Appendicitis in Neutropenic Pediatric Oncology Patients: A Case Series and Review of the Literature.

Appendicitis is the most common reason for which pediatric patients require emergent surgery. Although surgical removal of the appendix is the standard of care for appendicitis, neutropenic patients require special consideration as optimal management for these patients remains controversial. We present a case series of 3 neutropenic patients with appendicitis, all of whom were managed differently according to the circumstances unique to each case. By reviewing key articles in the literature, we explore the safety and efficacy of both medical and surgical management of neutropenic appendicitis and propose an algorithm to help guide decision making.

Lemierre's Syndrome as a Trigger for Secondary Hemophagocytic Lymphohistiocytosis.

Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare hyperinflammatory disorder caused by an aberrant immune response to a number of infectious or inflammatory conditions. Successful treatment of this potentially fatal condition requires early recognition and prompt therapy directed at the underlying trigger. In this report, we describe the clinical presentation, diagnostic findings, management, and outcome of a child with Lemierre's syndrome-associated sHLH. This is the first reported association of these 2 rare conditions and expands the number of known triggers for sHLH.

Plasmacytoma-like Posttransplant Lymphoproliferative Disorder in a Pediatric Heart Transplant Recipient.

Posttransplant lymphoproliferative disorder (PTLD) is a diversely manifesting group of lymphoid or plasmacytic proliferations found in solid organ and bone marrow transplant recipients. PTLD occurs as a result of immunosuppression and is often driven by the Epstein Barr virus. Although most commonly of B-cell origin, similar to B-cell lymphomas, PTLD can rarely present as a plasmacytic process, resembling multiple myeloma. Although more common in adults, 8 cases of plasmacytoma-like PTLD have been reported in pediatric renal and combined small bowel-liver transplant recipients. Here, we present a rare report of a plasmacytoma-like PTLD case in a pediatric heart transplant recipient.

Comparison of allergic reactions to intravenous and intramuscular pegaspargase in children with acute lymphoblastic leukemia.

Pegaspargase (PEG) is a standard component of therapy for pediatric acute lymphoblastic leukemia (ALL). Because PEG preparations are bacterially derived, they are highly immunogenic. PEG has traditionally been delivered intramuscularly (IM), but over the last several years, more PEG has been given intravenously (IV) in order to provide a less painful and more convenient means of delivery. However, there are limited data comparing allergic reactions between IV and IM PEG recipients, especially in a large cohort of patients. We reviewed the charts of pediatric ALL patients diagnosed from 2006 to 2011 who received PEG at our institution and compared the incidence, time to onset of symptoms, reaction grade, and hospitalization rate for patients who had allergic reactions to PEG. Of 318 evaluable patients, 159 received IV and 159 received IM PEG. Thirty-one (19.5%) IV patients had an allergic reaction, compared to 17 (10.7%) IM patients (P = .028). Time to onset of symptoms was ≤ 30 minutes for 26 of 27 evaluable IV patients (96.3%) versus only two of 11 evaluable IM patients (18.2%; P < .001). Four of 31 IV patients (12.9%) and six of 17 IM patients (35.5%) required hospitalization (P = .134). There is increased incidence of allergy in patients who received IV PEG compared to IM. Grade of reaction was similar between IV and IM, but allergic reactions to IV PEG had a more rapid onset. While the risk of allergy may be increased, IV delivery appears to have an acceptable safety profile for administration in ALL patients.

Pseudohypopyon: Extramedullary relapse of acute myelogenous leukemia with poor prognosis.

An 11-month-old female presented to the emergency department with a 2-week history of fever, increasing fussiness, emesis, and decreased urine output. She was diagnosed with acute myelogenous leukemia. Systemic chemotherapy with intensified intrathecal cytarabine was started, and the patient achieved a clinical remission after the first course of induction. Towards the end of her second course of induction she developed pseudohypopyon in each eye on consecutive days, heralding a central nervous system relapse.

11 Years Later-Isolated Testicular Relapse of ALL After Stem Cell Transplantation Treated With Surgery Only: A Case Report and Review of the Literature.

Despite advances in therapy for childhood acute lymphoblastic leukemia (ALL), relapses continue to occur and are associated with significant morbidity and mortality. Individuals who relapse after stem cell transplantation (SCT) have an especially poor prognosis and typically require intensive systemic therapy to provide any chance of cure. The majority of relapses occur within 2 years following SCT; relapses later than 4 years post SCT are rare. Here we describe an isolated testicular relapse of ALL 11 years after SCT, treated with local control measures only, and review the literature for other cases of very late ALL relapse following SCT.

Serial MRI evaluation of cardiac structure and function in mice after reperfused myocardial infarction.

This study evaluated the utility of cardiac MRI for assessing the impact of myocardial infarction (MI) on cardiac structure and function in mice following reperfused 1- or 2-hr occlusions of the left anterior descending coronary artery (LAD). When assessed 1 day after MI, the left ventricular ejection fraction (LVEF) had declined by more than half, and remained depressed for the duration of the study. Furthermore, MI initiated dramatic increases in both LV end-systolic volume (LVESV) and end-diastolic volume (LVEDV), with a greater than threefold increase in LVESV and a twofold increase in LVEDV by 4 weeks post-MI. Transmural LV wall thickening (WTh) analysis revealed that noninfarcted myocardium in the remote septal region exhibited an early deficit in contractile function after MI that transiently resolved by day 7, only to be followed by a late phase of dysfunction that became fully manifest by day 28 post-MI. In conclusion, MRI allows the serial assessment of cardiac structure and function after MI in mice, with a resolution adequate to document both regional and temporal changes. The application of these imaging techniques in transgenic and knock-out mice will greatly expedite research aimed at defining the functional roles of individual genes in the pathophysiology of LV remodeling (LVR) after reperfused MI.