María Elena abrera C

Valor pronóstico del nuevo sistema de etapificación internacional en mieloma múltiple. Comparación con el sistema de Durie-Salmon

Information of 81 patients aged 38 to90 years (43 women) was retrieved. According D&S system 11% were in stage I, 12% in stage II and73% in stage III. According to ISS, 34% were in stage I, 35% in stage II and 31% in stage III. Medianof survival of all patients was 32 months. Both staging systems had a prognostic value. However,median survival for the three stages of the ISS system was significantly different (67, 29 and 14months in stages I, II and III, respectively, p =0.02). Patients in advanced stages II and III of the ISS,had a higher frequency of anemia, hypercalcemia, renal failure and hypoalbuminemia. In stages IIand III of ISS the presence of renal failure was associated with a non significantly different lowersurvival.

Frecuencia de los genes de fusión TEL/AML1 y BCR/ABL en pacientes pediátricos con leucemia linfoblástica aguda

TEL/AML 1 and BCR/ABL fusion gene transcriptswere detected in 13 (23%) and 2 (4%) children, respectively. No differences in survival wereobserved between children with positive or negative transcripts for TEL/AML1 fusion gene. However,those positive for BCR/ABL fusion gene, had a significantly lower survival.

Mieloma múltiple en Chile: Características clínicas y sobrevida

Mortality rate records are the only data available in Chile about the prognosis of patients with multiple myeloma (MM). Aim To characterize clinical features, survival rate and factors related to mortality in cases with MM treated in six large medical centers in Chile. Material and Method: Retrospective analysis of demographic data, clinical features and survival rate records of patients with MM, collected between 1998 and 2002. Survival curves were generated and a multivariate analysis of factors associated to early mortality was carried out. Results: Data from 245patients aged 38 to 95years (129 women) was collected. Fifty two percent had an IgG myeloma, 25% had and IgA and 6.1% had light chains myeloma. According to Durie and Salmon staging system, 8,2% were in Stage 112.6% in Stage II, 60.5% in Stage III and in 18.8% the information about staging was not available. Fifty percent had an hemoglobin level below 10 g/dL, 30% had a serum creatinine over 2 mg/dL and 28% had a serum calcium level over 10.5 mg/dL. Median survival was 33 months. Twenty percent of patients died within the first six months after diagnosis (early mortality). Predictive factors for early mortality were male sex, thrombocytopenia, anemia, renal failure, hypercalcemia, a beta2-microglobulin >5.5 mg/L and a serum albumin level <3.5 g/dL. There was a correlation between the number of bad prognosis factors present and the probability of early mortality. Conclusions: This group of Chilean patients with MM presented a short survival time, and 20% died within the first six months after diagnosis. More than a half of cases were diagnosed at an advanced stage (Durie and Salmon Stage III). Several factors were associated to early mortality, two of which (beta 2-microglobulin and serum albumin), are included in the new International Staging System for MM

Síndromes linfoproliferativos crónicos en Chile: Estudio prospectivo de 132 casos

Background: Chronic lymphoproliferative disorders include a variety of diseases which are often a diagnostic problem for clinical hematologists. Aim: To study prospectively the distribution and incidence of chronic lymphoproliferative disorders in Chile and compare them with those of other Western, Latin American and Oriental countries. Patients and methods: A group of 132 patients were studied in a 36 months period (1999-2001), with a panel of monoclonal antibodies. A score for chronic lymphocytic leukemia was employed to differentiate it from other B-cell disorders. Results: The median age was 63 years old (range 32-94). Most patients had B-cell tumors (109) and the rest (23), T-cell tumors (82% vs 18%). Forty five percent of patients with B-cell tumors had a chronic lymphocytic leukemia (CLL), while the others were disseminated lymphomas. The incidence of T-cell tumors was slightly higher than that of other Western countries. Noteworthy is that the most common of these disorders was adult T cell leukemia/lymphoma (ATLL), in concordance with the high HTLV-1 seroprevalence in Chile. Conclusions: A morphologic, immunophenotypic and pathological study in a large number of patients with chronic lymphoproliferative disorders in Chile, shows a relatively low incidence of CLL when compared to other chronic B-cell tumors and a high representation of ATLL associated to HTLV-1 infection, compared with other Western countries. The lower incidence of CLL in our study might be due to patients selection and/or underdiagnosis of this disease as a substantial proportion of CLL are asymptomatic (Rev Med Chile 2003; 131: 291-8).

Mejoría en el pronóstico de la leucemia linfoblástica aguda en niños de un país en desarrollo: Resultados del protocolo nacional chileno PINDA 87

El grupo oncologico pediatrico nacional, PINDA, reporta el primer protocolo prospectivo, no randomizado, para tratamiento de la leucemia linfoblastica (LLA), usando una version modificada del protocolo de Berlin-Frankfurt-Munster (LLA BFM 86). Los objetivos de este estudio fueron clasificar inmunofenotipos, disminuir radioterapia de craneo y comprobar si este protocolo podia mejorar la sobrevida de nuestros pacientes. Procedimiento: Desde junio 1987 a junio 1992 se registraron 444 pacientes, no seleccionados; de ellos 425 fueron evaluables. La terapia fue estratificada segun riesgo: Riesgo Bajo (RB), Riesgo Alto (RA) y Riesgo Muy Alto (RMA). Los pacientes en RB y RA recibieron induccion con protocolo I, consolidacion con protocolo M (RMA uso protocolo E), reinduccion con protocolo II y mantencion. Todos recibieron tratamiento de prefase con prednisona oral y metotrexato (MTX) intratecal. Radioterapia de craneo solo en RA y RMA (12-18 Gy). Los siguientes cambios se introdujeron al protocolo LLA BFM 86: en protocolo M 1 g/m2 en vez de 5 g/m2; en protocolo E, 1 g/m2 de citarabina en vez de 2 g/m2, la mitoxantrona e ifosfamida fueron sustituidas por teniposido y ciclofosfamida. Resultados: Inmunofenotipo: LLA comun 67,4%, LLA proB 14%, LLA T 10%, LLA preB 4,3%, indiferenciada 4,3%. La frecuencia de sobrevida libre de eventos (SLE) global a 5 anos fue 60% p 2% error standard; segun riesgo fue: RB 75%, RA 62%, RMA 28% con una mediana de seguimiento de 6,5 anos (rango 4,5 - 9,5 anos). La incidencia acumulada de recaida en sistema nervioso central SNC fue 5,4%. Conclusion: Hemos tenido exito en realizar un estudio a nivel nacional. Nuestra estrategia para adaptar el protocolo BFM fue efectiva para mejorar la SLE. La distribucion por fenotipos es similar a otras series

Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile

Clinical significance and frequency of the 11q23/MLL geneticmolecular alteration in Chilean infants with acute leukemia. Background Acute leukemia (AL) ill infants generally shows distinctive biologic features and has a poor prognosis. Aint: To Study the frequency of the cytogenetic alteration of 11q23 chromosome or the recombination of MLL gene ill infants less than 18 months old, with acute leukemia. Patients and methods. We analyzed 37 cases of AL ill infants less than 18 months of age diagnosed ill Chile from 1989 to 1999. The clinical features and cytogenetic/molecular defects of 11q23MLL gene rearrangement and their influence ill prognosis were determined. Results: There were 18 cases of acute Lymphoblastic leukemia (ALL) characterized by female Sex (67%) high presenting leukocyte count (median 99 x109/L), blast cells with a CD10 negative phenotype (50%) and 11q23/MLL rearrangement (39%). Molecular abnormalities Of 11q23 were significantly associated with adverse prognosis, with all event free survival (HF) of only 14 +/- 12%. Interestingly, infants with germ line 11q23 had a very good outcome with all EFS of 73 +/- 11% (P <0.025). There were 19 cases of acute myeloblastic leukemia (AML) characterized ky mate sex (63%) high leukocyte count (median 93 x 109/L), FAB-MS morphology (53%) and 11q23/MLL rearrangement (53%) EFS was very pool; 20 +/-9% and 33 +/-4% for rearranged and germinal group respectively (p=NS), due to a high mortality rate during the first month of diagnosis. Conclusions: These findings demonstrate that Chilean ALL infants with 11q23 abnormalities have a very poor Prognosis, However those with germinal state can enjoy a prolonged disease free survival with the current treatment protocols (Rev Mid Chile2001; 129: 634-642).

Neutropenia febril de alto riesgo en leucemia aguda. Experiencia en un hospital público: Programa Nacional de Drogas Antineoplásicas del Adulto (PANDA), Hospital del Salvador, 1991-2001

Febrile neutropenia is one of the most important problems to face during the treatment of acute leukemia. Aim: To assess the results of a standardized protocol for the treatment of febrile neutropenia and compare it with a period in which treatment was not standardized. Patients and methods: One hundred and eight episodes of febrile neutropenia in 69 patients, treated with a standardized antimicrobial protocol between 1996 and 2001, were analyzed. The protocol consisted in the use of a combination of antimicrobial whose spectrum was broadened progressively according to the isolated microorganisms and the involved foci. These were compared with 83 episodes in 54 patients, treated without standardized protocols between 1990 and 1995. Results: Both groups of patients were comparable. Their ages ranged from 15 to 65 years old. The male/female ratio was 1.3 and the lymphoblastic/myeloid leukemia ratio was 1.4. Sixty one percent of episodes occurred during induction chemotherapy and mean duration of neutropenia was 17 days. A clinically significant focus was identified in 72% of episodes and a microorganism was isolated blood culture in 35% of them. There was a predominance of gram negative organisms. The mortality decreased from 18 to 9% in the period 1996-2000 (p=0.094). Conclusions: The use of a standardized antimicrobial protocol reduced the mortality in febrile neutropenia, even when colony stimulating factors and filtered air rooms are unavailable

Miastenia gravis y su asociación con trastornos linfoproliferativos: casos clínicos

Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile. During a three-year period we identified four patients who developed different lymphoproliferative disorders: two with B-cell lymphoma, one with chronic lymphocytic leukaemia and one plasmacytoma with an associated amyloidosis. The MG was generalized but mild, all cases classified as type IIa according to the definition proposed by the MG Foundation of America. The neoplasia appeared two to 36 years after the onset of MG. These cases provide additional evidence of the association between MG and lymphoproliferative disorders.

Resultados del tratamiento del cáncer de mama, Programa Nacional de Cáncer del Adulto, Región Metropolitana. Ministerio de Salud de Chile. 2000-2010.

Background: Breast cancer is the most common malignant tumor in women in the world. In 2005, it was incorporated to the Explicit Guaranties Health System (GES) in Chile. Aim: To describe the demographic and clinical characteristics of breast cancer patients and to determine the effect of incorporating these women to GES. Material and Methods: Medical records of 5119 women with breast cancer aged 59 ± 14 years, attended at six public hospitals between 2000 and 2010 were reviewed. Median follow up was 87 months (range 1-182). Mortality was assessed using death certificates obtained at the National Identification Registry. Results: Sixty six percent of women were in stage I-II, 29% in stage III and 5% in stage IV. Surgery was performed in 4023/5119 cases (79%), adyuvant radiotherapy in 3627/4517 cases (80%), chemotherapy in 3204/3424 cases (94%) and hormone therapy in 1.695/2.375 cases (71%). Between 2000 and 2010, there was a significant increase in the proportion of cases in stage I, from 8% to 25%, (p<0.01). Overall survival (OS) increased 1% per year, since the beginning of GES system (p=0.024). Five year OS was 75.1%. The figures for Stage I, II, III and IV were 93, 84, 62 and 27% respectively (p<0.01). Patients without lymph node involvement and who were not triple negative, had significant better OS. Conclusions: There was a significant increase in stage I cases, and a 1% per year OS improvement after GES system started, compared with the previous period.Background Breast cancer is the most common malignant tumor in women in the world. In 2005, it was incorporated to the Explicit Guaranties Health System (GES) in Chile. Aim To describe the demographic and clinical characteristics of breast cancer patients and to determine the effect of incorporating these women to GES. Material and Methods Medical records of 5,119 women with breast cancer aged 59 ± 14 years, attended at six public hospitals between 2000 and 2010 were reviewed. Median follow up was 87 months (range 1-182). Mortality was assessed using death certificates obtained at the National Identification Registry. Results Sixty six percent of women were in stage I-II, 29% in stage III and 5% in stage IV. Surgery was performed in 4023/5119 cases (79%), adyuvant radiotherapy in 3627/4517 cases (80%), chemotherapy in 3,204/3,424 cases (94%) and hormone therapy in 1,695/2,375 cases (71%). Between 2000 and 2010, there was a significant increase in the proportion of cases in stage I, from 8% to 25%, (p < 0.01). Overall survival (OS) increased 1% per year, since the beginning of GES system (p = 0.024). Five year OS was 75.1%. The figures for Stage I, II, III and IV were 93, 84, 62 and 27% respectively (p < 0.01). Patients without lymph node involvement and who were not triple negative, had a significantly better OS. Conclusions There was a significant increase in stage I cases, and a 1% per year OS improvement after GES system started, compared with the previous period.

Lesiones cutáneas en mieloma múltiple: Descripción de un caso y revisión de la literatura

Cutaneous involvement in multiple myeloma (MM) is extremely rare. It may correspond to plasmocytomas, secondary amyloidosis, paraneoplastic syndromes or drug reactions. We report a 75 years old female with skin lesions, corresponding to extramedullary plasmacytomas (EPs), as a manifestation of MM progression. Most EPs appear in the upper airway and gastrointestinal tract, but have also been described in the genitourinary tract, lung, kidney, thyroid and in 2% of cases, in the skin. Cutaneous lesions are generally associated with terminal stages of the disease and a poor prognosis.