Extramedullary involvement occurs infrequently in acute promyelocytic leukemia and is said to be more common after treatment with all-trans retinoic acid. We describe a 9-year-old girl who had an isolated external auditory canal and middle ear relapse after treatment with all-trans retinoic acid and chemotherapy. A patient with cytogenetically and molecularly confirmed acute promyelocytic leukemia developed isolated extramedullary relapse in the auditory canal and middle ear 4 years and 9 months after initial diagnosis, while in hematologic and molecular remission, successfully treated with arsenic trioxide alone.
RESUMOAs infecções causadas por Dipodascus capitatus são raras e de difícil tratamento. Aqui se relata um caso em paciente com leucemia mielocítica aguda. O isolamento fúngico ocorreu a partir de hemocultura e a identificação fenotípica baseou-se em métodos micológicos clássicos; a identificação genotípica foi realizada através do sequenciamento da região D1/D2 do 26 rDNA. Os testes de suscetibilidade foram realizados através do Etest ® e microdiluição em caldo. A antifungicoterapia foi ineficaz, registrando-se óbito da paciente no 17° dia após o diagnóstico. Os autores comparam o caso com relatos similares e discutem a emergência destas infecções bem como suas dificuldades diagnósticas e terapêuticas. Palavras-chaves: Dipodascus capitatus. Micoses. ABSTRACTThe infections caused by Dipodascus capitatus are rare, and the treatment is difficult. We reported a case of a patient with acute myeloid leukemia. The fungus was first isolated from hemocultures, and the phenotypic identification was based on mycological methods. The genotyping was carried out by sequencing the region D1/D2 from 26 rDNA. The susceptibility tests were assayed by Etest ® and by the microdilution technique. None of the antifungal treatments employed were effective. The patient died on day 17 after the mycological diagnosis. The authors discussed the emergence of such infections as well as the difficulty regarding the diagnosis and treatment. Keywords: Dipodascus capitatus. Mycoses.A severa neutropenia que acompanha os pacientes com leucemia e submetidos à quimioterapia antineoplásica representa elevado risco para infecções fúngicas, notadamente, as causadas por Candida spp e Aspergillus spp. Espécies menos frequentes como Trichosporon, Geotrichum, Rhodotorula, têm emergido como importantes e desafiadores agentes de micoses oportunistas, entre estes pacientes [1][2][3][4] . Neste contexto, relatamos um caso de infecção sistêmica causada por Dipodascus capitatus em paciente com leucemia mielocítica aguda, com evolução fatal.Paciente feminina, 17 anos, procurou atendimento no Hospital Universitário de Santa Maria, relatando febre, náuseas, sudorese, tonturas e dor na axila esquerda há duas semanas. Ao exame físico apresentava palidez, febre e lesões crostosas sugestivas de Herpes simplex no mento e coxa esquerda. O hemograma revelou leucocitose (51.000/mm³) com células blásticas (68%), granulócitos (7,3%) e monócitos (22%); as plaquetas somavam 137.000/ mm³ e hemoglobina era de 6,1g/dL. Confirmou-se o diagnóstico de LMA com imunofenótipo CD13 e CD33 positivos. Iniciouse a antibioticoterapia com ceftazidima, penicilina G cristalina e aciclovir. A quimioterapia antineoplásica foi iniciada três dias após a internação com citosina arabinosídio (ARA-C) e 2 clorodeoxi-adenosina (2CDA). No 4° dia pós-internação, a lesão axilar passou a drenar secreção purulenta e, após medidas locais, evoluiu para cicatrização. Ao 5° dia pós-quimioterapia a leucometria era de 1.700/mm³ com ausência de neutrófilos; a febre de até 39,6°C persistia o que determinou a subst...
Lafayette, TCS: Non-Hodgkin's Lymphoma (NHL) associated to Epstein Barr virus (EBV) in children who underwent organ transplantation: characterization of the viral expression and treatment with Anti-CD20 antibodies. Post-transplant lymphoproliferative disease (PTLD) is the most common secondary tissue proliferation that occurs in children after solid organ transplantation and represents a spectrum of clinical lymphoid proliferation and morphologic heterogeneity that goes from an indolent polyclonal hyperplasia to aggressive lymphomas. Approximately 80% of PTLD is associated with Epstein Barr virus (EBV) and is of B-cell origin, 10 to 15% of T-cells and approximately 1% of natural killer cells. EBV pretransplant seronegativity and the degree of immunosuppression are the most relevant risk factors for developing the disease. Clinical presentation is diverse and constitutional symptoms may simulate infection and/or organ transplanted rejection. Histopathologic examination is usually necessary to confirm diagnosis and, generally, in situ hybridization detects the EBV particles in examined tissues. The best treatment option is yet to be determined and the current treatment consists of immunosuppression reduction associated with the use of anti CD20 antibody and/or cytotoxic chemotherapy besides cell therapy only available in some centers. This study aimed to evaluate tumor response to the use of anti CD20 antibody in positive B-cell EBV PTLD after solid organ transplantation and the association of the neoplasia to the eventual inclusion of genomic EBV/DNA in the tumor cell. We retrospectively analyzed medical records of twenty-three patients under 18 years of age who were admitted to the inpatient unit of Serviço de Onco-Hematologia do Instituto da Criança (ICR) e Instituto do Tratamento do Câncer Infantil (ITACI) who developed histologically proven CD20 positive pediatric PTLD after solid organ transplantation between 8 March 1995 and 13 August 2011. All patients were submitted to immunosuppression reduction, thirteen received isolated Anti CD20, three Anti CD20 associated with cytotoxic chemotherapy and seven patients did not use this drug. The estimated 2-year overall survival rates of patients who received anti CD20 was 81.45% and when compared to the overall survival rates of those who did not receive the drug it was 37, 5%, showing a statistically significant difference (p = 0.02). All patients had the Epstein-Barr virus latency protein (latent membrane protein1-LMP1) detected in tumor paraffin embedded stored at diagnosis by the in situ hybridization technic. The short duration of the Anti CD20 treatment, its acceptable toxicity compared to other therapeutic alternatives, the possibility of its exclusive use, its effectiveness in aggressive histology disease and the association with other treatment alternatives in refractory disease, suggest this drug inclusion to the currently available therapeutic arsenal.
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