/metabolismo , Síndromes Mielodisplásicos/tratamiento farmacológico , Neutrófilos/patología , Anciano , Antimetabolitos Antineoplásicos/uso terapéutico , Biopsia con Aguja , Células de la Médula Ósea/patología , Estudios de Seguimiento , Humanos , Masculino , Síndromes Mielodisplásicos/sangre , Síndromes Mielodisplásicos/diagnóstico , Recuento de PlaquetasRESUMEN
Abstract Familial platelet disorder with predisposition to acute myeloid leukemia (FPD/AML) is an autosomal dominant disorder characterized by mild to moderate thrombocytopenia with or without its impaired function, inherited RUNX1 mutation and high incidence of myeloid malignancy, such as myelodysplastic syndrome or acute myeloid leukemia. A 72-year-old male visited our institute because of gradually progressive pancytopenia and splenomegaly, and was diagnosed as having hairy cell leukemia. He was administered one course of intravenous cladribine (0.12 mg/kg, day 1-5) and achieved hematological complete response. Mutation analyses of RUNX1 gene were underwent because familial history of hematological malignancies evoked a possibility of FPD/AML. As a result, RUNX1 L445P mutation was identified in the peripheral blood and the mutation was considered as germ-line mutation because the same mutation was detected in the buccal mucosa. BRAF V600E mutation was also identified in the peripheral blood but not in the buccal mucosa. To our knowledge, this is the first report of B cell malignancy arising from FPD/AML.
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Trastornos de las Plaquetas Sanguíneas/complicaciones , Susceptibilidad a Enfermedades , Leucemia de Células Pilosas/etiología , Anciano , Biopsia , Trastornos de las Plaquetas Sanguíneas/diagnóstico , Médula Ósea/patología , Subunidad alfa 2 del Factor de Unión al Sitio Principal/genética , Análisis Mutacional de ADN , Mutación de Línea Germinal , Homocigoto , Humanos , Leucemia de Células Pilosas/diagnóstico , Masculino , Linaje , Esplenomegalia/diagnóstico , Tomografía Computarizada por Rayos XAsunto(s)
Capilares/patología , Eritema/etiología , Linfohistiocitosis Hemofagocítica/complicaciones , Linfoma de Células B/etiología , Neoplasias Cutáneas/etiología , Piel/irrigación sanguínea , Piel/patología , Neoplasias Vasculares/etiología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Examen de la Médula Ósea , Eritema/tratamiento farmacológico , Eritema/patología , Femenino , Humanos , Inmunohistoquímica , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfoma de Células B/tratamiento farmacológico , Linfoma de Células B/patología , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Resultado del Tratamiento , Neoplasias Vasculares/tratamiento farmacológico , Neoplasias Vasculares/patologíaRESUMEN
We report a case of bucillamine-induced interstitial pneumonia accompanied by severe hypoxemia in an 83-year-old woman who had rheumatoid arthritis. Respiratory failure worsened even after withdrawal of bucillamine and administration of high-dose corticosteroids, and mechanical ventilation was required. A review of 15 cases with bucillamine-induced pulmonary injury suggests that advanced age may be associated with the development of severe interstitial pneumonia. Bucillamine can cause corticosteroid-resistant and life-threatening lung injury, especially in the elderly.