Assaying ADAMTS13 Activity as a Potential Prognostic Biomarker for Sinusoidal Obstruction Syndrome in Mice.

Sinusoidal obstruction syndrome (SOS) is a serious liver disorder that occurs after liver transplantation, hematopoietic stem cell transplantation, and the administration of anticancer drugs. Since SOS is a life-threatening condition that can progress to liver failure, early detection and prompt treatment are required for the survival of patients with this condition. In this study, female CD1 mice were divided into treatment and control groups after the induction of an SOS model using monocrotaline (MCT, 270 mg/kg body weight intraperitoneally). The mice were analyzed at 0, 12, 24, and 48 h after MCT administration, and blood and liver samples were collected for assays and histopathology tests. SOS was observed in the livers 12 h after MCT injection. In addition, immunohistochemical findings demonstrated CD42b-positive platelet aggregations, positive signals for von Willebrand factor (VWF), and a disintegrin-like metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) in the MCT-exposed liver sinusoid. Although ADAMTS13's plasma concentrations peaked at 12 h, its enzyme activity continuously decreased by 75% at 48 h and, inversely and proportionally, concentrations in the VWF-A2 domain, in which the cleavage site of ADAMTS13 is located, increased after MCT injection. These findings suggest that the plasma concentration and activity of ADAMTS13 could be useful biomarkers for early detection and therapeutic intervention in patients with SOS.

A case of midgut volvulus related to adult intestinal malrotation found with weight loss after streptococcus infection: A case report and literature review.

INTRODUCTION AND IMPORTANCE: The incidence of intestinal malrotation is 1 in 6000 births, and 90% of cases occur within the first year of life. Adult cases are rare, with a reported incidence of 0.2%-0.5% of all cases. The significance of reporting this case is to recognize that some adult-onset cases require surgery even in the absence of intestinal necrosis. CASE PRESENTATION: A 36-year-old man was infected with streptococcus and treated with antibiotics. He developed appetite loss and his weight decreased 12 kg in 4 months. His abdomen was flat and soft with no tenderness. A computed tomography scan showed that the horizontal duodenal leg was not anchored to the retroperitoneum. Rotation of the mesentery, which was wrapped around the superior mesenteric artery in a clockwise direction, was observed, suggesting midgut volvulus. We performed emergency surgery and Ladd's procedure. CLINICAL DISCUSSION: A previous study reported that the most common symptom in the chronic course of intestinal malrotation was abdominal pain in 41.2% of cases, and weight loss was observed in only 2.6% of patients. The high degree of intestinal adhesion suggests that repeated torsion and release and the development of collateral vessels may have contributed to the asymptomatic course. CONCLUSION: Adult-onset intestinal malrotation should be considered as a differential diagnosis in the presence of weight loss and gastrointestinal symptoms. The timing of surgery is still controversial. In chronic cases, severe adhesion might be expected and laparoscopic surgery should be considered carefully.

[A Case of Pneumocystis Pneumonia Developed during Chemotherapy for Sigmoid Colon Cancer].

A 69-year-old man with multiple liver metastases from sigmoid colon cancer received mFOLFOX6 plus cetuximab(Cmab) chemotherapy. A partial response was observed; hence, we performed an extended left hepatectomy, 3 partial liver resections, and a sigmoidectomy. After 4 courses of CapeOX, a recurrent lesion occurred between S8 and S7 of the liver, and we changed the regimen to FOLFIRI plus bevacizumab(BV). Three months later, he had Grade 3 febrile neutropenia and CT scan findings showed ground glass opacity in the superior lobes of both lungs. We diagnosed pneumocystis pneumonia(PCP)and administered steroids and trimethoprim/sulfamethoxazole. The signs of PCP thus improved. PCP during chemotherapy for gastrointestinal cancer is rarely reported, but recently it has increased.

Rapid improvement of paraplegia caused by epidural involvements of Burkitt's lymphoma with chemotherapy.

STUDY DESIGN: Case report. OBJECTIVE: The authors present a case of atypical Burkitt's lymphoma with multiple epidural involvements. SUMMARY OF BACKGROUND DATA: Spinal cord compression in children is an emergency that requires urgent attention to minimize neurologic dysfunction. Although it is not life-threatening in most patients, cord compression can cause severe neurologic morbidity. MATERIALS AND METHODS: Because the patient showed rapid neurologic deterioration, we started chemotherapy and high-dose steroids without laminectomy or radiotherapy immediately after a tumor biopsy from the left mandible. RESULT: The combined therapies were very effective and his neurologic symptoms improved immediately. The epidural involved masses disappeared in imaging studies after the first course of chemotherapy including methylprednisolone (20 mg/kg per day for 3 consecutive days and gradually tapered off over 2 weeks), vincristine (1.5 mg/m2 per day), cyclophosphamide (2 g/m2 per day for 2 days) and pirarubicin (40 mg/m2 per day). After completing seven courses of chemotherapy, the patient is now fully ambulant. CONCLUSION: Considering the severe late effects of laminectomy and radiotherapy, chemotherapy should be considered as a first choice of treatment for spinal cord compression caused by malignant lymphoma.

Second transplantation from HLA 2-loci-mismatched mother for graft failure due to hemophagocytic syndrome after cord blood transplantation.

A 7-year-old girl with acute myelogenous leukemia with multilineage dysplasia received unrelated cord blood transplantation but developed hemophagocytic syndrome (HPS) after sepsis with methicillin-resistant coagulase-negative staphylococci before engraftment. Bone marrow aspiration on day 20 revealed a markedly increased number of activated macrophages showing hemophagocytosis. The presence of donor-type chimera in the bone marrow was confirmed at that time. We therefore quickly started immunosuppressive and antibacterial treatment. Although her condition gradually improved, the patient suffered graft failure due to HPS. She received peripheral blood stem cell transplantation from her HLA 2-loci-mismatched mother on day 54 and continued in complete remission 12 months after the second transplantation. The results in this case suggested that because of fetomaternal microchimerism it may be useful to select an HLA-haploidentical mother as a backup donor for stem cell transplantation.