Cardiac Toxicity After Craniospinal Irradiation: A Late Effect That May be Eliminated With Proton Therapy
Conclusions: Spinal irradiation can cause significant cardiac risk due to exit dose through anterior structures. This toxicity may be avoided with proton therapy, which eliminates visceral exit dose. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm Mimicking Acute Rheumatic Fever: Report of an Unusual Clinical Presentation and Review of Literature
Conclusions: The case is reported due to its unique unusual clinical presentation and its rarity in pediatric population. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

The Challenge of Next Generation Sequencing in a Boy With Severe Mononucleosis and EBV-related Lymphoma
We describe the case of a boy with severe mononucleosis accompanied by the development of hemophagocytic lymphohistiocytosis and lymphoma. By whole exome sequencing, we identified a mutation of uncertain significance in CTPS2, a gene closely related to CTPS1, which is involved in a primary immune deficiency with susceptibility to herpesviruses. We discuss the challenge of a correct interpretation of data from whole exome sequencing, questioning whether the CTPS2 variant found in our patient is just an incidental finding or a mutation with variable penetrance. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Clinical Challenges: Identification of Patients With Novel Primary Immunodeficiency Syndromes
We describe 1 patient with cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) haploinsufficiency who had recurrent enhancing brain lesions, nodular pulmonary infiltrates, hepatosplenomegaly, immune cytopenias, as well as progressive hypogammaglobulinemia and lymphopenia. We describe a second patient with activated p110δ syndrome (APDS)/p110δ activating mutation causing senescent T cells, lymphadenopathy, and immunodeficiency (PASLI) in association with recurrent respiratory tract infections, Epstein-Barr virus infection, lymphadenopathy, elevated serum IgM, and progressive lymphopenia. These presentations hig...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Complete Resolution of Lymphoid Interstitial Pneumonia in a Patient With Juvenile Myelomonocytic Leukemia Treated With Allogeneic Bone Marrow Transplant: Killing 2 Birds With 1 Stone
We report the first case of LIP in a patient with juvenile myelomonocytic leukemia (JMML) who was found to have a novel germline mutation of unknown significance in additional sex combs-like-1 (ASXL1) gene and a pathogenic somatic mutation of protein tyrosine phosphatase, nonreceptor type 11 (PTPN11) gene at diagnosis. The patient underwent a matched unrelated bone marrow transplant for JMML with complete resolution of JMML and LIP with no recurrence to date. We also emphasize the importance of considering LIP in differential diagnosis of pulmonary lesions seen in conjunction with hematologic malignancies and distinguishin...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Successful Treatment of Mediastinal γδ T-Cell Lymphoblastic Lymphoma in a 3-Year-Old Girl by Allogeneic Cord Blood Transplantation
A 3-year-old girl presenting with a huge mediastinal tumor and massive pleural effusion, was diagnosed with stage III γδ T-cell lymphoblastic lymphoma (γδ T-LBL) by flow cytometry of effusion cells. Four courses of chemotherapy achieved complete remission, and 5/6 HLA allele-matched cord blood transplantation was performed with success. The patient remains in first complete remission>16 months after transplantation. γδ T-LBL is very rare, especially that of mediastinal origin, and is acknowledged as having an extremely poor clinical outcome. The present case study is the first to repo...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Toxicity of Vincristine Combined With Posaconazole in Children With Acute Lymphoblastic Leukemia
Conclusions: There are limited data in children with ALL for posaconazole prophylaxis. We recommend that VCR side effects should be evaluated by careful monitoring of the patients who are on this combination therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Autoimmune Lymphoproliferative Syndrome Masquerading as Posttransplant Lymphoproliferative Disorder
We present a case of a 2-year-old female presenting with diffuse lymphadenopathy 2 years following orthotopic heart transplant. Initially, she was diagnosed with posttransplant lymphoproliferative disease based on clinical presentation and pathology and she was treated accordingly. Because of persistent lymphadenopathy following the completion of chemotherapy and new onset of autoimmune cytopenias, repeat flow of the lymph node showed an elevated double negative T-cell population prompting evaluation for autoimmune lymphoproliferative syndrome (ALPS). A complete workup was confirmative of a germline Fas mutation consistent...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Novel Variations of FANCA Gene Provokes Fanconi Anemia: Molecular Diagnosis in a Special Chinese Family
This study not only shows the complexity of FA molecular diagnosis via comprehensively studying the FA pathogenic genes and the mutational spectrum, but also has significant reference value for the future molecular diagnosis of FA. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Induction Dosage of Propofol for Repeated Sedations in Children With Hematological Disorders
Pediatric patients with hematologic malignancies require several procedural sedations by means of propofol infusion. We retrospectively analyzed the medical records of leukemic pediatric patients who had undergone procedural sedations at an Italian tertiary referral center (San Gerardo Hospital, Monza) from January 2011 to November 2013. We retrieved the following: demographics; diagnosis; chemotherapy phase; use of corticosteroids; induction dosage of propofol, fentanyl and/or ketamine; and the type of procedure. We used a multivariate linear mixed model to evaluate the factors affecting induction propofol dose. We analyz...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

SLCO1B1 Polymorphisms are Associated With Drug Intolerance in Childhood Leukemia Maintenance Therapy
Conclusions: SLCO1B1 rs4149056 and rs11045879 polymorphisms may be important genetic markers to individualize 6-MP/MTX doses. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Utility of Echocardiography as Screening for Late-onset Anthracycline-induced Cardiotoxicity in Pediatric Cancer Survivors: Observations from the First Decade After End of Therapy
Current screening guidelines are available for anthracycline-induced cardiotoxicity. However, the utility of echocardiogram screening for late-onset anthracycline cardiotoxicity especially in the decade immediately after end of therapy is debatable. A retrospective chart review of patients seen in the Thriving after Cancer Clinic at Rady Children’s Hospital January 2006 to December 2013 was performed. Treatment data, echocardiogram results, cardiology referral notes and cardiac medication data were abstracted from anthracycline-exposed survivors. Descriptive and univariate comparative statistics were performed. Of 36...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Safety of Dexamethasone for Nausea and Vomiting Prophylaxis in Children Receiving Hematopoietic Stem Cell Transplantation
Conclusions: In children undergoing HSCT who received dexamethasone for CINV prophylaxis, immediate AEs attributable to dexamethasone were most often of minor clinical importance and transient. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Vincristine-associated Neuropathy With Antifungal Usage: A Kaiser Northern California Experience
The dose-limiting toxicity for vincristine is peripheral neuropathy which can be potentiated with concurrent usage of azole antifungals. The current retrospective study assessed the incidence of concurrent vincristine and azole antifungal usage to determine if it led to increased neurotoxicity for the Kaiser Northern California pediatric acute lymphoblastic leukemia (ALL) and Hodgkin lymphoma patient population. Data were obtained from the electronic medical record (2007 to 2014). In total, 130 subjects received at least one dose of vincristine for ALL or Hodgkin lymphoma (median age 9, 88% ALL, 58% male, 47% Caucasian). T...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Characterization of 4 New Mutations in the CYBB Gene in 10 Iranian Families With X-linked Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) is an inherited disease of the innate immune system that results from defects in 1 of the 5 subunits of nicotinamide adenine dinucleotide phosphate oxidase complex and leads to life-threatening infections with granuloma formation. During 3 years of study, we recognized 10 male patients with X-linked CGD from a tertiary referral center for immune deficiencies in Iran. The CGD patients were diagnosed according to clinical features and biochemical tests, including nitroblue tetrazolium and dihydrorhodamine-1, 2, 3 tests, performed on patients and their mothers. In all patients, Western blot...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Giant Cell Tumor of the Ribs and Aneurysmal Bone Cyst Presenting with Hemothorax in a Child: Erratum
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Erratum Source Type: research

Sickle Cell Trait Testing Should Not Be a Player in NCAA Athletics: Examining the Media’s Role in Disseminating Awareness and Information
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Letters to the Editor Source Type: research

The Moonshot Initiative: Randomized, Controlled Trials, and Its Impact in Pediatric Oncology
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Letters to the Editor Source Type: research

Clinical Course of Homozygous Hemoglobin Constant Spring in Pediatric Patients
Background: Hemoglobin (Hb) Constant Spring is an alpha-globin gene variant due to a mutation of the stop codon resulting in the elongation of the encoded polypeptide from 141 to 172 amino acid residues. Patients with homozygous Hb Constant Spring are usually mildly anemic. Methods: We retrospectively describe clinical manifestations, diagnosis, laboratory investigations, treatment, and associated findings in pediatric patients with homozygous Hb Constant Spring followed-up at Srinagarind Hospital. Results: Sixteen pediatric cases (5 males and 11 females) were diagnosed in utero (N=6) or postnatal (n=10). Eleven ca...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Fetal Anemia Causing Hydrops Fetalis From an Alpha-Globin Variant: Homozygous Hemoglobin Constant Spring
We report 7 cases with fetal anemia causing hydrops fetalis. Serial Doppler ultrasound for measurement peak systolic velocity (PSV) of middle cerebral artery (MCA) was used for evaluation of fetal anemia. Fetal anemia is suggested if the MCA/PSV ratio is>1.5 multiple of median. Cordocentesis was performed subsequently to find the cause of fetal anemia and check fetal hemoglobin for consideration of intrauterine infusion. Investigations for fetal anemia include complete blood count, blood morphology, and blood group of mother and fetus, reticulocyte counts, red cell indices, screening for thalassemia, hemoglobin typing, ...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Successful Management of a Rare Cause of Hemolytic Uremic Syndrome With Eculizumab in a Child
Conclusion: In conclusion, eculizumab may be a treatment of choice to prevent further systemic damage in recurrent HUS episodes of patients with borderline changes in the bone marrow until ALL is constantly diagnosed. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Iron Overload in Survivors of Childhood Cancer
We describe iron overload (ferritin>500 ng/mL) prevalence and identify risk factors in CCS. Observations: Of 116 participants, 3 (2.6%) had elevated ferritin. All were teenagers at cancer diagnosis and received>8000 mL PRBC. Total PRBC volume correlated best with elevated ferritin (r=0.74; P (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Hemophagocytic Lymphohistiocytosis Associated With Visceral Leishmaniasis
This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol. Repeated bone marrow aspiration showed amastigotes on follow-up. In endemic countries, visceral leishmaniasis should be considered in the differential diagnosis to avoid chemotherapy toxicity. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Morphology Corner Source Type: research

Cancer Management in Kabuki Syndrome: The First Case of Wilms Tumor and a Literature Review
A 3-year-old Japanese girl treated for hypoplastic left heart syndrome and Dandy-Walker syndrome was diagnosed with Kabuki syndrome (KS) with a mutation of KMT2D; c.13285C>T:p.Q4429*. Concurrently, macrohematuria portended the diagnosis of Wilms tumor. Postoperative chemotherapy has achieved complete remission despite a prolonged and reduced regimen due to liver dysfunction and convulsions. Cancer predisposition has been suggested for KS due to oncogenic mutations in KMT2D or KDM6A. The first case of nephroblastoma exemplified the treatability of malignancies in KS patients, as shown in the 9 cases reviewed. Active scre...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Excessive Treatment Failures in Patients With Parameningeal Rhabdomyosarcoma With Reduced-dose Cyclophosphamide and Delayed Radiotherapy
We report our early experience on a multi-institutional prospective trial incorporating delayed primary site radiation and reduced dose cyclophosphamide in all patients with parameningeal tumors. We observed an excessive number of locoregional treatment failures following this approach and have subsequently amended our trial to move radiation therapy upfront for those patients with high-risk features. We suggest that investigators should be vigilant for treatment failure given our early prospective experience with delayed radiotherapy and reduced dose cyclophosphamide. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Can Pediatric Risk of Mortality Score (PRISM III) Be Used Effectively in Initial Evaluation and Follow-up of Critically Ill Cancer Patients Admitted to Pediatric Oncology Intensive Care Unit (POICU)? A Prospective Study, in a Tertiary Cancer Center in Egypt
Pediatric Risk of Mortality Score (PRISM III-12) is a physiology-based predictor for risk of mortality. We conducted prospective study from January 1, 2014 to 2015 in pediatric oncology intensive care unit (POICU) at South Egypt Cancer Institute, Egypt to explore the ability of 1st PRISM III-12 to predict the risk of mortality in critically ill cancer patients and the ability of serial PRISM III measured every 72 hours to follow-up the patients’ clinical condition during POICU stay. In total, 123 (78 males) children were included. Median age was 5 years (1 to 15 y). Death rate was 20%. 1st PRISM III-12 mean wa...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Kidney-protective Effect of Magnesium Supplementation in Cisplatin-containing Chemotherapy for Pediatric Cancer: A Retrospective Study
In total, 158 chemotherapy courses containing cisplatin for 37 pediatric cases of newly diagnosed cancer were divided into 2 groups depending on whether magnesium (Mg) supplementation was administered (Mg+: 92 courses) or not (Mg−: 66 courses). Renal impairment was defined as grade 2 or higher creatinine elevation (CE) after each chemotherapy course. The incidence of CE in the Mg+ was significantly lower than in the Mg− (9.8% vs. 22.7%; P=0.025). Multivariate analysis revealed that Mg supplementation significantly reduced the incidence of CE (odds ratio, 0.36; confidence interval, 0.13-0.99). In pediatric patie...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Acid-suppressing Drugs and a Low 1 Level of Antithrombin as Risk Factors for L-Asparaginase-associated Pancreatitis: A Case-control Study in the Japan Association of Childhood Leukemia Study (JACLS)
We examined correlations between AAP development and clinical symptoms, laboratory data, and concomitant medication. Abdominal pain and nausea were common presenting symptoms for AAP. There was an increased risk of AAP in patients using gastric acid-suppressing agents and antithrombin (AT) supplementation. Mean fibrinogen and AT levels before the onset of AAP were lower in AAP patients than in controls. Decreased AT and fibrinogen levels resulting from the strong suppression of protein synthesis by L-asparaginase were predictive signs for AAP. Our epidemiological approach should prove clinically useful for the diagnosis th...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Cold Pressor Pain Response in Children With Cancer
Conclusions: This study provides data on experimental pain responses in a sample of children undergoing cancer treatment and suggests that pain experience may be moderated by cancer treatment type. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Pentoxifylline Added to Steroid Window Treatment Phase Modified Apoptotic Gene Expression in Pediatric Patients With Acute Lymphoblastic Leukemia
Pentoxifylline is a xanthine that possesses antitumor properties and that can induce higher apoptosis in the leukemic cells of pediatric patients with acute lymphoblastic leukemia (ALL) during treatment with prednisone. We conducted a phase 1 pilot, controlled, randomized trial to evaluate the gene expression modified by pentoxifylline during the steroid window of induction to remission phase in patients newly diagnosed with ALL. Experimental and control treatments induced broad changes in the gene expression profile. Patients who received just prednisone upregulated 377 and downregulated 344 genes, in contrast with patien...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Hematologic Findings of Inherited Metabolic Disease: They are More Than Expected
Inherited metabolic diseases are pathologic conditions that generally develop as a result of impairment of the production or breakdown of protein, carbohydrate, and fatty acids. Early determination of hematological findings has a positive effect on the prognosis of metabolic diseases. Three hundred eighteen patients who were being followed-up within the previous 6 months at Department of Pediatric Nutrition and Metabolism, Gazi University, Turkey, were included in the study. The hematological findings were classified under 7 main groups: anemia of chronic disease, iron deficiency anemia, vitamin B12 deficiency anemia, hemo...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Muscle Strength, Power, and Torque Deficits in Children With Type SS Sickle Cell Disease
In African-American children aged 5 to 17 years with and without type SS sickle cell disease (SCD-SS), dominant hand maximal handgrip strength, peak power, and plantar flexion isometric maximal voluntary contraction (MVC) torque were compared with adjustments for body size and composition. Children with SCD-SS (n=21; age, 11±1 y) compared with healthy control children (n=23; 10±1 y) did not differ by age, sex, or maturation stage, but had significantly lower Z scores for height, weight, body mass index, arm circumference, upper arm muscle area, and lean mass-for-height. Children with SCD-SS had ...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

A Retrospective Analysis of Sociodemographic and Hematologic Characteristics Associated With Achieving Optimal Hydroxyurea Therapy in Children With Sickle Cell Disease
Hydroxyurea (HU) has proven hematologic and clinical benefits, especially when escalated to the maximum tolerated dose (MTD). We reviewed clinical data from patients with sickle cell disease (January 2011 to 2016) to determine baseline sociodemographic and laboratory parameters associated with reaching HU MTD without significant delays. In total, 210 patients (mean HU start age, 6.6 y) were included. Initial Kaplan-Meier event analysis showed 1 year to be an inflection point for reaching MTD. In total, 116 patients (55%) reached MTD in 1 year to reach MTD and 38 (18%) patients not successfully reaching MTD during fo...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Cutaneous Hemorrhage Types as Supportive Factors for Predicting Chronic Immune Thrombocytopenia in Children
Our objective was to assess risk factors for developing chronic immune thrombocytopenia (ITP) in children. The charts of all consecutive children diagnosed with ITP between 2000 and 2015 at a single center were retrospectively reviewed, and clinical characteristics at initial presentation were analyzed. Sixty-two children were included in the study (mean age, 6.15 y); 44 (71%) were found to have acute ITP, and 18 (29%) developed chronic ITP (permanent or relapsing thrombocytopenia>12 mo). In a univariate analysis, cutaneous hemorrhages were observed significantly more in acute patients (90.9%) than in chro...
Source: Journal of Pediatric Hematology Oncology - June 26, 2018 Category: Hematology Tags: Original Articles Source Type: research

Drug Rash, Eosinophilia, Systemic Symptoms (DRESS) in a Child With Acute Lymphoblastic Leukemia
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Letter to the Editor Source Type: research

Thiamylal Plus Pentazocine Shows Similar Efficacy as Ketamine Plus Midazolam for Painful Procedures in Children With Leukemia
This retrospective study compared the use of thiamylal plus pentazocine (TP) to ketamine plus midazolam (KM) in children with leukemia who were undergoing bone marrow aspiration and/or intrathecal chemotherapy. A total of 268 procedures in 35 children with leukemia were retrospectively analyzed for efficacy and adverse events. All procedures were successfully completed without severe adverse events. TP induced significantly faster sedation. The incidents of desaturation were significantly greater in the TP group, but were transient and recovered by oxygen supplementation alone. Therefore, TP can be a useful combination wit...
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Mesenteric Primitive Myxoid Mesenchymal Tumor in a 7-Month-Old Boy
We present a case of a 7-month-old boy with an incidental abdominal mass identified as a Primitive Myxoid Mesenchymal Tumor of Infancy derived from the small bowel mesentery. This neoplasm is part of a spectrum of myofibroblastic lesions, with distinct clinical, morphologic, IHQ reactivity, and an aggressive clinical course. There is a paucity of evidence for the management of these tumors, and surgical resection remains the mainstay of treatment. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Successful Extracorporeal Life Support in a Pediatric Hematopoietic Stem Cell Transplant Recipient With Periengraftment Respiratory Failure
The use of extracorporeal life support (ECLS) as ultimate salvage therapy for hematopoietic stem cell transplant recipients remains controversial among oncologists and critical care specialists. Prognosis is poor, particularly after allogeneic transplantation, and literature to guide clinical decision-making is scarce. Our report describes successful ECLS in a pediatric patient undergoing allogeneic hematopoietic stem cell transplantation, who developed acute respiratory failure during severe neutropenia, followed by immediate neutrophil engraftment. This unique case highlights periengraftment respiratory failure as a poss...
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Spontaneous Regression in a Patient With Infantile Fibrosarcoma
We describe spontaneous regression in a 5-month-old female infant with biopsy proven, molecularly confirmed, right leg infantile fibrosarcoma currently at 26 months of age with no signs of local recurrence. Previously reported cases of spontaneous regression are reviewed, suggesting a benign clinical course in some cases. Although evidence for spontaneous regression is anecdotal in this rare tumor type, physicians should weigh the risks and benefits of surgery and chemotherapy against watchful waiting. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Reduced Neutrophil Elastase Activity and Neutrophil Extracellular Traps in Pediatric Acute Myeloid Leukemia May Increase the Rate of Infections
Data on the production of neutrophil extracellular traps (NETs) in leukemia patients are scant. Phagocytosis, hydrogen peroxide, neutrophil elastase and myeloperoxidase enzymatic activity as well as NETs formation were studied in 10 pediatric acute lymphoblastic leukemia and 7 pediatric acute myeloid leukemia (AML) patients after induction chemotherapy. Median neutrophil elastase activity and NETs formation were lower in AML versus acute lymphoblastic leukemia (41% vs. 90%, P=0.005 and 51% vs. 94%, P=0.008, respectively). AML patients had more episodes of febrile neutropenia during the first 2 blocks of treatment (100% vs....
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Impact of Hydroxyurea on Anthropometry and Serum 25-Hydroxyvitamin D Among Children With Sickle Cell Disease
Conclusions: HU possibly ameliorate growth retardation and vitamin D deficiency in children with SCD. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a syndrome of platelet trapping known as Kasabach-Merritt phenomenon that, through deposition of platelet derived growth factors, may perpetuate the growth of the tumor. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often refractory even to aggressive treatment. Herein, we describe a patient with a refractory, recurrent KHE despite multiple attempts at local control and intensive chemotherapy, that ultimately was successfully treated with ration...
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Cup-like Blasts in 2 Pediatric Patients With NPM-1-Positive Acute Myeloid Leukemia
Cup-like phenotype is defined in some subtypes of acute myeloid leukemia (AML) and have been associated with NPM-1 and/or FLT3-ITD positivity in the presence of normal karyotype in>60% of patients. Herein we present two pediatric AML-M1 patients with cuplike nuclear morphology and NPM-1 positivity. Both patients were negative for FLT3-ITD mutation. NPM-1 mutation and FLT3-ITD mutation should be kept in mind in AML patients with cup-like blast morphology as these two mutations are important molecular markers for prognosis, risk group classification and also for response to treatment. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Morphology Corner Source Type: research

Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adults: A National Study of 369 Patients
Conclusions: This study provides the largest comparison of pediatric and adult SPN to date. Children with SPN have similar disease severity at presentation, receive similar treatments, and demonstrate equivalent postoperative outcomes compared with their adult counterparts. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

The Value of Neutrophil to Lymphocyte Ratio and Platelet to Lymphocyte Ratio for Detecting Early-onset Neonatal Sepsis
Conclusions: NLRs and PLRs were positively correlated with EOS in term neonates, and these ratios can be used as diagnostic adjunct tests for neonate EOS workups. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

GATA2 Deficiency Due to de Novo Complete Monoallelic Deletion in an Adolescent With Myelodysplasia
GATA2 deficiency is an inherited bone marrow failure syndrome that can manifest with myelodysplasia (myelodysplastic syndrome) with chromosomal aberrations and high risk of evolution to leukemia (particularly, acute myeloid leukemia); immunodeficiency with opportunistic infections; and/or lymphedema. It can be transmitted in families in autosomal-dominant fashion, or present de novo as sporadic disease in adults or children. The authors report a case of an adolescent male with features of GATA2 deficiency resulting from a complete monoallelic deletion, review chromosomal anomalies associated with this disorder, and discuss...
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Allogeneic Matched Related Donor Bone Marrow Transplantation for Pediatric Patients With Severe Aplastic Anemia Using “Low-dose” Cyclophosphamide, ATG Plus Fludarabine
Conclusion: This small pilot study shows that this cytoreductive regimen could be considered as the standard of care for transplantation of pediatric patients with aplastic anemia from HLA-matched siblings. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Failed Vacuum and the Long-term Hematologic Morbidity of the Offspring
Conclusions: Failed vacuum delivery does not seem to be associated with an increased risk for pediatric hematologic morbidity of the offspring up to 18 years of age. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Maternal Exposure to Pesticides, Paternal Occupation in the Army/Police Force, and CYP2D6*4 Polymorphism in the Etiology of Childhood Acute Leukemia
Epidemiologic studies have suggested that parental occupations, pesticide use, environmental factors, and genetic polymorphism are involved in the etiology of childhood acute leukemia (CAL). In total, 116 cases of CAL and 162 controls were recruited and submitted to blood drawing to assess the presence of genetic polymorphisms. Parental occupations, pesticides exposure, and other potential determinants were investigated. Increased risk for CAL was associated with prenatal maternal use of insecticides/rodenticides (odds ratio [OR]=1.87; 95% confidence intervals [CI], 1.04-3.33), with subjects living (Source: Journal of Pedi...
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Use of Intravenous Posaconazole in Hematopoietic Stem Cell Transplant Patients
Conclusions: Higher dosing per body weight of IV posaconazole may be required in the pediatric population compared with adults to consistently achieve therapeutic concentrations. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 26, 2018 Category: Hematology Tags: Online Articles: Original Articles Source Type: research