Search

Castleman disease (CD) represents a heterogeneous group of lymphoproliferative disorders that share well-defined histopathological features. An observational study of patients with CD was conducted. A total of 53 patients had CD: 20 had the unicentric form (UCD) and 33 the multicentric (MCD) variant; 10 of the latter cases were infected with human herpesvirus-8 (HHV-8) and 23 were idiopathic (iMCD). Median age differed between UCD and iMCD (30

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.

14 p.-8 fig.-1 tab, Hereditary hemorrhagic telangiectasia (HHT) is a vascular dysplasia characterized by recurrent and spontaneous epistaxis (nose bleeds), telangiectases on skin and mucosa, internal organ arteriovenous malformations, and dominant autosomal inheritance. Mutations in Endoglin and ACVRL1/ALK1, genes mainly expressed in endothelium, are responsible in 90% of the cases for the pathology. These genes are involved in the transforming growth factor-β(TGF-β) signaling pathway. Epistaxis remains as one of the most common symptoms impairing the quality of life of patients, becoming life-threatening in some cases. Different strategies have been used to decrease nose bleeds, among them is antiangiogenesis. The two main angiogenic pathways in endothelial cells depend on vascular endothelial growth factor and fibroblast growth factor (FGF). The present work has used etamsylate, the diethylamine salt of the 2,5-dihydroxybenzene sulfonate anion, also known as dobesilate, as a FGF signaling inhibitor. In endothelial cells, in vitro experiments show that etamsylate acts as an antiangiogenic factor, inhibiting wound healing and matrigel tubulogenesis. Moreover, etamsylate decreases phosphorylation of Akt and ERK1/2. A pilot clinical trial (EudraCT: 2016–003982–24) was performed with 12 HHT patients using a topical spray of etamsylate twice a day for 4 weeks. The epistaxis severity score (HHT-ESS) and other pertinent parameters were registered in the clinical trial. The significant reduction in the ESS scale, together with the lack of significant side effects, allowed the designation of topical etamsylate as a new orphan drug for epistaxis in HHT (EMA/OD/135/18)., The present work was funded by public projects of the Spanish Ministry of Economy and Competitiveness: MINECO SAF 2014 52374 R and SAF2017–83351 to L.M. Botella as the main researcher; PIE-201820E073 CSIC to Lucia Recio, CIBER Rare Diseases ISCIII, Madrid, Spain, funded Virginia Albiñana, and the Spanish HHT association acted as a sponsor of the clinical trial.

Resumen Fundamento y objetivo REACH es el mayor registro mundial disenado para obtener informacion sobre el control de factores de riesgo vascular y tratamiento preventivo en una poblacion con aterotrombosis sintomatica o de riesgo alto para desarrollarla. El objetivo del presente estudio es conocer la tasa de episodios cardiovasculares en la muestra de la poblacion espanola incluida en el registro, tras un ano de seguimiento e intervencion. Material y metodo El registro REACH Espana es un estudio de cohorte prospectivo de sujetos con solo factores de riesgo vascular (SFR) para aterotrombosis o con enfermedad vascular (EV) sintomatica: enfermedad coronaria (EC) y/o cerebrovascular (ECV) y/o arterial periferica (EAP). Las variables principales del estudio fueron la tasa de mortalidad global, cardiovascular y la del conjunto de mortalidad cardiovascular mas infarto de miocardio (IM), mas accidente cerebrovascular, mas hospitalizacion cardiovascular al ano de seguimiento. Resultados Espana incluyo a 2.516 sujetos, de los cuales 2.252 completaron el ano de seguimiento, con una edad media de 68,1 anos (73,8% de varones). El 11,6% presentaban SFR y el 88,5% EV establecida, el 55% EC, el 33% ECV y el 17% EAP. La tasa anual en los grupos EV y SFR fue, respectivamente, para la mortalidad global del 3,57 y el 1,98% (p=NS), para la mortalidad cardiovascular del 2,69% y el 0,62% (p Conclusiones Los datos del registro REACH espanol al ano de seguimiento en sujetos con aterotrombosis establecida o con factores de riesgo para desarrollarla muestran una alta tasa de mortalidad total y de la combinacion de episodios cardiovasculares mayores, que se acentua cuando mayor es el numero de territorios vasculares afectados.

Varon de 84 anos, con antecedentes de hipertension arterial, cardiopatia isquemica cronica y fibrilacion auricular anticoagulada, que consulto por lesion preesternal dolorosa, con exudacion de material seroso, de varios meses de evolucion. En la exploracion presentaba una placa eritematosa sobreelevada de 8 5 cm, de consistencia petrea, localizada en la linea media esternal (fig. 1A). La tomografia computarizada del torax revelo la presencia de una voluminosa masa de partes blandas afectando al esternon, con fractura patologica en su tercio inferior, y extension a planos muscular y cutaneo (fig. 1B). La biopsia de piel demostro proliferacion de celularidad linfoide atipica, con estudio inmunohistoquimico positivo para CD20, CD10, Bcl-6 y MUM-1 (fig. 1C). Estos hallazgos fueron compatibles con el diagnostico de linfoma B de alto grado. Se inicio tratamiento con esquema CHOP-R (ciclofosfamida, vincristina, doxorrubicina, prednisona y rituximab), lograndose la remision completa tras 4 ciclos. Figura 1. www .e lsev ier .es /m ed i c in ac l in ic a

We report two patients with large-cell non-Hodgkin's lymphomas who were treated with intensive chemotherapy (MACOP-B), and in whom large isolated intraparenchymal masses (splenic in one case, renal in the other) were found with computed tomography after clinical remission. It was found at surgery that both masses were constituted by acellular necrotic material, without tumoral infiltration and limited by fibrous tissue. The finding of these residual masses in patients in remission raises problems of approach, as they may be nontumoral and not require new therapy. At present, until new radiological or biochemical markers are available, a judicious clinical attitude should be taken, with radiological control of the evolution of the masses and reserving surgery only for those cases with progression, diagnostic doubts, or patient's decision to know his real status.

A patient with common variable immunodeficiency developed lymphoid nodular hyperplasia and, subsequently, a follicular non-Hodgkin lymphoma with excellent response to chemotherapy. The patient remained in remission after 4 years. The very unusual type of this lymphoma and its localization are discussed, and the possible relations between these different conditions as a single spectrum of B lymphocyte are analyzed.