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Journal of Pediatric Hematology/oncology

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https://www.readbyqxmd.com/read/30334905/activated-phosphoinositide-3-kinase-delta-syndrome-apds-a-primary-immunodeficiency-mimicking-lymphoma
#1
Frederic Baleydier, Emmanuelle Ranza, Michela Schäppi, Anne-Laure Rougemont, Laura Merlini, Marc Ansari, Geraldine Blanchard-Rohner
Malignant or nonmalignant lymphoproliferative disorders together with repeated ear, nose, and throat infections should strongly motivate immunologic investigations. Indeed, we report a 7-year-old patient with a history of persistent abdominal symptoms along with recurrent ear, nose, and throat infections, who presented with intra-abdominal masses highly suggestive of a diagnostic of lymphoma, and who was diagnosed with activated-PI3K-delta syndrome, a recently described primary immunodeficiency prone to lymphoproliferation...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30334904/prevalence-and-clinical-correlations-of-somatostatin-receptor-2-sstr2-expression-in-neuroblastoma
#2
Natasha Alexander, Paula Marrano, Paul Thorner, Arlene Naranjo, Collin Van Ryn, Daniel Martinez, Vandana Batra, Libo Zhang, Meredith S Irwin, Sylvain Baruchel
Alternative radiolabeled, targeted agents are being investigated for children with relapsed neuroblastoma (NB) who do not respond to I-metaiodobenzylguanidine (MIBG) therapy. (DOTA-Tyr)-octreotate targets somatostatin receptors (SSTRs), particularly SSTR2, which are expressed on NB cells. We investigated SSTR2 expression in NB tumors (36 high-risk [HR]; 33 non-HR patients) and correlated SSTR2 levels with clinical features, norepinephrine transporter (NET) expression, and MIBG avidity. SSTR2 and NET immunohistochemistry scores (0 to 3) were calculated on biopsies using digital image analysis based on staining intensity and distribution...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30334903/young-adult-with-osteosarcoma-of-the-mandible-and-the-challenge-in-management-review-of-the-pediatric-and-adult-literatures
#3
Christopher Kuo, Paul M Kent
Neoadjuvant chemotherapy for osteosarcoma of the jaw (OSJ) remains controversial despite being a standardized treatment in osteosarcoma of the long bones. We present a case of a 22-year-old male with OSJ and performed a retrospective systemic review of previously published literatures of OSJ. We identified 27 articles: 7% recommended neoadjuvant chemotherapy, 22% recommended adjuvant chemotherapy, 19% recommended both neoadjuvant and adjuvant chemotherapy, 33% recommended against chemotherapy and 19% stated the role of chemotherapy is unknown...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30334902/serum-and-urinary-hepcidin-for-diagnosing-iron-deficiency-anemia-in-under-5-children
#4
Pooja Dewan, Anupriya Dixit, Sunil Gomber, Mrinalini Kotru, Basu D Banerjee, Vipin Tyagi, Rajeev K Malhotra
Bone marrow iron estimation remains the gold standard for diagnosing iron-deficiency anemia (IDA); serum ferritin, total iron-binding capacity, and transferrin saturation are routinely used as surrogate markers of IDA. However, these tests are marred by problems like poor specificity and sensitivity. Recently, hepcidin, a protein hormone synthesized in the liver and excreted in urine, has been shown to be related to iron status. We estimated the serum and urinary hepcidin levels in healthy children aged 6 to 60 months with (n=30) and without IDA (n=30)...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30334901/idiopathic-purpura-with-gray-platelets-an-acquired-form-of-gray-platelet-syndrome
#5
Anselm Chi-Wai Lee
An acquired, transient bleeding disorder that predominantly affects children in Southeast Asia has been reported for the last 4 decades. The condition has been named idiopathic purpura with gray platelets (IPGP) or acquired platelet dysfunction with eosinophilia. In a retrospective review from a private pediatric clinic over an 8-year period, 10 consecutive children were diagnosed as IPGP with a mean age of 8.4 (3.7 to 16.2) years. Eosinophilia (>0.5×10/L) was absent in 1, while gray platelets were consistently found in all cases with a mean proportion of 64...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30334900/long-term-complications-and-management-of-gastrointestinal-bleeding-in-multifocal-lymphangioendotheliomatosis
#6
Asiya K Shakir, Zhongxin Yu, Muhammad Adnan Altaf
Multifocal lymphangioendotheliomatosis with thrombocytopenia is a rare disease characterized by multiple cutaneous and gastrointestinal (GI) vascular lesions and thrombocytopenia refractory to platelet and blood cell transfusions. GI bleeding can become life-threatening in this condition. We report a case of multifocal lymphangioendotheliomatosis with thrombocytopenia in a male infant with isolated GI involvement, diagnosed when he was 3 months old. The patient was managed with daily aminocaproic acid, octreotide drip, and corticosteroids for 13 months after diagnosis; he had complete resolution of symptoms by 2 years of age and showed adequate height and gain by 5 years of age...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30334899/vandetanib-tumor-shrinkage-in-metastatic-medullary-thyroid-cancer-allowing-surgical-resection-of-the-primary-site-a-case-report
#7
Thomas D Milner, Milind Ronghe, Mohamad G Shaikh, Fiona B MacGregor, Nicholas Reed
Vandetanib has been shown to improve progression-free survival in adults with advanced medullary thyroid cancer. This article describes a pediatric patient with metastatic medullary thyroid cancer secondary to sporadic multiple endocrine neoplasia 2B, treated with vandetanib. At presentation, he had an inoperable primary tumor, with carotid encasement, and pulmonary metastases. Vandetanib induced a significant response: calcitonin and carcinoembryonic antigen levels both fell considerably, primary tumor maximal diameter decreased by 68%, and pulmonary metastases became no longer detectable...
October 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30303870/acute-megakaryoblastic-leukemia-with-diffuse-periosteal-reaction-of-bilateral-lower-extremities
#8
Erin E Doherty, Erica K Schallert, Alexandra M Stevens, Jyotinder N Punia, Jennifer H Foster
We report the case of a 3-year-old girl diagnosed with acute megakaryoblastic leukemia, who presented after >1 year of bilateral leg pain. At times the pain was severe enough to prevent ambulation, prompting visits to her primary care provider. However, it was not until acute respiratory failure occurred with subsequent hospitalization in the pediatric intensive care unit that severe anemia and thrombocytopenia were discovered and the diagnosis of acute myeloid leukemia was made. Bilateral lower extremity swelling was noted on admission and radiographs showed diffusely abnormal appearance of the long bones of her lower extremities with periosteal reaction and echogenic debris in the subperiosteal space, thought to represent leukemic cells...
October 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30303869/osteosarcoma-in-a-child-below-2-years-of-age-case-report-and-review-of-the-literature
#9
Edward M Pascoe, Matthew Free, Peter S Mackie, Leo Donnan, Mark O'Sullivan, Michael J Sullivan, John A Heath
BACKGROUND: Osteosarcoma in children below the age of 5 is extremely rare. OBSERVATION: We report on a previously well 14-month-old male infant, who presented with a reluctance to weight-bear on his right leg and had an associated limp. Plain imaging and a magnetic resonance imaging scan demonstrated a lytic lesion in the right distal femur. An open surgical biopsy confirmed the diagnosis of osteosarcoma. There was no significant family history of cancer and genetic screening for Li-Fraumeni syndrome was negative...
October 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30299352/use-of-tranexamic-acid-in-an-uncommon-triad-hemorrhagic-stroke-sickle-cell-disease-and-severe-coagulopathy
#10
Evan Roter, Gianluca Bertolizio, Vincent Collard, Catherine Paquet, Pablo Ingelmo
No abstract text is available yet for this article.
October 5, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30299351/successful-treatment-of-an-adolescent-male-with-severe-refractory-evans-syndrome-using-bortezomib-based-therapy
#11
Tristan Knight, Yaddanapudi Ravindranath, Michael U Callaghan
Evans syndrome is defined by bilineal autoimmune cytopenia, typically coombs positive hemolytic anemia and thrombocytopenia. Corticosteroids are the mainstay of treatment, with rituximab and/or mycophenolate mofetil often used in steroid-refractory cases. However, no treatment methodology has ever evaluated by a randomized clinical trial. We present a 15-year-old boy with Evans syndrome and common variable immunodeficiency who experienced a severe, refractory flare 16 months postsplenectomy. After failing to respond to multiple other agents, he achieved a durable response to a bortezomib-based regimen...
October 5, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30299350/gray-zone-lymphoma-arising-in-the-neck-of-a-teenager-with-a-germline-mutation-in-tp53
#12
Sophie Gatineau-Sailliant, Karine Turcotte, Marie-Claude Quintal, Sophie Turpin, Josette Champagne, Tony Petrella, Mathieu Roussy, Sonia Cellot, Dorothée Bouron-Dal Soglio
Gray zone lymphoma is an aggressive disease for which appropriate management is still debated. We report a 15-year-old girl with a cervical mass, an enlarged ipsilateral tonsil, and anemia. Both sites showed hypermetabolism on F18-FG positron emission tomography/CT. Surgical resection was diagnostic of Epstein-Barr virus-negative gray zone lymphoma cervical and tonsillar involvement. No abnormality was found in cytogenetic analysis on tumor cells. However, exome sequencing in peripheral blood DNA revealed a germline mutation in TP53...
October 5, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30299349/not-all-pediatric-bladder-masses-are-rhabdomyosarcoma-report-of-a-rare-case-with-review-of-the-literature
#13
Suvradeep Mitra, Rashmi Joshi, Kirti Gupta, Jai Kumar Mahajan
Leiomyoma of the urinary bladder in the pediatric age group is one of the rarest tumors with only 2 case reports in the English literature. Rhabdomyosarcoma is the commonest tumor at this site in this age group. It needs to be distinguished from rhabdomyosarcoma by histomorphology for appropriate treatment and management. We report the third case of urinary bladder leiomyoma arising in a 6-year-old boy with a short review of the literature.
October 5, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30299348/burkitt-lymphoma-presenting-with-intracardiac-mass-and-tumor-thrombosis-in-the-anterior-mediastinum-with-literature-review
#14
Ayça Koca Yozgat, Derya Ozyoruk, Dilek Kacar, Ibrahim I Cetin, Inci Yaman Bajin, Kubilay Inan, Ayse S Oguz
Burkitt lymphoma manifesting as an intracardiac mass is a rare entity. This report describes the case of a 10-year-old boy who presented with an intracardiac mass and tumor thrombosis in the anterior mediastinum that proved to be Burkitt lymphoma. The LMB-96 chemotherapy protocol was given and at the end of the treatment there was still residual mass. A biopsy was performed and the pathology revealed thymus tissue. The patient has been in complete remission for 3 months. Burkitt lymphoma has a short doubling time and an intracardiac lesion can become life threatening...
October 5, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30281526/chemoradiotherapy-for-unresectable-ini1-negative-chordoma-in-a-child
#15
Hiroyuki Miyahara, Seishiro Nodomi, Katsutsugu Umeda, Satoshi Itasaka, Kenji Waki, Tsuyoshi Imai
The characteristics of chordomas in children are distinct from those in adults. In particular, the prognosis of patients with INI1-negative chordoma is dismal. The standard treatment for localized chordoma, complete surgical resection with a wide margin, is seldom feasible for chordomas arising at the clivus in children, mainly due to associated complications. Therefore, other treatments for unresectable chordomas in children, including chemoradiotherapy, must be explored. Here, we report a 7-year-old girl with an INI1-negative chordoma of the clivus, who responded to conventional chemotherapy plus radiotherapy...
October 2, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30273225/testis-involvement-in-neuroblastoma-report-of-3-cases-in-the-turkish-pediatric-oncology-group-neuroblastoma-study-and-review-of-the-literature
#16
Rejin Kebudi, Begum Sirin Koc, Ferhan Akici, Fatma Betul Cakir, Omer Gorgun, Nur Olgun
BACKGROUND: Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. Primary and secondary testicular involvement is extremely uncommon in neuroblastoma. PROCEDURE: All children with neuroblastoma treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma (NB) Study and who had testis involvement either at diagnosis or at relapse were retrospectively evaluated. A review of all cases with neuroblastoma and testis involvement in the literature was done...
September 29, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30256265/proliferative-cells-from-kaposiform-lymphangiomatosis-lesions-resemble-mesenchyme-stem-cell-like-pericytes-defective-in-vessel-formation
#17
Kathryn Glaser, Peter Dickie, Belinda Hsi Dickie
Kaposiform lymphangiomatosis (KLA) is a vascular anomaly featuring lymphatic expansion. It has no known cause, no effective treatment, and is associated with high morbidity. Proliferative cells from 3 KLA patient lesions were characterized relative to adiopose-derived mesenchyme stem cells (ADSCs) and cells derived from a patient with the related disease kaposiform hemangioendothelioma (KHE). KLA cells variably expressed markers of mesenchyme stem cells (CD73, CD90, CD105, CD146) and lacked endothelial cell markers (CD31, CD34) as determined by flow cytometry...
September 25, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30247183/outcome-of-high-risk-langerhans-cell-histiocytosis-lch-in-egyptian-children-does-intermediate-dose-methotrexate-improve-the-outcome
#18
Mohamed S Sedky, Asmaa Hamouda, Hala Taha, Iman Zaky, Omayma Hassanain, Ahmed El Hemaly, Alaa ElHaddad
High-risk multisystem organ (RO+) Langerhans cell histiocytosis (LCH) has the least survival. We present the outcome of RO+ LCH in a pediatric single center. Fifty RO+ LCH patients, treated between 07/2007 and 07/2015, were retrospectively analyzed. Induction vinblastine (VBL) and prednisone (PRED) with intermediate-dose methotrexate (idMTX) was adopted until 2012 (n=20) wherein idMTX was omitted (n=30). The 3-year overall survival (OS) of MTX and non-MTX groups was 75% and 63%, respectively, P=0.537, while the event-free survival (EFS) was 36...
September 21, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30222642/early-testing-of-serum-ferritin-facilitates-hemophagocytic-lymphohistiocytosis-diagnosis-in-children
#19
Hemant S Agarwal, John F Kuttesch
The clinical and laboratory features of hemophagocytic lymphohistiocytosis (HLH) are nonspecific that makes the definitive diagnosis of HLH very challenging. The disease is almost universally fatal in the absence of early recognition and appropriate therapy. Elevated serum ferritin level is one of the diagnostic markers of HLH disease. We report the value of testing serum ferritin level early in the disease process in 3 pediatric patients who presented with persistent fever and sepsis-like features. Detection of elevated serum ferritin levels facilitated further testing to confirm the diagnosis of HLH and initiate early therapy with good outcomes...
September 15, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30212417/childhood-and-adolescent-cancer-in-the-state-of-pernambuco-brazil-incidence-geographical-distribution-and-association-with-environmental-health-indicators
#20
Norma Lucena-Silva, Lídia N V Bastos, José C Silveira, Carlos F Luna
No abstract text is available yet for this article.
September 12, 2018: Journal of Pediatric Hematology/oncology
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