Cytosine arabinoside and mitoxantrone followed by second allogeneic transplant for the treatment of children with refractory juvenile myelomonocytic leukemia.

Hematopoietic stem cell transplantation (HSCT) remains the only curative option for most patients with juvenile myelomonocytic leukemia (JMML). However, persistent disease and relapse rates after transplant range from 26% to 58%. We report the successful use of second HSCT after preparation with mitoxantrone and cytosine arabinoside (Ara-C) for patients with refractory or recurrent disease. Between 1993 and 2006, 5 children who underwent HSCT at our institution as initial therapy for JMML had persistent disease or relapsed. Pre-HSCT conditioning varied and donors were either HLA-matched siblings (n=2) or matched unrelated donors (n=3). After initial HSCT, they subsequently received high-dose Ara-C (3 g/m IV) every 12 hours on days -8 through -3 and mitoxantrone (10 mg/m/d IV) on days -8, -7, -6 followed by second HSCT from their original donors. All 5 patients are alive at 88, 179, 199, 234, and 246 months with no evidence of JMML, no significant toxicity, and 100% donor chimera as determined by PCR short-tandem repeat analysis. Our experience supports second transplant utilizing high-dose Ara-C and mitoxantrone in children with JMML who do not respond or relapse after first transplant.

Multifocal histologically malignant Epstein-Barr virus-associated smooth muscle tumor in a pediatric transplant patient with an indolent course.

Epstein-Barr virus-associated smooth muscle tumors (EBV-SMTs) are rare lesions that occur in immunocompromised patients. Dural involvement appears to be less common in organ transplant recipients than in HIV patients. Due to the paucity of reported cases following organ transplantation, the natural history of these lesions is unclear. We describe an 8-year-old female who presented with adrenal, small bowel, and intracranial tumors 6 years following renal transplantation. Histopathological analysis revealed a highly cellular, mitotically active, smooth muscle neoplasm without necrosis. The tumor stained diffusely for smooth muscle actin and myosin. In situ hybridization for EBV-encoded RNA was diffusely positive. Following gross total resection, antiviral therapy, and a reduction in immunosuppression, the patient is tumor-free at 3 years follow-up. In patients with compromised immune systems, it is important to recognize this unique form of SMT because, even when there are multiple lesions, the prognosis may be excellent.

From library to discharge: a managing care student project.

BACKGROUND AND OBJECTIVES: The Patient Care Project (PCP) was a central component of the Undergraduate Medical Education for the 21st Century (UME-21) grant project at the University of Nebraska. With the primary goal of improving students' critical thinking skills, the PCP was directed more toward an understanding of managing care than the business aspects of managed care and emphasized written communication skills, clinical hypothesis testing, and exploring ways to solve medical and ethical questions. METHODS: All 239 students graduating in 2000 and 2001 were required to analyze the medical care received by one of their hospitalized patients. Using a criterion-based evaluation tool, students' written critiques were assessed in five specific areas, all of which required critical thinking skills. Students also received an overall grade for the project. The UME-21 Graduation Survey was used to assess changes in attitudes and behavior. Students graduating in 1999, prior to the institution of the PCP graduation requirement, served as a control group. RESULTS: The most frequently discussed topic of the PCPs was cardiovascular disease. The mean overall rating by the faculty for the PCPs was 3.7 and 3.8 in academic years 2000 and 2001, respectively (maximum=5). In a qualitative analysis of the PCPs, students demonstrated insight into their patients' overall medical care, including the use of evidence-based medicine (EBM), quality improvement, and cost containment. There were no statistically significant differences, however, between the PCP and control groups on the UME-21 Graduation Survey. Nonetheless, more students who had completed the PCP reported that they had identified the total cost of a patient's stay, designed a quality improvement loop, and obtained clinical evidence from an EBM computer database. On this same survey, all students agreed with the use of clinical practice guidelines and cost containment. CONCLUSIONS: The PCP appeared to be relevant to the students' learning needs, and they provided cogent critiques of the medical care they had rendered as well as critical analyses of their patients' discharge summaries and the cost of care including ways to reduce cost. On the other hand, we were unable to demonstrate any substantial differences in the results of the UME-21 Graduation Survey given to both the PCP and control groups. In spite of this lack of effect on students' attitudes, the PCP was perceived by the faculty to be valuable and has been incorporated into the required third-year family medicine clerkship at the University of Nebraska.