RESUMO
Patients <12 months with favorable biology, metastatic neuroblastoma have >90% overall survival following treatment with chemotherapy and surgery. We report two infants with favorable biology, stage 4 neuroblastoma with refractory disease after standard intermediate-risk chemotherapy and additional retrieval chemotherapy. One patient was treated with six additional cycles of isotretinoin and the other observed. Both remain clinically well with persistent disease but no evidence of tumor progression for 28 and 13 months following completion of cytotoxic treatment. Similar to residual tumor in primary sites, refractory metastatic disease may not portend a poor outcome in patients with favorable biology, intermediate-risk neuroblastoma.
Assuntos
Antineoplásicos/uso terapêutico , Isotretinoína/uso terapêutico , Neuroblastoma/secundário , Neoplasias Abdominais/tratamento farmacológico , Neoplasias Abdominais/genética , Neoplasias Abdominais/secundário , Neoplasias Abdominais/cirurgia , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/cirurgia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais , Terapia Combinada , Progressão da Doença , Feminino , Humanos , Lactente , Metástase Linfática , Masculino , Neuroblastoma/tratamento farmacológico , Neuroblastoma/genética , Neuroblastoma/cirurgia , Neoplasias Orbitárias/tratamento farmacológico , Neoplasias Orbitárias/genética , Neoplasias Orbitárias/secundário , Prognóstico , Neoplasias Retroperitoneais/tratamento farmacológico , Neoplasias Retroperitoneais/genética , Neoplasias Retroperitoneais/cirurgia , Risco , Terapia de Salvação , Neoplasias Torácicas/tratamento farmacológico , Neoplasias Torácicas/genética , Neoplasias Torácicas/secundário , Resultado do Tratamento , Conduta ExpectanteRESUMO
We report a case of a 14-year-old boy who presented with gross hematuria and focal bilateral renal masses as presenting features of a mature high-grade B-cell leukemia, Burkitt subtype. The patient was treated with standard chemotherapy with the addition of rituximab and has no evidence of disease on completion. To our knowledge, this is the first case to describe gross hematuria in conjunction with focal bilateral renal masses as presenting features in a pediatric patient with Burkitt leukemia. This unique presentation suggests that early leukemic infiltration of the urinary collecting system may occur but is extremely unusual.
Assuntos
Linfoma de Burkitt/diagnóstico , Neoplasias Renais/diagnóstico , Leucemia de Células B/diagnóstico , Adolescente , Linfoma de Burkitt/complicações , Hematúria/etiologia , Humanos , Neoplasias Renais/complicações , Leucemia de Células B/complicações , MasculinoRESUMO
Paroxysmal cold hemoglobinuria (PCH) is an acquired hemolytic anemia caused by immunoglobulin G (IgG) antibodies that sensitize red blood cells (RBCs) at cold temperatures by fixing complement to the RBCs causing intravascular hemolysis on rewarming. PCH usually appears in young children as recurrent high fevers, chills, and passage of red-brown urine. The diagnostic test for PCH is the Donath-Landsteiner test, an in vitro assay for biphasic hemolysis. Herein, we present 2 cases of PCH that occurred within 12 months of each other. We quickly diagnosed the second case and treated the patient successfully, in part due to our recognition of its characteristics based on the first case. PCH is a hemolytic anemia for which there is a specific diagnostic test; the timely recognition of this entity by physicians and laboratory staff will allow prompt, supportive therapy and will raise the odds of quick resolution of hemolysis.