Enfermedad de Castleman subcutánea: un caso adicional / Castleman’s disease of the subcutis

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Prolonged survival of patients with angioimmunoblastic T-cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation: the GELTAMO experience.

OBJECTIVES: Angioimmunoblastic T-cell lymphoma (AIL) is a rare lymphoma with a poor prognosis and no standard treatment. Here, we report our experiences with 19 patients treated with high-dose chemotherapy and autologous stem cell transplantation (HDC/ASCT) within the GELTAMO co-operative group between 1992 and 2004. METHODS: The median age at transplantation was 46 yr. Fifteen patients underwent the procedure as front-line therapy and four patients as salvage therapy. Most patients received peripheral stem cells (90%) coupled with BEAM or BEAC as conditioning regimen (79%). RESULTS: A 79% of patients achieved complete response, 5% partial response and 16% failed the procedure. After a median follow-up of 25 months, eight patients died (seven of progressive disease and secondary neoplasia), while actuarial overall survival and progression-free survival at 3 yr was 60% and 55%. Prognostic factors associated with a poor outcome included bone marrow involvement, transplantation in refractory disease state, attributing more than one factor of the age-adjusted-International Prognostic Index, Pretransplant peripheral T-cell lymphoma (PTCL) Score or Prognostic Index for PTCL. CONCLUSIONS: More than half of the patients with AIL that display unfavourable prognostic factors at diagnosis or relapse would be expected to be alive and disease-free after 3 yr when treated with HDC/ASCT. Patients who are transplanted in a refractory disease state do not benefit from this procedure.

Genetic abnormalities and clinical outcome in chronic lymphocytic leukemia.

B-cell chronic lymphocytic leukemia (B-CLL) is the most common leukemia in the elderly population. Under conventional cytogenetic (CC) analysis, approximately 50% of CLL cases show clonal aberrations. Using fluorescent in situ hybridization (FISH), the percentage of patients with abnormalities rises to almost 80%, the most frequent being 13q14, ATM, and TP53 deletions and trisomy 12. The aim of this study was to establish the incidence of genetic changes in B-CLL patients using CC and FISH and to evaluate the prognostic implications. Of the 65 patients analyzed, genetic aberrations were found in 36.7% with CC and in 68.4% with FISH. The frequencies of abnormalities were as follows: 13q deletion, 42.1%; trisomy 12, 19.2%; ATM deletion, 17.5%; and TP53 deletion, 8.7%. Significant differences were observed when the overall survival was correlated with Rai stage (P = 0.000). FISH abnormalities were correlated with age, sex, morphology, white blood cell count, CD38 expression, Rai stage, disease status, and survival. Significant differences were obtained with age (P = 0.05) and disease status (P = 0.01). Deletion of 13q was the most frequent abnormality (36.6%) among old patients (> or =60); trisomy 12 was the most frequent (31.3%) in younger patients (<60). Half of the patients with stable disease showed 13q deletion, and the most frequent abnormality in patients with progressive disease was ATM deletion (22.2%).

Diffuse large B-cell lymphomas with plasmablastic differentiation represent a heterogeneous group of disease entities.

Plasmablastic lymphoma was initially described as a variant of diffuse large B-cell lymphoma (DLBCL) involving the oral cavity of HIV+ patients and characterized by immunoblastic morphology and a plasma cell phenotype. However, other lymphomas may exhibit similar morphologic and immunophenotypic features. To determine the significance of plasmablastic differentiation in DLBCL and examine the heterogeneity of lymphomas with these characteristics, we examined 50 DLBCLs with low/absent CD20/CD79a and an immunophenotype indicative of terminal B-cell differentiation (MUM1/CD38/CD138/EMA-positive). We were able to define several distinct subgroups. Twenty-three tumors were classified as plasmablastic lymphoma of the oral mucosa type and showed a monomorphic population of immunoblasts with no or minimal plasmacytic differentiation. Most patients were HIV+ and EBV was positive in 74%. Eleven (48%) cases presented in the oral mucosa, but the remaining presented in other extranodal (39%) or nodal (13%) sites. Sixteen cases were classified as plasmablastic lymphoma with plasmacytic differentiation. These were composed predominantly of immunoblasts and plasmablasts, but in addition exhibited more differentiation to mature plasma cells. Only 33% were HIV+, EBV was detected in 62%, and 44% had nodal presentation. Nine cases, morphologically indistinguishable from the previous group, were secondary extramedullary plasmablastic tumors occurring in patients with prior or synchronous plasma cell neoplasms, classified as multiple myeloma in 7 of the 9. Two additional neoplasms were an HHV-8+ extracavitary variant of primary effusion lymphoma and an ALK+ DLBCL. HHV-8 was examined in 39 additional cases, and was negative in all. In conclusion, DLBCLs with plasmablastic differentiation are a heterogeneous group of neoplasms with different clinicopathological characteristics that may correspond to different entities.

Validez del cuestionario de calidad de vida SF-36 como indicador de resultados de procedimientos médicos y quirúrgicos / Clinical usefunlness of the SF-36 in several clinical problems

Objetivo: Estudiar la utilidad clínica del cuestionario genérico de calidad de vida relacionada con la salud (CVRS) SF-36 en los servicios de asistencia sanitaria. Método: Se llevó a cabo un estudio descriptivo con 691 pacientes con distintos diagnósticos médicos reclutados en el ámbito de consultas externas de un hospital general. Los grupos diagnósticos fueron cáncer de pulmón (CP) y hematológico (CH), osteoartrosis de cadera (OAC), trastornos de la alimentación (TA) y enfermedad inflamatoria intestinal crónica (EIIC). Los pacientes incluidos en el estudio cumplimentaron el SF-36, un instrumento genérico de CVRS. Con los pacientes con OAC que iban a ser operados de prótesis de cadera (PC) fue realizado un estudio de seguimiento antes y después de la intervención. Resultados: Los pacientes con TA presentaban puntuaciones bajas en las áreas mentales, funcionamiento social y rol físico. Aquellos con CP contaban con valores bajos en todas las áreas del SF-36, que eran menores en las físicas y ligeramente más bajas que en los pacientes con CH. Los pacientes con OAC presentaban un impacto importante en las áreas físicas, principalmente en el área de dolor corporal; las mejoras tras la intervención eran mayores en las áreas físicas. Aquellos pacientes con EIIC, evidenciaban afección principal en el área de salud general. Conclusiones: El SF-36 es capaz de discriminar entre distintas enfermedades. Estos resultados apoyan su validez discriminante como un indicador para la evaluación de resultados de procedimientos médicos entre centros y entre grupos de pacientes (AU)