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Journal of Pediatric Hematology/oncology

Yueli Rao, Yuanyuan Wu, Ao Dong, Kun Zhu, Wei Li, Shenyang Cai, Min Yang, Jie Yan
Nonleukemic myeloid sarcoma (MS) is a rare tumor that can occur in several locations without myeloid leukemia. We reported a first case of nonleukemic MS of the spleen involving the liver in a 5-month-old boy presenting with hematochezia, petechial hemorrhage, fever, and hepatosplenomegaly. Bone marrow trephine biopsy and immunophenotypic flow cytometry revealed no evidence of myeloid leukemia. The patient underwent liver biopsy and splenectomy. Clinicopathology and immunohistochemistry suggested a disseminated nonleukemic MS...
January 24, 2017: Journal of Pediatric Hematology/oncology
Mehran Karimi, Tahereh Zarei, Sezaneh Haghpanah, Mohamad Moghadam, Ahmad Ebrahimi, Narges Rezaei, Ghazaleh Heidari, Afsaneh Vazin, Maryam Khavari, Hamid R Miri
OBJECTIVE: To evaluate the possible relationship between hydroxyurea (HU) response and some single-nucleotide polymorphism (SNP) in patients affected by β-thalassemia intermedia. MATERIALS AND METHODS: In this cross-sectional study, 100 β-thalassemia intermedia patients who were taking HU with a dose of 8 to 15 mg/kg body weight per day for a period of at least 6 months were randomly selected between February 2013 and October 2014 in southern Iran. HU response was defined based on decrease or cessation of the blood transfusion need and evaluation of Hb level...
January 24, 2017: Journal of Pediatric Hematology/oncology
Laura K Metrock, Muna Qayed, Dawn Simon, Thomas Cash, Michael G O'Connor, Shaina Johnson, Natia Esiashvili, Howard M Katzenstein
Treatment of high-risk neuroblastoma now includes antibody based antitumor immunotherapy as part of standard care. Although this therapy has resulted in dramatic improvements in survival, it is associated with significant side effects. Children with underlying respiratory issues, and in particular asthma, may be more susceptible to immunotherapy associated respiratory compromise and pulmonary complications. Early routine involvement of pulmonology care is warranted for these patients in an effort to allow maximal delivery of immunotherapy and minimize acute and long-term complications...
January 24, 2017: Journal of Pediatric Hematology/oncology
Eda Ataseven, Özlem Tüfekçi, Şebnem Yilmaz, Handan Güleryüz, Hale Ören
Dimethyl sulfoxide (DMSO) is a cryoprotective agent used in storage of frozen stem cells in stem cell transplantation. Central nervous system side effects of DMSO such as epileptic seizures, stroke, transient global amnesia, and temporary leucoencephalopathy are rarely seen. Here, we report a pediatric patient who developed seizures after DMSO-cryopreserved stem cell infusion and whose magnetic resonance imaging of the brain demonstrated parietal and occipital focal cortical T2-signal intensity increase. DMSO toxicity should be kept in mind in patients who received cryopreserved stem cell infusion and magnetic resonance imaging may be helpful in differential diagnosis of central nervous system involvement...
January 24, 2017: Journal of Pediatric Hematology/oncology
Michio Ozeki, Akifumi Nozawa, Kaori Kanda, Tomohiro Hori, Akihito Nagano, Akira Shimada, Tatsuhiko Miyazaki, Toshiyuki Fukao
Pseudomyogenic hemangioendothelioma (PMH) is a recently described vascular neoplasm that occurs most commonly in the soft tissue of the distal extremities of young adults. Metastatic PMH can be fatal and there are no effective medications. We describe a case of a 15-year-old boy with metastatic PMH, who responded to treatment with everolimus, a mammalian target of rapamycin inhibitor. Immunohistochemistry showed that mammalian target of rapamycin was expressed in PMH biopsy specimens, which may explain the reduction in PMH tumor size following treatment...
January 24, 2017: Journal of Pediatric Hematology/oncology
Anna Capsomidis, Andrew Hall, Hamid Daya, Jonathan Round, Donna Lancaster, Jessica Bate
Vocal cord palsy (VCP) is a rare but potentially life-threatening complication in children with cancer. This study reviews UK Intensive Care admissions for children with cancer and VCP using data obtained from the Pediatric Intensive Care Audit Network (PICANet) database. 26 children with cancer and VCP were admitted to intensive care from 2002 to 2012. The majority of admissions (23/26) required respiratory intervention (17 invasive ventilation, 8 noninvasive ventilation, and 5 tracheostomy). VCP should be considered early in children with cancer who present with signs of upper airway obstruction, especially in those receiving vinca-alkaloids as VCP is likely to be reversible...
January 17, 2017: Journal of Pediatric Hematology/oncology
Shinsaku Imashuku, Yoko Shioda, Akira Morimoto
No abstract text is available yet for this article.
January 17, 2017: Journal of Pediatric Hematology/oncology
Samuel A Adegoke, Josefina A P Braga, Adekunle D Adekile, Maria S Figueiredo
Although vitamin D deficiency (VDD) has been linked to anemia among sickle cell disease (SCD), its relationship with hemolysis is unclear. Serum 25-hydroxyvitamin D and biomarkers of hemolysis (hemoglobin [Hb]/hematocrit, reticulocyte percentage, absolute reticulocyte, and lactate dehydrogenase [LDH] levels) in 36 hydroxyurea-naive SCD children were quantified. Correlations were significantly positive with Hb/hematocrit (r=0.40, P=0.017; r=0.45, P=0.006, respectively); inverse with reticulocyte percentage, absolute reticulocyte, and LDH (r=-0...
January 17, 2017: Journal of Pediatric Hematology/oncology
Sarah Leonard, Lindsay M Anderson, Jude Jonassaint, Charles Jonassaint, Nirmish Shah
Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes. Pediatric patients were recruited to participate in the ITP over a 90-day period and were followed for 6 months...
January 17, 2017: Journal of Pediatric Hematology/oncology
Mohamed Y Elsaid, Kara G Gill, Ankush Gosain, Peter F Nichol, Charles M Leys, Darya Buehler, Catherine P Leith, Neha J Patel
BACKGROUND: Coincidence of renal cell carcinoma (RCC) and hematologic malignancies has been reported in adults but not in children. OBSERVATION: We report a case of a 16-year-old girl in whom RCC was incidentally discovered on the computed tomography scan that was performed to stage her underlying Hodgkin lymphoma. Analysis of constitutional cytogenetics for common genetic aberrations that predispose to RCC did not reveal any mutations or genetic variations. However, cytogenetics on the RCC tumor demonstrated a rare reciprocal translocation between chromosomes 6 and 11, t(6;11)(p21;q12)...
January 13, 2017: Journal of Pediatric Hematology/oncology
Abigail V W Kumral, William C Petersen, Christopher Heitz, Linda A Waggoner-Fountain, Brian C Belyea
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...
January 12, 2017: Journal of Pediatric Hematology/oncology
Amanda DiNofia, Kate Shafer, Katarina Steacy, Karim Thomas Sadak
Most childhood cancer survivors need life-long care with specialized late-effects surveillance and screening. As these children age into adulthood, it is imperative to continue their survivor-focused care. To do so, health care systems must be prepared to care for this growing and aging population. This includes creating models of care that take into account the needs and desires of all key stakeholders: survivors, parents, pediatric providers, and adult providers. This clinical observation describes that parents desire comprehensive and highly accessible survivorship care that promotes survivor independence; yet, they also want to have a central role in their child's survivorship care...
January 12, 2017: Journal of Pediatric Hematology/oncology
Kevin O McNerney, Juan C Vasquez, Michael W Kent, Joseph M McNamara
We report a case of a child with pre-B cell acute lymphoblastic leukemia undergoing maintenance chemotherapy with 6-mercaptopurine and methotrexate (MTX) who developed sinusoidal obstruction syndrome after being treated with ciprofloxacin for BK viremia. This case represents a rare complication of maintenance therapy with MTX and 6-mercaptopurine, and suggests a drug interaction between ciprofloxacin and MTX.
January 12, 2017: Journal of Pediatric Hematology/oncology
Mohsen S Elalfy, Nayera H K El Sherif, Tarek M Kamal, Nihal H Aly
BACKGROUND: The klf10 gene could indirectly modify γ-globin chain production and hence the level of fetal hemoglobin (HbF) ameliorating the phenotype of β-hemoglobinopathies and the response to hydroxycarbamide (hydroxyurea [HU]) therapy. In this study, we aimed to evaluate the frequency of different genotypes for the klf10 gene in β-thalassemia major (B-TM), β-thalassemia intermedia (B-TI), and sickle cell disease (SCD) patients by polymerase chain reaction and to assess its relation to disease phenotypes and HU response...
January 12, 2017: Journal of Pediatric Hematology/oncology
Amanda Christina C Dujua, Flerida G Hernandez
This retrospective cohort study is primarily aimed to evaluate the outcome of children ages 0 to 18 years old, with acute lymphoblastic leukemia and treated with a modified Berlin-Frankfurt-Muenster/Hong Kong Acute Lymphoblastic Leukemia (BFM95-HKALL97) protocol at University of Santo Tomas Hospital from January 2005 to December 2009. Seventy-eight patients were included. Majority were between 1 and 10 years old (87.2%), male (61.5%), with normal nutritional status, and classified as upper socioeconomic class (65...
January 12, 2017: Journal of Pediatric Hematology/oncology
Prasad R Koduru, Kathleen Wilson, Jiadi Wen, Rolando Garcia, Sangeeta Patel, Sara A Monaghan
Concurrent perturbations in different driver genes have been reported primarily in lymphoma. In acute myeloid leukemia (AML), cases with concurrent alterations in 2 driver genes are infrequently reported. In contrast to pathogenetic pathways in lymphoma with concurrently perturbed genes, the initial gene alteration in AML arrests maturation and the alteration in the second gene promote self-renewal of the blasts. Here, we report a unique case of infantile leukemia in which chromothripsis in chromosome 8 completely altered the G-band structure and resulted in concurrent changes in MOZ/NCOA2, FGFR1, RUNX1T1, and RUNX1...
January 12, 2017: Journal of Pediatric Hematology/oncology
Salaisudhanprabu Solmuthu Azhagan, Arathi Srinivasan, Shobana Rajendran, Dema Ravinder, Rahul Yadav, Priya Ramachandran, Mukul Vij, Julius Xavier Scott
Perinatal neuroblastoma is the most common solid malignant tumor in infancy which comprises one fifth of all neuroblastomas. Most of them are of adrenal origin and extra-adrenal neuroblastoma is uncommon. We present a rare case of perinatal intrarenal neuroblastoma in a neonate who presented with an incidentally detected abdominal mass. These tumors cause diagnostic and therapeutic dilemma because of its uncommon location. Although very rare, neuroblastoma should be considered in the differential diagnosis of perinatally detected renal tumors...
January 12, 2017: Journal of Pediatric Hematology/oncology
Brent D Bauman, Christopher L Moertel, Eugene Zheng, Amanda Louiselle, Elizabeth Berdan, David Berger, Bradley Segura
INTRODUCTION: Neurofibromatosis type 1 (NF1) is the most commonly inherited autosomal dominant disorder in humans. NF1 patients have increased risk for gastrointestinal stromal tumors (GISTs). A Meckel's diverticulum (MD) represents a persistent embryonic omphalomesenteric duct characterized as a true diverticulum located near the ileocecal valve. We report a unique clinical case whereby a patient with NF1 developed a GIST within a MD. CASE: An adolescent male with NF1 presented with persistent lower abdominal pain...
January 12, 2017: Journal of Pediatric Hematology/oncology
Mozhgan Hashemieh, Mitra Radfar, Azita Azarkeivan, Giti Noghabaei, Kourosh Sheibani
The present study was performed to evaluate pancreatic hemosiderosis by means of magnetic resonance imaging (MRI) T2* and its relation to the diabetic state in thalassemic patients. One hundred thirty transfusion-dependent thalassemic patients from Zafar adult thalassemia clinic, Tehran, Iran, were enrolled in the study. Data such as age, type of thalassemia, age at diagnosis, transfusion duration, ferritin level, and fasting blood sugar results were gathered. Pancreatic MRI T2* was performed for all patients...
January 12, 2017: Journal of Pediatric Hematology/oncology
Alper Ozcan, Hamit Acer, Saliha Ciraci, Hakan Gumus, Musa Karakukcu, Turkan Patiroglu, Mehmet A Ozdemir, Ekrem Unal
Neuroblastoma is the most common extracranial solid tumor of childhood originating from sympathetic nervous system cells. Neuroblastoma has also been diagnosed in conjunction with other congenital conditions such as Hirschsprung's disease, congenital hypoventilation disorder, and neurofibromatosis type 1. Wolf-Hirschhorn syndrome is a congenital disorder caused by microdeletion of short arm of chromosome 4 encoding MSX1 gene with characteristic facial features. We describe a child with dysmorphic features, developmental delay, mental retardation who developed neuroblastoma at 2 years of age and cytogenetic analysis of blood lymphocytes revealed an interstitial deletion of 4p(15,2)...
January 12, 2017: Journal of Pediatric Hematology/oncology
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