RESUMEN
Hepatosplenic T-cell lymphoma (HSTL) is an aggressive lymphoma. In post-transplant immunosuppressed patients, HSTL is usually rapidly fatal. We report successful treatment of post-transplant HSTL in a 50-year-old renal allograft recipient by reducing immunosuppression and using intensive chemotherapy consisting of alternating cycles of HyperCVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and MTX/HiDAC (methotrexate, Ara-C). Remission is ongoing at 8+ years. Literature review identified another 20 cases of HSTL in solid organ transplant recipients: median survival was 4 months; no other patients survived beyond 12 months. Bone marrow involvement was universal, but changes were often subtle: 6 of 12 cases had nondiagnostic examinations earlier on. High index of suspicion may lead to more timely diagnosis of this uncommon form of post-transplant lymphoproliferative disorder, and treatment with intensive chemotherapy such as HyperCVAD may be curative.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Hepáticas/tratamiento farmacológico , Linfoma de Células T/tratamiento farmacológico , Neoplasias Primarias Secundarias/tratamiento farmacológico , Complicaciones Posoperatorias/tratamiento farmacológico , Neoplasias del Bazo/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Médula Ósea/patología , Carcinoma de Células Transicionales/cirugía , Ciclofosfamida/administración & dosificación , Citarabina/administración & dosificación , Dexametasona/administración & dosificación , Doxorrubicina/administración & dosificación , Femenino , Humanos , Terapia de Inmunosupresión/efectos adversos , Trasplante de Riñón , Neoplasias Hepáticas/etiología , Neoplasias Hepáticas/patología , Linfoma de Células T/etiología , Linfoma de Células T/patología , Metotrexato/administración & dosificación , Persona de Mediana Edad , Neoplasias Primarias Secundarias/etiología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/patología , Receptores de Antígenos de Linfocitos T gamma-delta/análisis , Inducción de Remisión , Neoplasias del Bazo/etiología , Neoplasias del Bazo/patología , Neoplasias de la Vejiga Urinaria/cirugía , Reflujo Vesicoureteral/cirugía , Vincristina/administración & dosificaciónRESUMEN
AIMS: Our objective was to establish a multiplexed assay using the Biomed 1 primers to detect AML1-ETO transcripts and 10 different CBFB-MYH11 transcripts, using BCR and ABL transcripts as controls. METHODS: Control genes were systematically tested for characteristics of optimal controls. The final assay was validated on 50 AML patient samples. RESULTS: Testing confirmed that the designated control gene criteria were fulfilled. Of 50 patient samples tested, four RT-PCR results were discordant with the cytogenetic result. In three cytogenetically negative cases, RT-PCR detected cryptic CBF rearrangements (one AML1-ETO and two CBFB-MYH11). The fourth case was inv(16) positive but negative by RT-PCR; however, the control gene result revealed suboptimal RNA quality. CONCLUSIONS: We have described a robust multiplex RT-PCR assay that incorporates experimentally validated control genes that are important for accurate interpretation. The assay is more sensitive than cytogenetics in the detection of CBF AML. Application to large patient cohorts will determine the prognostic significance of cryptic CBF rearrangements compared with their cytogenetic counterparts.
Asunto(s)
Leucemia Mieloide/diagnóstico , Leucemia Mieloide/genética , Tamizaje Masivo/métodos , Proteínas de Neoplasias/genética , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa/métodos , Factores de Transcripción/genética , Enfermedad Aguda , Subunidad alfa 2 del Factor de Unión al Sitio Principal , Factores de Unión al Sitio Principal , Análisis Citogenético , Reordenamiento Génico , Humanos , Proteínas de Fusión Oncogénica/genética , Proteínas de Fusión Oncogénica/aislamiento & purificación , Proteína 1 Compañera de Translocación de RUNX1 , Sensibilidad y Especificidad , Factores de Transcripción/aislamiento & purificaciónRESUMEN
This report discusses the case of a 52 year old female with post-transplant lymphoproliferative disorder, confined to the central nervous system, which was managed with high dose methotrexate (HDMTX) in the context of end stage renal disease. The patient received two doses of HDMTX followed by extended hours high-flux hemodialysis, plasma methotrexate concentration monitoring and leucovorin rescue. The hemodialysis technique used was effective in clearing plasma methotrexate and allowed delivery of HDMTX to achieve complete remission with limited and reversible direct methotrexate-related toxicity. Dialysis-dependent renal failure does not preclude the use of HDMTX when required for curative therapy of malignancy.