Management considerations of Hodgkin lymphoma for patients with Fontan physiology.

While treatment protocols for Hodgkin lymphoma (HL) are well established, there is no literature available to guide therapy or estimate prognosis for patients with Fontan physiology who develop HL. The physiology of a Fontan procedure can result in the inability to tolerate chemotherapy toxicities, supportive care, and infection. We present a series of three patients with Fontan physiology who were treated for HL and discuss their clinical course and treatment.

Successful whole lung lavage in a child with pulmonary alveolar proteinosis secondary to hematologic malignancy.

Pulmonary alveolar proteinosis (PAP) describes the accumulation of surfactant in the alveolar space. Secondary PAP has been reported in a variety of diseases, and in rare cases has been associated with hematologic malignancy. Treatment for PAP is based on the underlying disease process, and may include whole lung lavage, inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor, or statins.

Daily text message assessments of 6-mercaptopurine adherence and its proximal contexts in adolescents and young adults with leukemia: A pilot study.

Background: This pilot study explored the feasibility and acceptability of implementing text-based assessments of oral chemotherapy adherence in adolescents and young adults (AYA) with leukemia.

Methods: AYA prescribed maintenance 6-mercaptopurine (6MP) received daily text message surveys and utilized an electronic pill bottle for 28 days. Text surveys assessed 6MP adherence and contextual associates (eg, mood).

Adherence to Multiple Treatment Recommendations in Adolescents and Young Adults with Cancer: A Mixed Methods, Multi-Informant Investigation.

This mixed methods study sought to assess adolescent and young adult (AYA) adherence to three cancer treatment recommendations (medications, diet, physical activity), and determine the individual, family, and health system factors associated with suboptimal adherence. In Stage 1, 72 AYA-caregiver dyads completed a validated adherence interview and surveys about individual and family functioning. Matched providers ( = 34 who reported on 61 AYAs) completed global adherence ratings through survey.

Feasibility and acceptability of a pilot tailored text messaging intervention for adolescents and young adults completing cancer treatment.

Purpose: Despite cure, adolescents and young adults (AYA) who complete cancer treatment remain at risk for numerous physical and psychological late effects. However, engagement in recommended follow-up care, knowledge of cancer treatment history and risks, and adoption of health promoting behaviors are often suboptimal. The pilot randomized controlled trial assessed the feasibility and acceptability of a text messaging intervention (THRIVE; Texting Health Resources to Inform, motiVate, and Engage) designed to promote well-being, and health knowledge and behaviors.

Gain-of-Function STAT1 Mutation With Familial Lymphadenopathy and Hodgkin Lymphoma.

In this report, we describe a novel T437N STAT1 mutation found in a mother and 3 of her 4 children which we demonstrate yields gain-of-function. All of the four patients with the T437N STAT1 mutation experienced lymphadenopathy. However, two of the children developed Nodular Lymphocyte Predominant Hodgkin Lymphoma (NHLPL) and have responded to chemotherapeutic regimens.

Patient and hospital factors associated with induction mortality in acute lymphoblastic leukemia.

Background: Deaths during induction chemotherapy for pediatric acute lymphoblastic leukemia (ALL) account for one-tenth of ALL-associated mortality and half of ALL treatment-related mortality. We sought to ascertain patient- and hospital-level factors associated with induction mortality.

Procedure: We performed a retrospective cohort analysis of 8,516 children ages 0 to <19 years with newly diagnosed ALL admitted to freestanding US children's hospitals from 1999 to 2009 using the Pediatric Health Information System database.

"To be a phenomenal doctor you have to be the whole package": physicians' interpersonal behaviors during difficult conversations in pediatrics.

Background: Delivery of bad news is a challenging task for physicians and other health care professionals. Several studies have assessed parental perceptions of the delivery of bad news, but none have focused on the role of physicians' interpersonal behaviors in the communication process.

Objective: The study's objective was to assess parental perceptions of physicians' interpersonal behaviors and their role in communication of bad news.

Protecting pediatric oncology patients from influenza.

Influenza is a common respiratory pathogen. Its severity can be unpredictable, but people with chronic illness are at increased risk of severe infection, complications, and death from influenza. This review examines evidence to support various strategies to protect pediatric oncology patients from influenza-related morbidity.

A prospective study of chemotherapy immunologic effects and predictors of humoral influenza vaccine responses in a pediatric oncology cohort.

Background: Pediatric oncology patients represent a cohort of individuals uniquely at risk of complications from influenza, yet less likely to respond to the vaccine. It is not yet clear how to best protect this vulnerable population.

Methods: We performed a prospective analysis of 177 pediatric oncology patients to define the predictors of influenza vaccine responses.

Immunologic consequences of chemotherapy for acute myeloid leukemia.

There are few data characterizing the immunologic consequences of chemotherapy for acute myeloid leukemia (AML) and almost nothing is known about the effects of chemotherapy in a pediatric AML cohort. We identified T-cell subsets, B-cell subsets, and used Enzyme-linked immunosorbent spot analyses to define the function of T cells and B cells in 7 pediatric patients with AML on chemotherapy. The data show that the effects of chemotherapy disproportionately target the B cell and depletion of B cells is associated with impaired responses to the inactivated influenza vaccine.

Establishment of an 11-year cohort of 8733 pediatric patients hospitalized at United States free-standing children's hospitals with de novo acute lymphoblastic leukemia from health care administrative data.

Background: Acute lymphoblastic leukemia (ALL) accounts for almost one quarter of pediatric cancer in the United States. Despite cooperative group therapeutic trials, there remains a paucity of large cohort data on which to conduct epidemiology and comparative effectiveness research studies.

Research Design: We designed a 3-step process utilizing International Classification of Diseases-9 Clinical Modification (ICD-9) discharge diagnoses codes and chemotherapy exposure data contained in the Pediatric Health Information System administrative database to establish a cohort of children with de novo ALL.

The efficacy of influenza vaccination in a pediatric oncology population.

There is little known about the impact of the timing of influenza vaccine administration on seroconversion in patients on chemotherapy. Recommendations for other vaccines state that the vaccines should be readministered several months after the completion of chemotherapy outside of the stem cell transplant setting. This is not often possible with the influenza vaccine because of its seasonal nature.

Screening for depression and anxiety in adolescent cancer patients.

The goals of this study were to evaluate the feasibility of depression and anxiety screening in on-therapy adolescents with cancer, determine the prevalence of depression and anxiety in this sample, and assess the concordance between patient and oncologist report of patient symptoms. Forty-one adolescents (ages 12 to 18 y) undergoing cancer therapy in an outpatient oncology clinic completed the Beck Youth Inventory II (BYI II) Depression and Anxiety scales. Treating oncologists independently rated patient depression and anxiety.

Community acquired influenza requiring hospitalization: vaccine status is unrelated to morbidity in children with cancer.

Background: Community acquired influenza can be severe and there are few data regarding hospitalization for children with cancer and influenza. Association between prior vaccination and infection severity has not been studied, although vaccination is standard practice.

Procedure: Patients with malignancy or prior stem cell transplant (SCT) were identified using a database of children with laboratory confirmed influenza (2000-2005).

Burkitt lymphoma involving the clivus.

Burkitt lymphoma (BL) is a rapidly dividing tumor that commonly presents itself in the jaw in its endemic form and the abdomen in the sporadic type. Central nervous system involvement at diagnosis is not uncommon, but there have been no previously published reports of BL involving the clivus. Increased tumor burden is associated with complications such as tumor lysis syndrome, and recognition of unusual presentations is important for timely management.

Depressive symptoms and SSRI use in pediatric oncology patients.

This review of depressive symptoms in pediatric cancer patients describes the challenge of recognizing depression in this group, prevalence, risk factors, and treatment, primarily with the selective serotonin reuptake inhibitors (SSRIs). Pediatric oncologists prescribe SSRIs, but there is limited data regarding their use in this setting. Adverse effects, pharmacokinetics and metabolism of SSRIs are reviewed to provide a reference for physicians and inform choices for SSRI prescription.

Phase II study on the effect of disease sites, age, and prior therapy on response to iodine-131-metaiodobenzylguanidine therapy in refractory neuroblastoma.

Purpose: To evaluate the effect of disease sites and prior therapy on response and toxicity after iodine-131-metaiodobenzylguanidine (131I-MIBG) treatment of patients with resistant neuroblastoma.

Patients And Methods: One hundred sixty-four patients with progressive, refractory or relapsed high-risk neuroblastoma, age 2 to 30 years, were treated in a limited institution phase II study. Patients with cryopreserved hematopoietic stem cells (n = 148) were treated with 18 mCi/kg of 131I-MIBG.

Pediatric primary bone lymphoma-diffuse large B-cell lymphoma: morphologic and immunohistochemical characteristics of 10 cases.

Most primary bone lymphomas (PBLs) are diffuse large B-cell lymphomas (DLBCLs). Pediatric PBL-DLBCL has a favorable prognosis but remains poorly characterized. Herein, 10 such cases are detailed.

Primary non-Hodgkin's lymphoma of bone in children.

Background: Primary non-Hodgkin's lymphoma of bone, often more simply referred to as primary lymphoma of bone, is a rare subset of non-Hodgkin's lymphoma in children. There are only a few small series of primary lymphoma of bone in children with long-term follow-up, and none have appeared in the orthopaedic literature.

Methods: A review of our institution's Pediatric Tumor Registry identified fifteen cases of primary lymphoma of bone among 306 cases of diagnosed non-Hodgkin's lymphoma between 1970 and 2003.

Prescribing practices of selective serotonin reuptake inhibitors (SSRIs) among pediatric oncologists: a single institution experience.

Although children and adolescents with cancer often experience symptoms of anxiety and depression, there are no published data regarding the use of selective serotonin reuptake inhibitors (SSRIs) for treating depression in the pediatric oncology population. The goal of this study was to characterize SSRI prescribing practices among pediatric oncologists. A brief (14 item) written survey was distributed to all attending and fellow physicians at a large center with an 85% response rate (N = 40).