SINUSOIDAL OBSTRUCTION SYNDROME/VENO-OCCLUSIVE DISEASE AFTER UNMANIPULATED HAPLOIDENTICAL HSCT WITH POST-TRANSPLANT CYCLOPHOSPHAMIDE: A STUDY ON BEHALF OF THE SPANISH HEMATOPOIETIC STEM CELL TRANSPLANTATION AND CELLULAR THERAPY GROUP (GETH).

BACKGROUND: Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life threatening complication after both autologous and allogeneic hematopoietic stem cell transplantation (HSCT). However, its characterization after haploidentical HSCT (haplo-HSCT) with post-transplant cyclophosphamide (PT-Cy) is scarce. OBJECTIVES: To describe characteristics and outcomes of patients with SOS/VOD after haplo-HSCT with PT-Cy. STUDY DESIGN: We conducted a retrospective study of 797 patients undergoing a haplo-HSCT with PT-Cy between 2007 and 2019 in 9 centers in Spain. SOS/VOD was defined according to modified Seattle, Baltimore or revised EBMT criteria. Severity was retrospectively graded according to revised EBMT severity criteria into 4 categories: mild, moderate, severe and very severe. RESULTS: From 797 haplo-HSCT performed, 46 patients (5.77%) were diagnosed from SOS/VOD at a median of 19 days (range 4-84) after transplant. Based on revised EBMT severity criteria, there were 4 mild (8.7%), 10 moderate (21.7%), 12 severe (26.1%) and 20 very severe (43.5%) grade SOS/VOD cases. Overall, 30 patients (65%) achieved SOS/VOD complete response, 25 (83%) of whom were treated with defibrotide. Twenty patients (43%) died before day 100 after HSCT. Death was attributed to SOS/VOD in 11 patients, and 5 patients died of other causes without resolution of SOS/VOD. CONCLUSIONS: Incidence of SOS/VOD after haplo-HSCT with PT-Cy was comparable to those reported after HLA-identical HSCT series. Most of the patients developed very severe SOS/VOD according to revised EBMT severity criteria. In spite of a promising SOS/VOD CR rate (65%), 100-day mortality remained high (43%), indicating that further improvement in the management of this potentially fatal complication is needed.

Pseudothrombocytopenia by ethylenediaminetetraacetic acid can jeopardize patient safety - report.

Pseudothrombocytopenia by ethylenediaminetetraacetic acid (EDTA) is an infrequent phenomenon of in vitro platelet agglutination due to the presence of antiplatelet autoantibodies. It has no clinical significance, but misdiagnosis may lead to clinical or therapeutic decision-making. In this study we report a case of an 8-year-old boy with no history of platelet disorder presenting a low platelet count and a peripheral blood smear showing clumping of platelets by EDTA. The initial diagnosis hypothesis was of an idiopathic thrombocytopenic purpura, and an unnecessary bone marrow aspirate was made even though he did not have personal or family history of bleeding. A second sample collected in sodium citrate confirmed the pseudothrombocytopenia by EDTA. In conclusion, the laboratory should enhance a strong relationship with clinicians trying to avoid misunderstandings as that reflected in this case report. It should be reminded that, in those cases where a pseudothrombocytopenia by EDTA is suspected, a blood smear is mandatory to confirm platelet clumps and blood must be tested anticoagulated with another anticoagulant (i.e., sodium citrate or heparin).

Detection of pancreatic neuroendocrine tumors: 23 years of experience. / Detección de tumores neuroendocrinos pancreáticos: 23 años de experiencia.

INTRODUCTION AND AIMS: Neuroendocrine tumors are of great scientific interest, given that they are difficult to diagnose and treat. Despite being relatively rare (< 1/100,000 individuals, 1-2% of the gastrointestinal neoplasias) and indolent, their potential malignancy must not be forgotten. An increase in the number of diagnosed tumors has been observed in recent years. The aim of the present study was to update a published case series of 19 patients suspected of presenting with pancreatic neuroendocrine tumor with 51 current cases, to study and compare the new results with those of the previous case series, as well as with other recent publications from Spain, the United States, China, and India. MATERIALS AND METHODS: A retrospective, multicenter case series was conducted on 70 patients (19 cases published in 2011), whose data has been collected over a period of 23 years. The variables analyzed were: age, sex, symptomatology, tumor size, location, metastasis, final diagnosis, and surgery, among others. RESULTS: Mean patient age was 55 years and 60% of the patients were men. Disease location was the pancreatic head in 28.5% of the patients and the tail in 27.1%, mean tumor size was 3.9cm (0.2-10cm), 71.4% of the patients had non-functioning tumors, 32.8% had metastases (100% to the liver), 74.2% of the patients were operated on, and actuarial survival was 75%. CONCLUSIONS: Differences were observed between the previously published case series and the current results. There was an increase in incidentalomas and non-functioning tumors, but no variation in the overall survival rate. The differences with other case series (age, sex, and tumor location) were dependent on the country where the cases were compiled. The increase in tumors could be related to a higher number of diagnoses made through imaging studies and to the greater sensitivity of the devices employed.

Evaluation of Elecsys Syphilis Assay for Routine and Blood Screening and Detection of Early Infection.

Treponema pallidum infections can have severe complications if not diagnosed and treated at an early stage. Screening and diagnosis of syphilis require assays with high specificity and sensitivity. The Elecsys Syphilis assay is an automated treponemal immunoassay for the detection of antibodies against T. pallidum The performance of this assay was investigated previously in a multicenter study. The current study expands on that evaluation in a variety of diagnostic settings and patient populations, at seven independent laboratories. The samples included routine diagnostic samples, blood donation samples, samples from patients with confirmed HIV infections, samples from living organ or bone marrow donors, and banked samples, including samples previously confirmed as syphilis positive. This study also investigated the seroconversion sensitivity of the assay. With a total of 1,965 syphilis-negative routine diagnostic samples and 5,792 syphilis-negative samples collected from blood donations, the Elecsys Syphilis assay had specificity values of 99.85% and 99.86%, respectively. With 333 samples previously identified as syphilis positive, the sensitivity was 100% regardless of disease stage. The assay also showed 100% sensitivity and specificity with samples from 69 patients coinfected with HIV. The Elecsys Syphilis assay detected infection in the same bleed or earlier, compared with comparator assays, in a set of sequential samples from a patient with primary syphilis. In archived serial blood samples collected from 14 patients with direct diagnoses of primary syphilis, the Elecsys Syphilis assay detected T. pallidum antibodies for 3 patients for whom antibodies were not detected with the Architect Syphilis TP assay, indicating a trend for earlier detection of infection, which may have the potential to shorten the time between infection and reactive screening test results.

Increased lung neutrophil apoptosis and inflammation resolution in nonresponding pneumonia.

Neutrophil activation state and its relationship with an inflammatory environment in community-acquired pneumonia (CAP) remain insufficiently elucidated. We aimed to evaluate the neutrophil apoptosis and cytokine pattern in CAP patients after 72 h of treatment, and their impact on infection resolution. Apoptosis of blood and bronchoalveolar lavage (BAL) neutrophils was measured in nonresponding CAP (NCAP), in responding CAP (blood only) and in patients without infection (control). Pro-inflammatory (interleukin (IL)-6, IL-8) and anti-inflammatory (IL-10) cytokines were measured. Main outcomes were clinical stability and days of hospitalisation. Basal neutrophil apoptosis was higher in the BAL and blood of NCAP, whereas spontaneous apoptosis (after 24 h culture) was lower. Cytokines in NCAP were higher than in responding CAP and control: IL-6 was increased in BAL and blood, IL-8 in BAL and IL-10 in blood. An increased basal apoptosis (≥20%) in BAL of NCAP was associated with lower systemic IL-10 (p<0.01), earlier clinical stability (p=0.05) and shorter hospital stay (p=0.02). A significant correlation was found for systemic IL-6 and IL-10 with days to reach stability and length of stay. After 72 h of treatment, an increased basal alveolar neutrophil apoptosis might contribute to downregulation of inflammation and to faster clinical stability.

Long-standing malignant pancreatic carcinoid treated with octreotide.

A male presented with a metastatic, plasma serotonin-secreting (high 5-HIAA in urine), malignant pancreatic carcinoid with a carcinoid-like syndrome, and was assessed using ultrasounds (US), computerized tomography (CT), magnetic resonance imaging (MRI), endoscopic ultrasonography (EUS) and Octreoscan; he sequentially received chemotherapy, interferon and octreotide, with long-term, 12-year survival after diagnosis. Given this unusual case, the second reported in our country, the overall literature is reviewed.

Gastrointestinal carcinoid tumors.

OBJECTIVE: carcinoid tumors (CTs) represent the commonest neuroendocrine tumors. Those in the gastrointestinal tract are diagnosed in surgical specimens, clinically, and using imaging techniques (endoscopy, echoendoscopy, CT, Octreoscan, etc.). The goal of this retrospective study was to review a personal series of gastrointestinal carcinoid tumors, and to compare it to those in the literature. PATIENTS AND METHODS: the medical records of 40 Caucasian patients with over 50 gastrointestinal carcinoid tumors (including multiple cases) who were seen for a period of 16 years (1994-2009) were reviewed. RESULTS: mean age at presentation was 52 years, 50% were females, and mean tumor size was 9.9 mm. Most were gastroduodenal (42.5%) or rectal (30%), and were treated endoscopically. Metastases and carcinoid syndrome (CS) were seen in 5% of patients. Survival at study endpoint was 85%. CONCLUSIONS: age and gender were consistent with the literature. There was an increase in gastroduodenal (multifocal) and rectal carcinoids, likely because the series was essentially endoscopical in nature (bias). There was a lower rate of CS and higher survival, likely due to earlier diagnosis and treatment.

Natural orifice transluminal endoscopic surgery (NOTES).

A current review and update of an exceedingly novel and appealing topic, namely natural orifice transluminal endoscopic surgery (NOTES), is discussed, as well as the authors viewpoint thereon. Most reviewed studies were performed in laboratory animals, but reports on transvaginal cholecystectomy and the emergence of editorials and review articles on this topic pose a number of as yet unanswered questions on this type of surgery, which represents a potential advance towards "endoscopic surgery with no scars, no infection, minimal anesthesia requirements, and immediate recovery".

Neuroendocrine tumors - fascination and infrequency.

In this article, I review and update of gastro-entero-pancreatic neuroendocrine tumors, which so much fascination have risen among healthcare providers on grounds of their infrequency, hormonal syndromes, and high survival rate, is performed based on references from the past fifteen years.

[Ultrasound-guided biopsy of the pancreas: a multicenter study]. / Punción pancreática ecodirigida: estudio multicéntrico.

OBJECTIVE: Members of "Asociación de Ecografía Digestiva" decided to carry out a multicenter retrospective study on fine-needle aspiration biopsy for pancreatic space-occupying lesions under ultrasonographic guidance and via the percutaneous route in order to assess this technique s performance versus endoscopic ultrasound-guided biopsy. SUBJECTS: 10 hospitals for a total of 222 patients with suspiciously malignant, 8-120-mm pancreatic lesions were included in the study. RESULTS: The analysis of results shows a sensitivity of 89%, a specificity of 98%, a positive predictive value of 99%, and a negative predictive value of 74%, for an overall diagnostic accuracy of 91%. No major complications occurred. CONCLUSION: Percutaneous fine-needle aspiration for pancreatic lesions is highly cost-effective and has few and mild complications.

Atypical thymic carcinoid in multiple endocrine neoplasia type 1 syndrome.

An asymptomatic, non-smoker patient carrier of a multiple endocrine neoplasia syndrome type 1 (MEN1) mutation was diagnosed with invasive atypical thymic carcinoid tumor. After surgical treatment the tumor reappeared albeit without metastasis. Thymic carcinoid is a well-known cause of mortality in MEN1, and usually metastatic disease is present at diagnosis. Male sex, smoking, and previous hyperparathyroidism probably play a role in the pathogenesis of this neoplasia.

[Tridimensional (3D) endoscopic ultrasonography]. / Ultrasonografía endoscópica tridimensional (USE-3D).

A review and update on 3D endoscopic ultrasonography is included regarding all of this technique s aspects, technical details, and current indications. Images from our own clinical experience are presented.

EUS-guided mucosectomy for gastrointestinal cancer.

INTRODUCTION: the only way of improving prognosis and survival in gastrointestinal cancer is early diagnosis, with intramucosal localization as confirmed by endoscopic ultrasonography (EUS) or 20-MHz miniprobes (MPs) (T1) being most appropriate. Endoscopic mucosal resection (EMR) has proven effective in the treatment of this sort of lesions. PATIENTS AND METHOD: in a group (18 cases) with 15 cases of superficial gastrointestinal cancer and 3 cases of severe gastric dysplasia, 9 cases (3 esophageal, 4 gastric, 2 rectal) underwent a classic EMR following EUS or a 7.5- and 20-MHz miniprobe exploration. RESULTS: ultrasonographic studies showed a T1 in all but one esophageal case (Tis), and in both gastric dysplasias, with no changed layer structure being demonstrated in the latter (T0). No complications arose with classic EMR, and all 9 patients are alive and free from local or metastatic recurrence, except for one esophageal case, which recurred distally to the esophageal lesion (metachronous). CONCLUSIONS: echoendoscopically-assisted EMR is a safe, effective technique in the endoscopic management of superficial gastrointestinal (esophageal, gastric, colorectal) cancer. Recurrence most likely depends upon cancer multiplicity.