Long Term Remission of Capillary Leak Syndrome Associated with Monoclonal Gammopathy with Progression to Multiple Myeloma After Autologous Stem Cell Transplantation: a Case Report and Review of the Literature.

BACKGROUND: Clarkson's disease is a very rare entity characterised by acute episodes of systemic oedema and severe hypotension associated with paraproteinaemia. Its classical treatment relies on methylxanthine combined with terbutaline. Although this prophylactic therapy reduces the mortality rate, relapses are frequent. Eighty percent of patients with Clarkson's disease present with monoclonal gammopathy of unknown significance (MGUS). The risk of progression to multiple myeloma is 1% per year. CASE DESCRIPTION: Here, we present a 49-year-old woman who suffered multiple such episodes requiring treatment in the intensive care unit. Treatment with terbutaline and theophylline was ineffective. She was diagnosed with multiple myeloma (MM) 8 years after the first of these acute episodes. Antimyeloma treatment with bortezomib and dexamethasone was started, followed by autologous haemopoietic transplantation, with no further acute episodes since then. CONCLUSION: Our case is, to our knowledge, unique because eradication of MM was followed by complete disappearance of acute episodes of capillary leakage. Our case report is also the first to support the use of bortezomib and dexamethasone in this setting. Furthermore, autologous peripheral blood progenitor cell transplantation consolidated the MM stringent complete remission achieving a very long progression-free survival (> 11 years) of both MM and Clarkson's disease.

Cytomegalovirus antibody levels and mortality among hospitalised elderly patients.

BACKGROUND: The impact of cytomegalovirus infection in elderly subjects remains unclear. This study examined the relationship between humoral immune response to cytomegalovirus (CMV) and all-cause mortality in a cohort of elderly hospitalised patients. METHODS: Data were obtained from a random sample of 715 patients (≥65 years old) admitted for any cause in a third level hospital. Serum IgG antibody against CMV was determined by enzyme-linked immunosorbent (ELISA) assay. RESULTS: A total of 480 deaths occurred in seropositive patients (n = 671) during a follow-up of 7.6 years (mean, 4.6); of which 112 patients died in-hospital or within 30 days after discharge (short-term mortality). For patients with CMV IgG antibody levels in the highest quartile compared with lower quartile, fully adjusted models showed that mortality was 1.40 times (95% CI 1.05-1.86) and 2.20 times (95% CI 1.15-4.21) higher, respectively. The exclusion of patients with cardiovascular disease (angina, myocardial infarction, heart failure, peripheral artery disease, or stroke) increases the risk of long-term (HR 2.22, 95% CI 1.36-3.62) and short-term mortality (OR 3.18, 95% CI 1.40-7.24). CONCLUSIONS: Increased IgG antibody levels against CMV are associated with increased short and long-term mortality in elderly hospitalised patients, especially in patients without cardiovascular disease. Key Messages The outcome of elderly hospitalised patients in relation to CMV is unknown. We demonstrate an association between increased anti-CMV IgG levels and mortality. This association is greater in elderly patients without cardiovascular disease.

Non-diagnostic anti-C. burnetii phase I IgG titres: Should they be discarded in elderly patients?

BACKGROUND: High anti-Coxiella burnetii phase I IgG titres are associated with chronic Q fever; an infectious disease with high mortality. Prognostic significance of lower or medium IgG phase I titres remain uncertain. The aim of this study was to explore this issue in a high-risk population. METHODS: Sero-epidemiological and prospective study of 456 hospitalised patients aged 65 and older (Burgos; Spain). Serum IgG antibody phase I and II were determined by immunofluorescence assay. RESULTS: A lower or medium IgG phase I titres (<1:1024) was observed in 180 (39.4%) patients. Atherosclerotic cardiovascular disease was associated with these titres, but not the traditional risk factors of chronic Q fever (cardiac valve disease, and vascular grafts or valvular prosthesis) (adjusted OR 1.75, 95% CI 1.18-2.61). Lower or medium IgG phase I titres were also associated with decreased survival at 30 months follow-up in patients with atherosclerotic cardiovascular disease (but not in the total sample) after adjusting for others comorbidities: IgG phase I titres≥1:32 (HR 1.77; 95% CI 1.14 4-2.74), ≥1:64 (HR 1.90; 95% CI 1.21-2.99)-3.25), and ≥1:128 (HR 2.00; 95% CI 1.23-3.25). CONCLUSION: Lower or medium IgG phase I titres against C. burnetii, even the lowest, should not be discarded in elderly patients with atherosclerotic cardiovascular disease. Serological follow-up should be recommended in this group of patients.

[Tularemia: study of 27 patients]. / Tularemia: análisis de 27 casos.

BACKGROUND: There has been an important tularemia outbreak recently in Spain, which has prompted a high epidemiological interest. Our objective was to describe the series of tularemia cases with emphasis being paid on clinical characteristics and evolution of the disease. PATIENTS AND METHOD: We describe 27 cases of tularemia studied clinically and epidemiologically (survey of the Health Territorial Service at Burgos). Two cases were seen in December 2000 as isolated forms, while the rest of cases accumulated in form of outbreak between September 1997 and the first months of 1998. We performed a follow-up of patients including a questionnaire (via phone) to assess relapses or disease's consequences and complications. RESULTS: 48% patients were males and patients' mean age was 52 years (27 to 72). 96% patients had handled hares in relation to hunting practices. Main symptoms were weariness (92%), general discomfort (88%), lymph node enlargement (85%) and fever (81%). Skin lesions were seen in 77% patients. A delay in the initial diagnosis was found in 40% cases. Main complications included: lymph node suppuration (25%) and persistent weariness (18%). In patients with and without a diagnostic delay, 62/21% had lymph node enlargement longer than 1 month, 46/7% had adenopathy suppuration (p < 0.05) and 31/7% had persistent weariness, respectively. CONCLUSIONS: Clinical and epidemiological features of tularemia are similar to those described in previous reports. The disease behaved in a uniform and benign form without serious or evolutive complications. Diagnostic delay was the main added morbidity factor.

Tularemia: análisis de 27 casos / Tularemia: study of 27 patients

FUNDAMENTO: Recientemente en nuestro país se ha descrito un importante brote epidémico de tularemia, que ha suscitado un gran interés epidemiológico. El objetivo de este trabajo es presentar nuestra serie incidiendo en las características clínicas y evolución de la enfermedad. PACIENTES Y MÉTODO: Se describen 27 casos de tularemia que fueron estudiados clínica y epidemiológicamente (encuesta del Servicio Territorial de Sanidad de la provincia de Burgos). Dos de los casos ocurrieron en diciembre del año 2000 de forma aislada, mientras que el resto se acumuló con el brote epidémico observado entre septiembre de 1997 y los primeros meses de 1998. Se completó el estudio mediante seguimiento y cuestionario telefónico dirigido a valorar recaídas o secuelas de la enfermedad. RESULTADOS: El 48 por ciento fueron varones, siendo la edad media de los pacientes de 52 años (extremos, 27-72). El 96 por ciento había manipulado liebres en relación con la caza. La clínica principal fue astenia (92 por ciento), malestar general (88 por ciento), presencia de adenopatías (85 por ciento) y fiebre (81 por ciento). Se observaron lesiones cutáneas en el 77 por ciento de los casos. En el 40 por ciento se detectó un retraso diagnóstico inicial. La principales complicaciones descritas fueron: supuración de las adenopatías (25 por ciento) y astenia persistente (18 por ciento). En los pacientes con y sin retraso diagnóstico, un 62 y un 21 por ciento presentaron persistencia de las adenopatías durante más de un mes, un 46 y un 7 por ciento supuración de éstas (p < 0,05) y un 31 y un 7 por ciento astenia persistente, respectivamente. CONCLUSIONES: La características clínicas y epidemiológicas de esta enfermedad son consistentes con la bibliografía previa. La enfermedad se ha comportado de forma uniforme, benigna y sin complicaciones graves o evolutivas de consideración, siendo el retraso diagnóstico el principal factor de morbilidad sobreañadido. (AU)