Relapse remains the main cause of treatment failure in patients with acute myelogenous leukemia (AML) after allogeneic hemopoietic stem cell transplantation (SCT). The Wilms' tumor 1 gene (WT1) is reportedly overexpressed in >90% of patients with AML and thus can be useful for minimal residual disease (MRD) monitoring. The aim of this study was to evaluate the usefulness of WT1 expression as a relapse predictor marker in patients with AML after SCT and compare it with flow cytometry (FC) and chimerism studies. WT1 expression was assessed retrospectively using quantitative RT-PCR in bone marrow and peripheral blood from 21 patients. Patients were classified according to WT1 dynamics posttransplantation. Eleven of the 21 patients had low and stable WT1 levels. All of these 11 patients showed complete chimerism and negative MRD by FC and remained in complete remission with a median follow-up of 27 months (range, 18-98 months). In contrast, 10 of 21 patients showed WT1 overexpression after SCT, and 9 of these 10 patients relapsed. The incidence of relapse differed significantly between the 2 groups of patients according to WT1 expression post-SCT (P = .00003). Relapse in the 9 patients occurred at a median of 314 days (range, 50-560 days). Interestingly, in these patients, relapse was first predicted by WT1 (with negative FC and complete chimerism) in 7 patients. WT1 overexpression was correlated with disease burden in patients with AML before and after allogeneic SCT. In patients who relapsed, both medullary and extramedullary relapse were better anticipated by WT1 overexpression compared with FC and chimerism.

Umbilical cord blood (CB) is increasingly used as an alternative source of stem cells in adult unrelated transplantation. Although registry studies report similar overall outcomes in comparison with BM/PB, comparative studies focusing on severe infections and infection-RM (IRM) with a long follow-up are scarce. A total of 434 consecutive unrelated transplants (1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009) were retrospectively analyzed to compare overall outcomes, incidence and risk factors of severe viral and invasive fungal infections in CB (n = 65) vs BM/PB recipients (n = 369). The 5-year OS was 38 vs 43%, respectively (P = 0.2). CB transplantation (CBT) was associated with a higher risk of invasive aspergillosis (100-days-cumulative incidence 16 vs 6%, P = 0.04) and CMV infection without differences in RM. No statistically significant differences were found regarding NRM (NRM of 38% in CB vs 37% in BM/PB at 1 year) nor IRM (30% in CB vs 27% in BM/PB at 1 year). In the overall population, NRM and IRM improved in more recent years. In adults who receive a single CBT, the risk of severe infections is increased when compared with unrelated BM/PB recipients, but mortality from infections is similar, leading to similar NRM and survival. INTRODUCTIONInfections are a major concern after allo-HSCT, and the main cause of mortality in at least 35-45% of deaths.1-7 The use of unrelated donors (UD) in adults increases the risk of severe infectious complications, although recent reports have found similar survival with matched sibling and UD allo-HSCT in the setting of specific diseases, such as AML. 8,9 Moreover, the use of cord blood (CB) as an alternative source of HSC in adults who lack an HLA-matched UD BM or PB has shown to be a valid alternative for an allo-HSCT. Recent studies have shown that similar NRM and OS can be achieved with CB if an accurate choice of the CB unit(s) is made, in comparison with other alternative donor sources.10-15 However, slow hematologic and immune recovery after CB transplantation (CBT) still remains a concern, and a high rate of serious pre and post-engraftment infections has been reported. However, few studies have actually compared the incidence of severe infections and infection-RM (IRM) of adult CBT recipients as opposed to UD BMT/PBT.For this purpose, and as a continuation of a previously published study by our group, 16 we performed a multicenter 12-year retrospective study to analyze the impact of the source of HSC for UD transplantation, including single CBT and BM/PBT on overall transplantation outcomes, especially. We also analyzed the

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4676 INTRODUCTION: Bernard Soulier Syndrome (BSS) is a rare qualitative and quantitative autosomal recessive platelet disorder molecularly characterized by defective GPIb-IX-V membrane glycoprotein that is related to platelet adhesion and interaction with both Von Willebrand factor and endothelium. It is usually expressed by an enlarged bleeding time and decreased yet giant sized platelets, and can cause severe hemorrhagic complications during pregnancy. CASE REPORT: We present two successful pregnancies and labour management on a 33-year-old woman diagnosed in her childhood with BSS. Ristocetin induced platelet agglutination test and membrane glycoprotein study were performed to get the diagnosis. Furthermore, in the last year we have completed the diagnosis with platelet receptor expression flow citometry analysis, finding out depleted antiGPIbα and antiGPIX antibodies. Changes which prevent GPIbα for correct expression and processing were observed. Also integrin αIIbβ3 levels were risen due to the big sized platelets. A PCR product was obtained from genomic DNA using the GPIbα oligonucleotide pair; sense and antisense, and direct DNA sequencing of the amplification was performed in a model ABIprism 377 DNA sequencer (Perkin-Elmer Cetus). The direct sequencing of the sense strand of the 5x fragment shows in the patient the simultaneous presence of G and C nucleotids in 688 position, and the T and A nucleotids in 715 position. These punctual mutations give Ala200Pro substitutions and Cys209Ser in heterozygosis in mature peptide, respectively, Figure 1. The Ala200Pro mutation has not been reported in others cases of Bernard-Soullier, previously. The Cys209Ser has been described in other two Spanish patients: one in homozygosis and the other in heterozygosis associated to the mutation. Our patient presented hemorrhagic symptoms from 18 month old with an average of 40×103/μ l platelet count and increased platelet volume who required multiple platelet transfusions during her childhood. In the menarche main hemorrhagic manifestations consisted on methrorragia and epistaxis which were controlled with tranexamic acid. At the age of 26 a spontaneous ovarian cyst rupture required urgent hospital admission and two platelet pool transfusion. On December 2007 she consulted for epistaxis, and on September 2008 cesarean delivery was planned at 38th week of pregnancy; platelet count was 40×103/μ l, antiplatelet antibodies were negative for membrane glycoproteins and antiHLA-1 was positive against 98% donors tested. As a result, an HLA-compatible donor was searched, and two platelet apheresis were transfused one hour before surgery and every 12 hours after the procedure. The next 4 days one apheresis every 48 hours were transfused with no hemorrhagic symptoms. On January 2010, she presented with her second pregnancy; the patient had severe anemia, with high transfusion requirements; neither hemorrhage or hemolysis was detected, and even the possibility of a Munchausen syndrome was questioned. Unplanned caesarean was performed at 32th week because of the clinical instability of the patient, and this time we could not find on time an HLA-compatible donor, so two random platelet pools were transfused one hour before surgery and one platelet apheresis every day during 4 days after caesarean deliver, without any hemorrhagic complication. The newborn was asymptomatic with normal platelet count. The patientxs haemoglobin level returned to normal after five days of delivery. CONCLUSIONS: We present the successful labour management of two consecutive pregnancies on a young woman diagnosed with BSS. The low prevalence of this syndrome (less than one case per million population) explains the absence of well-established protocols for the management of pregnancy in this disease. Up to date there are only twelve reported cases of pregnancy and delivery in BSS patients, most of them presenting with severe hemorrhagic complications. Correct platelet transfusion is the mainstay of treatment, and screening for anti-platelet antibodies study is mandatory due to its high prevalence in these patients. Disclosures: No relevant conflicts of interest to declare.

ResuMenEl Agdal es una finca real situada al sur de Marrakech y anexa a la kasba, fundada en época almohade. El perí-metro amurallado actual contiene 340 ha que en su mayor parte son huertas que se han mantenido cultivadas ininterrumpidamente. En su interior se han estudiado más de 40 edificios conservados y numerosos restos arqueológicos; los más significativos han permitido identificar el recinto de Dar al-Hana como el área residencial de la finca almohade y saadí. Al sur de este recinto se han localizado los restos de un gran palacio saadí de patio central, edificado sobre otro más antiguo de época almohade. La prospección arqueológica origen de la presente investigación ha documentado el proceso de expansión del Agdal hacia el norte, que acabó transformando lo que era una finca aislada (una almunia) en un apéndice de la kasba. La prospección arqueológica de la llanura de Tasltante, inmediata al Agdal, ha permitido definir un modelo de finca y un patrón de implantación al que pertenece también esta finca real. Esta información arqueológica y el análisis exhaustivo de las fuentes cronísticas han hecho posible el primer intento de reconstrucción del Agdal y del paisaje periurbano, con anterioridad a la restauración alauí de la capitalidad imperial en Marrakech en el siglo XVIII.Palabras clave: Agdal, Menara; almunia; jattara; jardines islámicos; arquitectura palatina; arquitectura almohade; arquitectura saadí; arquitectura alauí. AbstRActThe Agdal is a royal country estate located to the South of Marrakech and annexed to the kasba, founded in the Almohad period. The current enclosure is an area of 340 ha and consists mainly of orchards farmed uninterruptedly for centuries. Over 40 preserved buildings and numerous archaeological remains have been catalogued in its interior. Among these remains, the enclosure of the Dar al-Hana has been identified with the residential area of the Almohad and Saadian periods. In the southern area of this complex, the remains of a large central court Saadian palace, built on a previous Almohad one, have been identified. The enlargement of the Agdal northwards, which converted an isolated country estate (an almunia) into an appendix of the kasba, has been also recorded. The archeological prospection of the Tasltante plain, close to the Agdal, has allowed defining a model of country state and a settlement pattern, which is also followed by this royal property. The archaeological information and the analysis of the written sources have made possible to reconstruct the Agdal and the peri-urban landscape previous to the Alaouite restoration of the imperial capital status to Marrakech in the eighteenth century.