Safety and tolerability of allogeneic dendritic cell vaccination with induction of Wilms tumor 1-specific T cells in a pediatric donor and pediatric patient with relapsed leukemia: a case report and review of the literature.

A 15-year-old girl with acute lymphoblastic leukemia received allogeneic dendritic cell vaccination, pulsed with Wilms tumor 1 (WT1) peptide, after her third hematopoietic stem cell transplantation (HSCT). The vaccines were generated from the third HSCT donor, who was her younger sister, age 12 years. The patient received 14 vaccines and had no graft-versus-host disease or systemic adverse effect, aside from grade 2 skin reaction at the injection site. WT1-specific immune responses were detected after vaccination by both WT1-tetramer analysis and enzyme-linked immunosorbent spot assay. This strategy may be safe, tolerable and even feasible for patients with a relapse after HSCT.

Successful cord blood transplantation after repeated transfusions of unmobilized neutrophils in addition to antifungal treatment in an infant with chronic granulomatous disease complicated by invasive pulmonary aspergillosis.

BACKGROUND: Chronic granulomatous disease (CGD) is a rare inherited primary immunodeficiency that affects phagocytic cells. CGD patients are susceptible to fungal infections, especially Aspergillus infections. The management of life-threatening Aspergillus infections in CGD is particularly difficult because some infections cannot be eradicated with standard antifungal treatments and, hence, result in death. CASE REPORT: A 2-week-old girl developed invasive pulmonary aspergillosis, which rapidly progressed to respiratory failure. Liposomal amphotericin B, micafungin, and voriconazole were not effective. At the age of 2 months, she was diagnosed with p67phox-deficient CGD. In addition to antifungal treatment, the patient received 21 granulocyte transfusions (GTX), which were obtained from 300- or 400-mL whole blood samples from healthy random donors who were not treated with granulocyte-colony-stimulating factor or dexamethasone. The median neutrophil count of the GTX was 1.88 × 10(8) /kg body weight. Rituximab was administered to reduce alloimmunization to human leukocyte antigens (HLA) after the eighth GTX, resulting in their absence of anti-HLA before and after cord blood transplantation (CBT). A marked improvement in her invasive pulmonary aspergillosis was achieved, although the first CBT was rejected. Complete hematopoietic recovery was obtained after the second CBT. CONCLUSION: Repeated GTX containing relatively low doses of neutrophils might be able to control severe Aspergillus infections in infants with CGD.

The Role of Colon in Isolated Intestinal Transplantation: Description of 4 Cases.

Intra-abdominal desmoid tumors are a rare and complex clinical problem. These tumors are locally invasive, and surgical ablation represents the mainstay of treatment. When localized at the root of the mesentery, their resection may require extensive excision of the intestine resulting in intestinal failure and life-long total parenteral nutrition. Intestinal transplantation, either autotransplantation or allotransplantation, has been used as a viable option to treat this group of patients. Herein, we describe a series of 4 patients with unresectable intra-abdominal desmoid tumor who underwent cadaveric isolated intestinal and ascending colon transplantation.

Replaced platelet concentrates containing a new additive solution, M-sol: safety and efficacy for pediatric patients.

BACKGROUND: Allergic transfusion reactions (ATRs), particularly those caused by plasma-rich platelet concentrates (P-PCs), are an important concern in transfusion medicine. Replacing P-PCs with PCs containing M-sol (M-sol-R-PCs) is expected to prevent ATRs. However, this has not yet been verified by sufficient clinical evidence. STUDY DESIGN AND METHODS: A retrospective cohort study was performed between 2008 and 2011. Pediatric patients with hematologic disorders, solid tumors, primary immunodeficiency disorders, or inherited metabolic disorders were transfused with M-sol-R-PCs between 2010 and 2011; the transfusions of P-PCs administered between 2008 and 2011 were compared in terms of frequency and severity of ATRs, corrected count increment (CCI), and occurrence of bleeding. Data were collected for 6 consecutive months on a per-patient basis. RESULTS: Data obtained during 2008 to 2011 showed that of the 78 patients receiving 515 P-PC transfusions, 14 (17.9%) had 17 ATRs (3.3%); 14 and three ATRs were of Grades 1 and 2, respectively. In 2010 to 2011, 49 patients received 620 transfusions of M-sol-R-PCs, and two patients (4.1%) had Grade 1 ATRs (0.3%). Thus, the frequency of ATRs per bag and per patient differed significantly between the two transfusions. No steroid agents were used for the prevention or treatment of ATRs in the M-sol-R-PC group. The CCI (24 hr) for M-sol-R-PCs did not differ from that for P-PCs. CONCLUSION: M-sol-R-PCs were found to be effective in preventing ATRs without loss of transfusion efficiency in children; however, its efficacy should be further evaluated in prospective clinical trials.

Usefulness of allogeneic hematopoietic stem cell transplantation in first complete remission for pediatric blastic plasmacytoid dendritic cell neoplasm with skin involvement: a case report and review of literature.

No standard treatment has been established in childhood blastic plasmacytoid dendritic cell neoplasma (BPDCN) because of its rarity. We treated with acute lymphoblastic leukemia-type regimen for a child with BPDCN with skin and leukemic involvement. She has been disease-free for 4 years after allogeneic bone marrow transplantation in first complete remission. In 33 cases of pediatric BPDCN, the over survival was significantly lower in the patients with skin manifestation than those without cutaneous involvement. Accordingly, it is important to determine whether allogeneic hematopoietic stem cell transplantation should be applied to first complete remission in the patients with poor prognosis.

Myelodysplastic syndrome in a child with 15q24 deletion syndrome.

15q24 deletion syndrome is a recently-described chromosomal disorder, characterized by developmental delay, growth deficiency, distinct facial features, digital abnormalities, loose connective tissue, and genital malformations in males. To date, 19 patients have been reported. We report on a 13-year-old boy with this syndrome manifesting childhood myelodysplastic syndrome (MDS). He had characteristic facial features, hypospadias, and mild developmental delay. He showed neutropenia and thrombocytopenia for several years. At age 13 years, bone marrow examination was performed, which showed a sign suggestive of childhood MDS: mild dysplasia in the myeloid, erythroid, and megakaryocytic cell lineages. Array comparative genomic hybridization (array CGH) revealed a de novo 3.4 Mb 15q24.1q24.3 deletion. Although MDS has not been described in patients with the syndrome, a boy was reported to have acute lymphoblastic leukemia (ALL). The development of MDS and hematological malignancy in the syndrome might be caused by the haploinsufficiency of deleted 15q24 segment either alone or in combination with other genetic abnormalities in hematopoietic cells. Further hematological investigation is recommended to be beneficial if physical and hematological examination results are suggestive of hematopoietic disturbance in patients with the syndrome.

Planned Versus On-Demand Relaparotomy Strategy in Initial Surgery for Non-occlusive Mesenteric Ischemia.

BACKGROUND: There has been insufficient evidence regarding a treatment strategy for patients with non-occlusive mesenteric ischemia (NOMI) due to the lack of large-scale studies. We aimed to evaluate the clinical benefit of strategic planned relaparotomy in patients with NOMI using detailed perioperative information. METHODS: We conducted a multicenter retrospective cohort study that included NOMI patients who underwent laparotomy. In-hospital mortality, 28-day mortality, incidence of total adverse events, ventilator-free days, and intensive care unit (ICU)-free days were compared between groups experiencing the planned and on-demand relaparotomy strategies. Analyses were performed using a multivariate mixed effects model and a propensity score matching model after adjusting for pre-operative, intra-operative, and hospital-related confounders. RESULTS: A total of 181 patients from 17 hospitals were included, of whom 107 (59.1%) were treated using the planned relaparotomy strategy. The multivariate mixed effects regression model indicated no significant differences for in-hospital mortality (61 patients [57.0%] in the planned relaparotomy group vs. 28 patients [37.8%] in the on-demand relaparotomy group; adjusted odds ratio [95% confidence interval] = 1.94 [0.78-4.80]), as well as in 28-day mortality, adverse events, and ICU-free days. Significant reduction in ventilator-free days was observed in the planned relaparotomy group. Propensity score matching analysis of 61 matched pairs with comparable patient severity did not show superiority of the planned relaparotomy strategy. CONCLUSIONS: The planned relaparotomy strategy, compared with on-demand relaparotomy strategy, did not show clinical benefits after the initial surgery of patients with NOMI. Further studies estimating potential subpopulations who may benefit from this strategy are required.

Acute pancreatitis followed by retroperitoneal perforation of the descending colon and a duodenal fistula: Report of a case.

INTRODUCTION: There are several reports of colon perforation in patients with acute pancreatitis, but the mechanism is not understood. We describe a patient with acute pancreatitis followed by retroperitoneal perforation of the descending colon and a duodenal fistula. CASE PRESENTATION: A 51-year-old male presented with acute pancreatitis. He was hemodynamically unstable, had respiratory failure on admission and was treated in the intensive care unit (ICU). He recovered and left the ICU on day 13. Although his general condition improved, a computed tomography (CT) scan showed air and fluid in the left retroperitoneum. Gastrografin enema and CT scan showed extraluminal leakage in the descending colon and retroperitoneal drainage was performed on day 27. After drainage, there was continuous voluminous feculent discharge, and a loop ileostomy was performed on day 34. A repeat CT scan revealed ascites. A percutaneous catheter injected with contrast showed a duodenal fistula. After drainage, the fever resolved and the patient was discharged on hospital day 106. DISCUSSION: Although there is no clear mechanism of colonic perforation in patients with acute pancreatitis, one hypothesis is that ischemia secondary to inflammation caused by pancreatitis plays a role. The involved area is usually in the watershed areas of the colon. Retroperitoneal drainage of the colon perforation may have necessitated creation of a diverting loop ileostomy. CONCLUSION: Retroperitoneal colon perforation must be considered in patients with acute pancreatitis.

Successful manual reduction for ureterosciatic hernia: A case report.

INTRODUCTION: Sciatic hernias are the least common type of pelvic floor hernias. The purpose of this study was to present a novel technique for manual reduction and to conduct a systematic review of previous reports of sciatic hernias to characterize them and review the outcomes. PRESENTATION OF CASE: An 86-year-old female presented with left-sided lumbar pain. She had a past medical history of rheumatoid arthritis and was treated with prednisolone and methotrexate. Her left abdomen and left lumbar area were tender. An unenhanced abdominal computed tomography scan revealed invagination of the left ureter into the left sciatic foramen and a dilated left proximal ureter and renal pelvis. Ultrasonography showed an invaginated left ureter viewing from the left buttock. She was diagnosed with a sciatic hernia. Ultrasound-guided manual transvaginal reduction was performed. Post-procedure unenhanced abdominal computed tomography scan confirmed reduction of the ureter. After 10-months of follow-up, there is no evidence of recurrence. DISCUSSION: Previous reports of patients with sciatic hernia were identified. Clinical data associated with the hernia, reduction technique and clinical outcomes were collected for 72 patients. Open reduction was performed in 24 patients. A ureteral stent was placed in eight patients when the hernia contained the ureter. Four postoperative complications including one death were reported in adults. There were no reports of closed manual reduction. CONCLUSION: A sciatic hernia in women may be manually reduced without surgery. Further reviews of this rare entity are needed to determine the best management strategy.

Metastatic colon cancer derived from a diverticulum incidentally found at herniorrhaphy: a case report.

BACKGROUND: There are few reports of metastases from colon cancer to an inguinal hernia sac, and few reports of colon cancer originating in diverticula. We report a patient with carcinoma of the sigmoid colon arising in two diverticula, who presented with peritoneal seeding to an inguinal hernia sac, and a review of the literature. CASE PRESENTATION: A 55-year-old male underwent open herniorrhaphy for a left inguinal hernia. At operation, a nodule in the inguinal hernia sac was resected and histologic examination revealed adenocarcinoma, which was suspected to be a metastasis from a distant primary lesion. Postoperative evaluation included colonoscopy and positron emission tomography which showed two suspected lesions in sigmoid diverticula. Laparoscopic subtotal colectomy was performed, and pathology revealed adenocarcinoma in two sigmoid diverticula. CONCLUSIONS: If a nodule is found in an inguinal hernia sac, especially in older patients, peritoneal metastases should be considered. Resection of the nodule with histopathologic evaluation is essential. Colon cancer arising in a diverticulum should be considered as a possible site of the primary lesion.

Delayed graft duodenal perforation due to impacted food five years after simultaneous pancreas-kidney transplantation: A case report.

INTRODUCTION: Pancreas transplantation is the best treatment option in selected patients with type 1 diabetes mellitus. Here we report a patient with a nonmarginal duodenal perforation five years after a simultaneous pancreas-living donor kidney transplantation (SPLKT). PRESENTATION OF CASE: A 31-year old male who underwent SPLKT five years previously presented with severe abdominal pain. He had a marginal duodenal perforation four years later, treated by primary closure and drainage. Biopsy of the pancreas and duodenum graft at that time showed chronic rejection in the pancreas and acute inflammation with an ulcer in the duodenum. At presentation, computerized tomography scan showed mesenteric pneumatosis with enteric leak and ileal dilatation proximal to the anastomotic site. We performed emergent laparotomy and found a 1.0cm perforation at the nonmarginal, posterior wall of the duodenum. Undigested fiber-rich food was extracted from the site and an omental patch placed over the perforation. An ileostomy was created proximal to the omega loop for decompression and a drain placed nearby. The postoperative course was unremarkable. DISCUSSION: There are only eight previous cases of graft duodenal perforation in the literature. Fiber-rich food residue passing through the anastomosis with impaction may have led to this perforation. CONCLUSION: When a patient is stable, even in the presence of delayed duodenal graft perforation, graft excision may not be necessary. Intraoperative exploration should include Doppler ultrasound examination of the vasculature to rule out thrombosis as a contributor to ischemia. Tissue biopsy should be performed to diagnose rejection.

Massive Preperitoneal Hematoma after a Subcutaneous Injection.

Preperitoneal hematomas are rare and can develop after surgery or trauma. A 74-year-old woman, receiving systemic anticoagulation, developed a massive preperitoneal hematoma after a subcutaneous injection of teriparatide using a 32-gauge, 4 mm needle. In this patient, there were two factors, the subcutaneous injection of teriparatide and systemic anticoagulation, associated with development of the hematoma. These two factors are especially significant, because they are widely used clinically. Although extremely rare, physicians must consider this potentially life-threatening complication after subcutaneous injections, especially in patients receiving anticoagulation.

Afferent Loop Syndrome after Roux-en-Y Total Gastrectomy Caused by Volvulus of the Roux-Limb.

Afferent loop syndrome is a rare complication of gastric surgery. An obstruction of the afferent limb can present in various ways. A 73-year-old man presented with one day of persistent abdominal pain, gradually radiating to the back. He had a history of total gastrectomy with a Roux-en-Y reconstruction. Abdominal computed tomography scan revealed dilation of the duodenum and small intestine in the left upper quadrant. Exploratory laparotomy showed volvulus of the biliopancreatic limb that caused afferent loop syndrome. In this patient, the 50 cm long limb was the cause of volvulus. It is important to fashion a Roux-limb of appropriate length to prevent this complication.

Reduced-toxicity myeloablative conditioning consisting of 8-Gy total body irradiation, cyclophosphamide and fludarabine for pediatric hematological malignancies.

Conventional myeloablative conditioning (MAC) regimens often cause severe regimen-related toxicity (RRT). Furthermore, many patients suffer from poor quality of life in accordance with the increase in long-term survivors. We therefore devised a reduced-toxicity myeloablative conditioning (RTMAC) regimen consisting of 8-Gy total body irradiation (TBI), fludarabine (FLU) and cyclophosphamide (CY) for pediatric hematological malignancies. A retrospective single-center analysis was performed on patients with leukemia or myelodysplastic syndrome (MDS), aged ≤20 years, who had received an 8-Gy TBI/FLU/CY RTMAC regimen followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT). Thirty-one patients underwent first allo-HSCT after an RTMAC regimen. The diagnoses were acute lymphoblastic leukemia (n = 11), acute myeloid leukemia (n = 13), MDS (n = 4), juvenile myelomonocytic leukemia (n = 1) and acute leukemias of ambiguous lineage (n = 2). While 3 patients showed early hematological relapse, the remaining 28 patients achieved engraftments. None of the patients developed grade 4 or 5 toxicities during the study period. The 5-year overall survival and relapse-free survival were 80% [95% confidence interval: CI, 61-91%] and 71% [95% CI, 52-84%], respectively. Our RTMAC regimen would be less toxic and offers a high probability of survival for children with hematological malignancies.

Risk factors for diabetes mellitus and impaired glucose tolerance following allogeneic hematopoietic stem cell transplantation in pediatric patients with hematological malignancies.

Long-term surviving recipients of allogeneic hematopoietic stem cell transplantation (HSCT) often suffer from diabetes mellitus (DM). We sought to identify risk factors for the development of post-transplant DM and impaired glucose tolerance (IGT) in pediatric HSCT patients. Glucose tolerance statuses were evaluated in 22 patients aged 6.3-21.8 years who had received allogeneic HSCT between the ages of 0.8-13.5 years. Five patients were diagnosed as having type 2 DM, and treated with insulin or oral hypoglycemic agents. Five patients were included in the IGT group, and the remaining 12 children were in the normal glucose tolerance (NGT) group. The cumulative incidence of DM plus IGT was 11.6 % at 5 years and 69.3 % at 10 years. None of the patients were obese/overweight and none had a family history of DM. There were no significant differences in serum levels of leptin and adiponectin between the DM + IGT and the NGT groups. An average preprandial glucose levels in the DM + IGT group were significantly higher than those in the NGT group from preparative conditioning to 60 days after HSCT. In multivariate analysis, an age of ≥6 years at the time of HSCT was significantly associated with the development of DM + IGT. Additionally, careful follow-up is necessary, even for NGT patients.