Compromiso endocrinológico en el síndrome de POEMS: revisión a propósito de un caso / Secondary amenorrhea and hypothyroidism in a women with POEMS syndrome: report of one case

POEMS is an acronym for a syndrome characterized by osteosclerotic myeloma: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Hypothyroidism, hypogonadism, diabetes mellitus and adrenal failure can be associated to POEMS syndrome. We report a 39 years old women with monoclonal gammopathy and paraparesis caused by a polyneuropathy, in whom a POEMS syndrome was diagnosed and treated with dexamethasone. The patient had a secondary amenorrhea and a clinical hypothyroidism with negative antithyroidal antibodies. Substitutionwith levothyroxine was started.

Valor pronóstico del nuevo sistema de etapificación internacional en mieloma múltiple: Comparación con el sistema de Durie-Salmon / Prognostic value of the new international staging system in multiple myeloma: Comparison with Durie-Salmon staging system

Background: Since 1975, the Durie-Salmon staging system (D&S) has been a widely accepted prognostic classification of multiple myeloma (MM) patients. Recently, the new International Staging System (ISS) was developed using only the values of albumin and betaZ-microglobulin. Aim: To compare survival of patients with MM treated in six medical centers in Chile according to the D&S system and the new ISS. Material and methods: Retrospective analysis of demographic information, clinical features and survival rate of patients treated between 1998 and 2002, and grouped according to both systems. Results: Information of 81 patients aged 38 to 90 years (43 women) was retrieved. According D&S system 11 percent were in stage I 12 percent in stage II and 73 percent in stage III According to ISS, 34 percent were in stage I 35 percent in stage II and 31 percent in stage III Median of 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 14 months in stages III 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 II and III of ISS the presence of renal failure was associated with a non significantly different lower survival. Conclusions: The ISS is a simple and effective grouping method for patients with MM, that predicts survival. The presence of renal insufficiency might identify a subgroup of patients included in stages II and III of ISS with a higher mortality.

Mieloma múltiple en Chile: características clínicas y sobrevida / Clinical features and survival of Chilean patients with multiple myeloma

Background: 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 percent had and IgA and 6.1 percent had light chains myeloma. According to Durie and Salmon staging system, 8,2 percent were in Stage 112.6 percent in Stage II, 60.5 percent in Stage III and in 18.8 percent the information about staging was not available. Fifty percent had an hemoglobin level below 10 g/dL, 30 percent had a serum creatinine over 2 mg/dL and 28 percent 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 percent 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.

Linfoma de Hodkin en Chile: Experiencia de 15 años del Programa Nacional de Cáncer del Adulto / Hodgkin lymphoma in Chile: Experience of the national adult cancer program

Background: Hodgkin lymphoma is a highly curable disease. Aim: To evaluate the clinical characteristics and the treatment results of Hodgkin lymphoma patients of the National Cancer Program in Chile. Patients and methods: Prospective assessment of 682 patients treated in 18 adult cancer centers. Progression free survival (PFS) and overall survival (OS) were calculated. Median follow up was 127, 95, 87, 72 and 50 months for C-MOPP, radiotherapy (RT), C-MOPP/ABV, NOVP and ABVD, respectively. Results: Median age was 37 years (15-84). Nodular sclerosis and mixed cellularity were equally expressed. Advanced stages (III & IV) were present at diagnosis in 61 percent of cases. Age over 40 was an adverse prognostic factor (p <0.001). The rate of PFS at 5 and 10 years for early stages was 73 percent and 66 percent with RT, 80 percent and 74 percent with C-MOPP+RT, 73 percent and 71 percent with C-MOPP/ABV, 59 percent and 59 percent with NOVP+RT, and 81 percent with ABVD+RT, at 5 years, being significantly lower for NOVP (p =0.02). The rate of OS at 5 and 10 years for advanced stages was 82 percent and 70 percent with RT, 82 percent and 76 percent with C-MOPP+RT, 82 percent and 80 percent with C-MOPP/ABV, 68 percent and 60 percent with NOVP, and 85 percent with ABVD at 5 years, also significantly lower for NOVP (p =0.04). For advanced stages, the rate of PFS at 5 and 10 years was 49 percent and 43 percent with C-MOPP, 69 percent and 62 percent with C-MOPP/ABVD or C-MOPP/ABV, and 71 percent at 5 years with ABVD, significantly lower for C-MOPP (p =0.01). The rate of OS at 5 and 10 years was 52 percent and 46 percent with C-MOPP, 70 percent and 63 percent with C-MOPP/ABVD or C-MOPP/ABV and 76 percent with ABVD at 5 years, significantly lower for C-MOPP (p =0.0002). Conclusions: Age over 40 years was an adverse prognostic factor. C-MOPP/ABVD, C-MOPP/ABV and ABVD had comparable results and reached a high tumor control and overall survival in both early...

Hipoacusia súbita secundaria a macroglobulinemia de Waldeström / Sudden sensoryneural hearing loss secondary to Waldeström's macroglobulinemia

Se presenta un caso clínico de alteraciones cocleovestibulares súbitas, provocado por Síndrome de Hiperviscosidad, secundario a Macroglobulinemia de Waldeström, analizando datos anamnésicos, de laboratorio, repercusión en otras parénquimas, correlacionándolos con mecanismos fisiopatológicos, respuesta terapéutica y evolución posterior en controles periódicos de 10 años. En la literatura se describe hipoacusia sensorioneural y tinnitus como manifestaciones crónicas de este Síndrome, pero no encontramos descripción de un cuadro de hipoacusia sensorioneural de aparición súbita. Nuestro objetivo es describir un caso clínico y analizar la probable etiología vascular como mecanismo etiopatogénico de la hipoacusia súbita

Tratamiento de la enfermedad de Hodgkin: protocolo nacional de drogas antineoplásicas: informe preliminar / Treatment of Hodgkin's disease: National Protocol on Antineoplastic Drugs: preliminary report

We report preliminary results of treatment of Hodgkin disease according to the National Protocol on Antineoplastic Drugs. 37 males and 22 females with a median age of 34 years (range 16 to 76) were treated. Patients with stages I or II received radiotherapy alone or chemotherapy (C-MOPP) followed by radiotherapy. Patients with stages III or IV received radiotherapy and chemotherapy or alternating courses of D-MOPP and ABVD. Complete remission was observed in 74% of 39 patients completing full therapy. Complete remission was more common in patients with nodular sclerosis without B symptoms and under 45 years of age. Actuarial survival at 22 months was 79%, significantly higher for patients with nodular sclerosis compared to patients with lymphocytic depletion. Different treatments in patients at stages I an II or III and IV gave comparable results. Complications included infection with the Chickenpox-Zoster virus, pneumonia and fever of Unknown origin. Mortality was associated to older age, complications of treatment or failure to comply with therapy