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Pediatric Hematology and Oncology

Dmitrijs Rots, Madara Kreile, Sergejs Nikulshin, Zhanna Kovalova, Linda Gailite
Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Modern treatment protocols allow achievement of long-term event-free survival rates in up to 85% of cases, although the treatment response varies among different patient groups. It is hypothesized that treatment response is influenced by the IL15 gene variations, although research results are conflicting. To analyze IL15 gene variations influence treatment response, clinical course and the risk of developing ALL we performed a case-control and family-based study...
March 12, 2018: Pediatric Hematology and Oncology
Elif Guler, Nilgun Col, Mithat Buyukcelik, Ayse Balat
AIM: In recent years, survival rates of childhood cancers have significantly increased, and occurrence of long-term adverse late effects (eg, insulin resistance, diabetes mellitus, metabolic syndrome, hypertension) has become increasingly important. Early diagnosis of obesity/hypertension in childhood is essential to avoid morbidity in the adulthood. Therefore, this study was aimed to determine the blood pressure (BP) profile by ambulatory BP monitoring (ABPM) method, and prevalence of hypertension, obesity, abdominal obesity among childhood cancer survivors...
March 12, 2018: Pediatric Hematology and Oncology
Laura Illade, Blanca Molina Angulo, Ines Solís, Miguel Ángel Diaz, Marta Gonzalez-Vicent
No abstract text is available yet for this article.
February 12, 2018: Pediatric Hematology and Oncology
Noa Granot, Ayelet Ben-Barak, Yael Fisher, Hana Golan, Myriam Weyl Ben-Arush
Nodular sclerosing Hodgkin lymphoma (HL) has an excellent prognosis in children. The syncytial variant (SV) of HL in adults represents a clinic pathologic entity with a worse outcome. We report the clinical features and the course of the disease of three children with refractory HL. The three patients with SV were analyzed in a retrospective multi-institutional study conducted in Israel in 51 children diagnosed with refractory or recurrent HL between 1997 and 2014. All the three children developed multiple recurrences soon after diagnosis...
February 8, 2018: Pediatric Hematology and Oncology
Amol Patel, Meher Chand Sharma, Saumyaranjan Mallick, Manali Patel, Sameer Bakhshi
BACKGROUND: Advanced stage is a known prognostic factor in B-Non-Hodgkin Lymphoma (NHL); however, factors within advanced stage and overall data on pediatric B-NHL from India are lacking. METHODS: This is a retrospective study wherein all consecutive pediatric (≤18 years) patients of advanced stage B-NHL (St. Jude stage 3 and 4) treated at our center from Jan 2003 to June 2016 with BFM-90 protocol were evaluated for outcome and pathology review. RESULTS: Total 140 patients were analyzed with median age 8 years; M:F ratio was 5...
February 1, 2018: Pediatric Hematology and Oncology
Prapai Dejkhamron, Karn Wejaphikul, Tuanjit Mahatumarat, Suchaya Silvilairat, Pimlak Charoenkwan, Suwit Saekho, Kevalee Unachak
BACKGROUND: Vitamin D deficiency is common in patients with thalassemia. Vitamin D deficiency could be related to cardiac dysfunction. Increased parathyroid hormone (PTH) is also known to be associated with heart failure. OBJECTIVES: To determine the prevalence of Vitamin D deficiency and to explore the impact of Vitamin D deficiency on cardiac iron and function in patients with transfusion-dependent thalassemia. METHOD: A cross-sectional study in patients with Transfusion-dependent thalassemia was conducted...
January 23, 2018: Pediatric Hematology and Oncology
Gitika Pant, Nishant Verma, Archana Kumar, Vishal Pooniya, Sanjiv Kumar Gupta
Retinoblastoma (RB) is the most common ocular malignancy in children, and is managed by multimodal treatment. There is a paucity of data regarding the clinical profile and outcome of children with extraocular retinoblastoma from Low Middle Income Countries (LMIC) including India. Case records of children with newly diagnosed extraocular RB from January 2013 to August 2016 treated at our unit were analysed for clinical profile, treatment, and outcome. Over the 44 month study period, 91 children were diagnosed with RB, out of which 41 had extraocular disease...
January 16, 2018: Pediatric Hematology and Oncology
Kun-Yin Qiu, Xiong-Yu Liao, Ruo-Hao Wu, Ke Huang, Jian-Pei Fang, Dun-Hua Zhou
X-linked hyper-immunoglobulin M (IgM) syndrome is characterized by recurrent infections, low or undetectable levels of IgG and IgA, and normal to increased serum IgM, and is also rare. It is associated with mutation in the gene encoding CD40 ligand. This study aimed to describe the first international report of hemizygous CD40LG c.542G>A mutation in a 5-year-old boy with a phenotype of Crohn's disease and hemophagocyticlymphohistiocytosis. Also, the clinical implications of this mutation and associated atypical phenotype are discussed...
January 5, 2018: Pediatric Hematology and Oncology
Hong Yul An, Kyung Taek Hong, Hyoung Jin Kang, Jung Yoon Choi, CheRy Hong, Hyun-Young Kim, Tae Hyun Choi, Chang Hyun Kang, Han-Soo Kim, Jung-Eun Cheon, Sung-Hye Park, June Dong Park, Kyung Duk Park, Hee Young Shin
Malignant peripheral nerve sheath tumors are rare tumors that originate from Schwann cells. Patients with neurofibromatosis type 1 are prone to develop these tumors. Due to their rarity and lack of established treatment, the prognosis of malignant peripheral nerve sheath tumors is poor. A retrospective study was conducted on children treated for malignant peripheral nerve sheath tumors at the Seoul National University Children's Hospital between 2007 and 2016. Eleven patients were diagnosed with malignant nerve sheath tumors at a median age of 12 years, eight of whom had neurofibromatosis type 1...
December 29, 2017: Pediatric Hematology and Oncology
Monica Khurana, Ellen B Fung, Elliott P Vichinsky, Elizabeth C Theil
Transfusion-independent patients with thalassemia intermedia (TI) develop fatal iron overload from excessive iron absorption triggered by ineffective erythropoiesis. More information about iron pharmacokinetics and nonheme, dietary iron absorption in such patients is needed to optimize management. To obtain more information, different forms of supplemental nonheme iron sources (ferritin and ferrous sulfate) were compared in 4 TI (hemoglobin <9 g/dL) and 6 control (hemoglobin 12-16 g/dL) patients. Serial serum iron concentrations were measured during the 24 hours following consumption of 1 mg/kg of elemental iron as ferritin or ferrous sulfate...
December 12, 2017: Pediatric Hematology and Oncology
Frank Berthold, Marc Hömberg, Inna Proleskovskaya, Pavel Mazanek, Margarita Belogurova, Angela Ernst, Jaroslav Sterba
The metronomic therapy concept uses low doses of continuously applied chemotherapeutic, anti-angiogenetic, and immunomodulating drugs. Twenty patients with recurrent and 3 with refractory high-risk neuroblastoma were treated by the metronomic concept using celecoxib, cyclophosphamide, vinblastine, and etoposide for up to 24 months. The outcome was compared to 274 matched patients with a first recurrence from stage 4 neuroblastoma using the variables time from diagnosis to first recurrence, number of organs involved, and MYCN amplification...
November 17, 2017: Pediatric Hematology and Oncology
Alan S Wayne, Erika Shin-Kashiyama, Richard Sposto, Paul Gaynon
Despite great success in the development of curative therapies for pediatric hematologic malignancies, new approaches are needed to overcome resistance to treatment and to reduce associated side effects. The Therapeutic Advances in Childhood Leukemia and Lymphoma (TACL) Consortium is an early phase clinical trial group dedicated to developing innovative therapies for currently incurable pediatric leukemias and lymphomas ( ). In November of 2016, a TACL Investigator Meeting was held, the proceedings of which appear in this edition of Pediatric Hematology Oncology...
November 9, 2017: Pediatric Hematology and Oncology
Ali H Algiraigri, Nicola A M Wright, Elizabeth Oddone Paolucci, Aliya Kassam
OBJECTIVE: Chronic blood transfusion remains the most feasible therapeutic option for lifelong transfusion-dependent β-thalassemia (lifelong TDβT). However, it is associated with serious risks and complications. Hydroxyurea (HU), an oral chemotherapeutic drug, is expected to increase hemoglobin levels, thereby minimizing the burden of blood transfusion and its complications. Growing literature over the last twenty years suggests promising results of the use HU in lifelong TDβT; however, its role and safety remain unanswered questions...
November 2017: Pediatric Hematology and Oncology
Anne-Charlotte Teyssier, Hélène Lapillonne, Marlene Pasquet, Paola Ballerini, André Baruchel, Stephane Ducassou, Odile Fenneteau, Arnaud Petit, Wendy Cuccuini, Christine Ragu, Claude Preudhomme, Thomas Mercher, Nicolas Sirvent, Guy Leverger
We report the outcome of 27 children with de novo acute megakaryoblastic leukemia (AMKL) (excluding Down syndrome) enrolled in the French multicenter prospective study ELAM02 (2005-2011). There was no difference in gender, initial leukocyte count, CNS involvement, and complete remission rate (88.9%), as compared to other acute myeloid leukemia (AML) subtypes. AMKL patients had a significantly poorer outcome (5-year overall survival 54% [CI 95% 33%-71%] than children with other AML subtypes (5-year overall survival 73% [CI 95% 68%-77%] p = 0...
November 2017: Pediatric Hematology and Oncology
Ahmar U Zaidi, Prabhakar Kedar, Prasad Rao Koduri, Gerard W Goyette, Steven Buck, Donald E Paglia, Yaddanapudi Ravindranath
After a thirty-year lag, we serendipitously reestablished contact with a patient with glucose phosphate isomerase deficiency and hydrops fetalis first reported in 1987. We now provide a clinical update and provide results of mutation analysis in this patient, from Southern India. The patient now an adult female of 36 years of age has moderate anemia but requires no transfusions except with some intercurrent illnesses. Exome sequencing studies showed a homozygous c.1018C>T (Pro340Ser) mutation in exon 12 of the glucose phosphate isomerase gene and later confirmed by direct sequencing...
November 2017: Pediatric Hematology and Oncology
Majid Naderi, Iraj Shahramian, Mojtaba Delaramnasab, Ali Bazi
The association of celiac disease (CD) with cancers of gastrointestinal origin has been noted. However, coincidence of CD with nongastrointestinal neoplasms is an unusual event. Here we present five children with concurrent CD and nongastrointestinal neoplasms. All of the patients had positive serologic results for anti-tTG antibodies. Histological investigation of intestinal mucosa showed inflammation (Marsh score = 2) in all the patients. Two of these patients represented with germ cell malignancies. One patient had Wilms' tumor...
November 2017: Pediatric Hematology and Oncology
Edward Allan Racela Sison, Peter Kurre, Yong-Mi Kim
Signaling between leukemia cells and nonhematopoietic cells in the bone marrow microenvironment contributes to leukemia cell growth and survival. This complicated extrinsic mechanism of chemotherapy resistance relies on a number of pathways and factors, some of which have yet to be determined. Research on cell-cell crosstalk the bone marrow microenvironment in acute leukemia was presented at the 2016 annual Therapeutic Advances in Childhood Leukemia (TACL) investigator meeting. This review summarizes the mini-symposium proceedings and focuses on chemokine signaling via the cell surface receptor CXCR4, adhesion molecule signaling via integrin α4, and crosstalk between leukemia cells and the bone marrow microenvironment that is mediated through extracellular vesicles...
September 2017: Pediatric Hematology and Oncology
Kara L Davis, Archana M Agarwal, Anupam R Verma
Immune surveillance comprising of adaptive and innate immune systems is naturally designed to eliminate cancer development; overexpression of inhibitory receptors and their ligands prevent this check and lead to evasion and hence cancer progression and metastasis. The use of tumor-specific monoclonal antibodies (MAbs) targeting these checkpoint regulators is promising and has led to this novel field of cancer immunotherapy. The first antibody directed against cytotoxic T-lymphocyte associated protein 4 (CTLA-4), ipilimumab, showed promising results in clinical trials and was approved by the US Food and Drug Administration (FDA) for the treatment of metastatic melanoma in 2011...
September 2017: Pediatric Hematology and Oncology
E Orgel, J J Auletta
A Supportive Care Committee was recently developed within the Therapeutic Advances in Childhood Leukemia & Lymphoma (TACL) Consortium. This was substantiated by the significantly high rate of serious adverse events (SAE) (CTCAE Grade ≥3 toxicity) experienced by patients with relapse/refractory acute leukemia enrolled on our phase I trials. Such treatment-related toxicity has resulted in patients being removed from study and thus potentially not receiving clinical benefit from the novel therapy. In addition, increased treatment-related toxicity may compromise new agents from moving forward in their clinical development...
September 2017: Pediatric Hematology and Oncology
Deepa Bhojwani, Michael J Burke, Terzah Horton, David S Ziegler, Maria Luisa Sulis, Kirk R Schultz, Alan Wayne, Shai Izraeli, Bill H Chang
During the 2016 Therapeutic Advances for Childhood Leukemia & Lymphoma (TACL) Consortium investigators' meeting (Los Angeles, CA), a Biology Working Group was established to support the consortium's mission of developing innovative therapies for currently incurable childhood leukemias and lymphomas. The charge of the Biology Working Group was to address how TACL could advance biological investigations of pediatric relapsed/refractory hematologic malignancies while undertaking forward-looking therapeutic trials...
September 2017: Pediatric Hematology and Oncology
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