RESUMEN
Autoimmune hemolytic anemia (AIHA) is a hematologic disorder that is rarely seen in infants and young children. Most cases are associated with viral or bacterial infection, but the immunologic events leading to hemolysis are poorly understood. We describe two infants with severe cytomegalovirus (CMV)-associated warm antibody AIHA. One case was immunohematologically analyzed and showed suggestive evidence that endogenous anti-CMV IgG antibodies were the pathogenic antibodies leading to hemolysis, implicating a possible causal relationship between AIHA and CMV infection. Both patients were ultimately treated with intravenous CMV immune globulin, with subsequent improvement. These cases suggest that investigation for the presence of CMV in infantile AIHA is warranted and that CMV immune globulin should be considered as a therapeutic option.
Asunto(s)
Anemia Hemolítica Autoinmune/etiología , Enfermedades Autoinmunes/etiología , Infecciones por Citomegalovirus/complicaciones , Anemia Hemolítica Autoinmune/sangre , Anemia Hemolítica Autoinmune/terapia , Anticuerpos Antivirales/sangre , Anticuerpos Antivirales/uso terapéutico , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/terapia , Terapia Combinada , Prueba de Coombs , Citomegalovirus/inmunología , Citomegalovirus/aislamiento & purificación , Infecciones por Citomegalovirus/sangre , Infecciones por Citomegalovirus/diagnóstico , Infecciones por Citomegalovirus/tratamiento farmacológico , Infecciones por Citomegalovirus/inmunología , Infecciones por Citomegalovirus/terapia , Femenino , Humanos , Sueros Inmunes , Inmunización Pasiva , Inmunoglobulina G/inmunología , Lactante , Metilprednisolona/uso terapéutico , Nasofaringe/virología , Orina/virologíaRESUMEN
PURPOSE: An atypical case of childhood intraabdominal desmoplastic small round cell tumor (DSRCT) is presented. PATIENT AND METHODS: An 11-year-old boy presented with progressive nausea and vomiting, abdominal pain, hepatomegaly, and an epigastric mass. Computed tomographic scanning as well as findings at gastroscopy and laparotomy revealed a large gastric mural tumor accompanied by multiple large intrahepatic masses. Histopathologic examination of biopsy samples revealed evidence of a polyphenotypic neoplasm diagnostic of DSRCT. RESULTS: Unlike most reported cases of DSRCT, no evidence of peritoneal involvement or ascites was detected. Despite an excellent initial response to multiagent chemotherapy, the patient eventually died of progressive tumor. CONCLUSIONS: Though the radiographic and surgical findings in DSRCT usually suggest a mesenteric, peritoneal, or retroperitoneal site of tumor origin, this case demonstrates that intraabdominal DSRCT can present with a primary visceral lesion without evidence of peritoneal or mesenteric involvement or ascites. Combination chemotherapy using vincristine, doxorubicin, cyclophosphamide, cisplatin, and 5-fluorouracil may be of some benefit to patients with this rare tumor.
Asunto(s)
Neoplasias Abdominales/diagnóstico , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/secundario , Neoplasias Gástricas/diagnóstico , Neoplasias Abdominales/tratamiento farmacológico , Neoplasias Abdominales/secundario , Neoplasias Abdominales/cirugía , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Terapia Combinada , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Humanos , Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/cirugía , Masculino , Neoplasias Gástricas/tratamiento farmacológico , Neoplasias Gástricas/patología , Neoplasias Gástricas/cirugía , Tomografía Computarizada por Rayos X , Vincristina/administración & dosificaciónRESUMEN
Giant hemangiomas occurring in the neonatal period often present a therapeutic challenge, especially when confounded by consumptive coagulopathy (Kasabach-Merritt syndrome). We treated three infants with tranexamic acid after therapy with corticosteroids was ineffective. One patient had a partial response. The remaining two developed progressive disease.