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Novel Mutations Resulting in a Moderate to Severe Phenotypic Manifestation of Hemophilia A in a Female
Hemophilia A is an X-linked, recessive disorder resulting from mutations in the f8 gene. Here we report the rare case of a female compound heterozygote with mild factor VIII deficiency (fVIII:C 9%) and moderate phenotype. On investigation she was confirmed to have normal Von Willebrand factor studies with a 46XY genotype. Further genetic testing revealed 3 mutations in the f8 gene: 1 novel missense mutation (c.6142T>G), 1 novel in-frame deletion (c.1281_1292del), and another missense mutation of unclear significance (c.3780C>G). Both parents had normal coagulation profiles; however, the 2 novel mutations were present...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Synchronous Presentation of Renal Cell Carcinoma and Hodgkin Lymphoma in an Adolescent
Conclusions: This case highlights the importance for histologic confirmation of a renal mass when concurrently discovered during the diagnostic evaluation of other malignancies. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Allogeneic Hematopoietic Cell Transplantation for Dyskeratosis Congenita: A Report of 3 Cases
Although bone marrow failure in patients with dyskeratosis congenita (DKC) can be successfully treated with allogeneic hematopoietic cell transplantation (allo-HCT) using a reduced intensity conditioning (RIC) regimen, the outcome of nonhematological disorders in patients with DKC treated with allo-HCT using RIC has not been fully elucidated. Here, we describe the clinical course of nonhematological disorders after allo-HCT with RIC in 3 consecutive patients with DKC. Allo-HCT with RIC was feasible in all cases; however, patient 1 developed lethal pulmonary disease and patient 2 experienced progression of hepatic fibrosis....
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Treatment of Refractory Infantile Hemangiomas and Pulmonary Hypertension With Sirolimus in a Pediatric Patient
This report supports prior studies that sirolimus is effective for vascular anomalies including IH refractory to conventional therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Variation in Transfusion Requirements Among Children With Thalassemia on Regular Transfusion Programs: Which Formula Closely Predicts the Actual Requirements?
Conclusions: To meet transfusion requirements, individualization of formulas by establishment of the transfusion factor for each patient is helpful. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Biallelic PMS2 Mutation and Heterozygous DICER1 Mutation Presenting as Constitutional Mismatch Repair Deficiency With Corpus Callosum Agenesis: Case Report and Review of Literature
This report is the first to allude to a possible interaction of the mismatch repair system with DICER1 to cause corpus callosum agenesis. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Parent-perceived Facilitators in the Transition of Care for Young Adult Survivors of Childhood Cancer
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 als...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Sinusoidal Obstruction Syndrome During Chemotherapy of Pediatric Cancers and its Successful Management With Defibrotide
Sinusoidal obstruction syndrome (SOS) is a life-threatening complication generally occurring after hematopoietic stem cell transplantation. SOS after standard dose chemotherapy in malignancies is rare. Between the year 1995 and 2016, 414 patients were diagnosed with acute lymphoblastic leukemia and 113 patients were diagnosed with Wilms tumor in our institution. Among these patients, 4 patients with acute lymphoblastic leukemia (0.96%) and 2 patients with Wilms tumor (1.7%) developed SOS during treatment. SOS behaves like a local disseminated intravascular coagulation. Defibrotide has proved to be effective in SOS. In this...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Development of Secondary Acute Myeloid Leukemia in a Pediatric Patient Concurrently Receiving Primary Therapy for Ewing Sarcoma
This report highlights the importance of monitoring for treatment-related effects in cancer therapy, as well as the need for novel, less toxic approaches in Ewing sarcoma therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Use of Electronic Consultation System to Improve Access to Care in Pediatric Hematology/Oncology
Conclusions: This study showed successful implementation and use of the eConsult service for pediatric hematology/oncology and resulted in avoidance of a large number of face-to-face consultation. The common topics identified areas for continuing medical education. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Novel Method Enabling the Use of Cryopreserved Primary Acute Myeloid Leukemia Cells in Functional Drug Screens
The ability to assess antileukemic drug activity on primary patient samples is a powerful tool in determining potential drug targets and selection of therapeutic agents with biological and functional rationale. We previously established small molecule inhibitor screens for use on freshly isolated leukemia cells for this purpose. Here we describe a method that produces functional small molecule inhibitor screening results using cryopreserved primary acute myeloid leukemia cells. This method was established to take advantage of biorepositories containing archival material, such as those established by the Children’s On...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Clinical Profile and Outcome of Influenza A/H1N1 in Pediatric Oncology Patients During the 2015 Outbreak: A Single Center Experience from Northern India
Conclusions: Childhood cancer patients infected with Influenza A/H1N1 are at risk of serious illness and higher mortality. Delay of anticancer treatment is a concern in these infected children. Prompt initiation of antivirals and an optimum duration of treatment are warranted to reduce the morbidity and mortality. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Children Receiving Hematopoietic Stem Cell Transplantation are at Increased Risk of Onychocryptosis Requiring Surgical Management
Conclusions: HSCT is strongly associated with onychocryptosis requiring surgical intervention. Children with a history of HSCT may also have more aggressive toenail disease, with higher rates of surgical intervention, bilateral ingrown toenails, recurrence, and need for return to the operating room. Clinicians should perform careful screening and early treatment in these patients. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Ambulatory High-dose Methotrexate Administration in Pediatric Osteosarcoma Patients at a Single Institution in Argentina
Conclusions: Ambulatory HDMTX administration is feasible and safe in a population with poor resources in a developing country. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Surgical Feeding Tubes in Pediatric and Adolescent Cancer Patients: A Single-institution Retrospective Review
The purpose of our study was to evaluate surgical enteric access in pediatric cancer patients to determine factors associated with postoperative complications. We performed a single-institution retrospective review of all patients below 21 years old with a primary cancer diagnosis who underwent surgical procedures for enteral access between 2004 and 2014. Multivariate logistic regression was performed to determine independent predictors of postoperative complications. During the study period, 122 patients had surgically placed feeding tubes, of whom 58% developed ≥1 complication(s) and 16% experienced a major complicati...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Ominous Trends in Childhood Cancer Mortality: Who was Right?
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Letters to the Editor Source Type: research

Activated Partial Thromboplastin Time versus Anti-Factor Xa Levels for Monitoring Unfractionated Heparin Therapy in Children: An Institutional Experience
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Letters to the Editor Source Type: research

Congenital Amegakaryocytic Thrombocytopenia: A Case Series Indicating 2 Founder Variants in the Mississippi Band of Choctaw Indians
In this report, we review 5 cases of congenital amegakaryocytic thrombocytopenia, all of whom belong to the Mississippi Band of Choctaw Indians. There are 2 common variants in these cases: R90X and R537W. One variant was previously reported only once and had unclear significance at that time. With these variants identified, we hope to improve screening that results in earlier diagnosis in the Choctaw population in the future. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Fanconi Anemia: A Rarely Considered Cause of Macrocytosis During Childhood
We describe a Turkish boy newly diagnosed with Fanconi anemia with mutation in the FANCA gene. The patient, with normal clinical phenotype and negative chromosomal breakage test result, presented with macrocytosis. No clinical or laboratory changes were observed in a follow-up period of 4 years. The diagnosis was confirmed molecularly after a prolonged and exhaustive investigation. He was found to be a compound heterozygote for 2 mutations in the FANCA gene (1 of which is novel, c.4261-2A>C). We present this experience to alert physicians that Fanconi anemia should be considered in the differential diagnosis of otherwis...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Very Long-term Sequelae After Nonradical Surgery Combined With Brachytherapy in an Infant With a Chemotherapy-resistant Rhabdomyosarcoma of the Tongue
In 2003, van Grotel and colleagues reported an infant suffering a chemotherapy-resistant eRMS of the tongue, that was treated with subtotal tumor resection and brachytherapy after major medical ethical discussions. As no long-term sequelae of such a procedure have been described, perspectives were uncertain at that time. Now, after 15 years, we describe hypoplasia of the mandibula, compromised dentation, osteopenia, neuropsychological deficits, and moderate speech impairment as the most prominent late effects. Also, mandibular cysts and basal cell carcinomas in the irradiated area, eventually led to the diagnosis Gorlin sy...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Induction Failure in Acute Leukemia or Parvovirus B19 Infection?
Parvovirus B19 infection may be seen in acute leukemia patients and clinical findings and cytopenia caused by the viral infection may complicate the evaluation of the remission status. Herein we present a standard risk pediatric acute lymphobiastic leukemia patient who developed myalgia, bone pain, bone marrow aplasia and sinusoidal obstruction syndrome at the end of the induction treatment and was diagnosed as having parvovirus B19 infection. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Morphology Corner Source Type: research

Epigenetic Combination Therapy for Children With Secondary Myelodysplastic Syndrome (MDS)/Acute Myeloid Leukemia (AML) and Concurrent Solid Tumor Relapse
Secondary myelodysplastic syndrome (MDS)/acute myeloid leukemia (AML) is a rare but devastating complication of solid tumor treatment involving high-dose topoisomerase II inhibitor and alkylator chemotherapy. For relapsed or elderly MDS and AML patients ineligible for hematopoietic stem cell transplantation, epigenetic therapies, including DNA methyltransferase inhibitors and histone deacetylase inhibitors, have been utilized as palliative therapy, offering a well-tolerated approach to disease stabilization, prolonged survival, and quality of life. Literature on the use of epigenetic therapies for both primary and relapsed...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

The Effect of Ophthalmic Artery Chemosurgery on Immune Function in Retinoblastoma Patients: A Single Institution Retrospective Analysis
Conclusions: Clinically significant immune suppression appears rare following OAC alone, but patients previously treated with IV chemotherapy may be immunosuppressed and may benefit from pneumocystis pneumonia prophylaxis until the CD4 count recovers. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Long-term Pulmonary Outcomes in Pediatric Survivors of High-risk Neuroblastoma
This study assessed respiratory symptoms, pulmonary function tests (PFTs), and risk factors for abnormalities among survivors. Materials and Methods: High-risk neuroblastoma survivors followed in the long-term follow-up clinic at Memorial Sloan Kettering Cancer Center were enrolled. Self-administered symptom questionnaires were completed. Medical records were reviewed for treatment information and comorbidities. PFTs included spirometry, plethysmography, and diffusion capacity of the lung for carbon monoxide (DLCO). Results: Thirty-nine survivors participated (median age at study: 11.4 y; median age at diagnosis: 2.3 y; ...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Pretreatment Neutrophil-to-Lymphocyte Ratio and Lymphocyte Recovery: Independent Prognostic Factors for Survival in Pediatric Sarcomas
Conclusions: Our study confirms that NLR and ALC recovery are independent prognostic factors for pediatric sarcomas, implying an important role of immune system in survival. Clinical utility of these prognostic biomarkers should be validated in larger pediatric studies. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A Systematic Literature Review
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases. Including 1 pediatric case from our institution, 74 were child...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Treatment of Congenital Thrombotic Thrombocytopenia Purpura: A New Paradigm
Congenital thrombotic thrombocytopenia purpura (cTTP) is a very rare disorder worldwide. Standard treatment of recognized cases has been to administer fresh frozen plasma as the source of ADAMTS13, to replenish the absent ADAMTS13 enzyme. An alternative source, a plasma-derived factor VIII concentrate used for hemophilia A, and found to contain this enzyme, was reported to be effective in 1 patient in the United States. We now report details on a US cohort of 8 cTTP patients who have been successfully treated for varying periods with a marketed antihemophilic factor concentrate Koate-DVI. This biological product has been u...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Six Weeks Versus 3 Months of Anticoagulant Treatment for Pediatric Central Venous Catheter-related Venous Thromboembolism
Conclusions: Six weeks of treatment for CVC-related VTE may provide noninferior clinical outcomes compared with 3 months of anticoagulation. An international randomized-controlled trial (Kids-DOTT) is underway to explore the optimal duration of anticoagulation for acute-provoked VTE in children. This manuscript highlights that data from such studies is urgently needed. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Health-related Quality of Life and Associated Factors Among Thalassemia Major Patients, Southeast of Iran
In conclusion, providing a psychiatric health package seems to be essential for improving QOL in TM patients, especially in social field. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Transcranial Doppler Screening in 50 Patients With Sickle Cell Hemoglobinopathies in Iran
Background: As previous studies had discordant results with regard to the correlation of transcranial Doppler (TCD) screening and brain MRI, the aim of this study was to find the correlation between TCD values and silent ischemia in sickle cell disease (SCD) patients. Method and Materials: In this cross-sectional study, 50 patients with proven diagnosis of sickle cell hemoglobinopathies based on their hemoglobin electrophoresis were included. Demographic data, their physical exam, information with regard to crises history, and their laboratory data were recorded. Brain MRI and TCD were requested for all patients. Results...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Longitudinal Analysis of Echocardiographic Abnormalities in Children With Sickle Cell Disease
Conclusion: Cardiac abnormalities began early in childhood and progressively increased with age. Our study highlights the high cumulative incidence of cardiac abnormalities in children with SCD, which could represent a marker of disease severity. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Comparison of Different Iron Preparations in the Prophylaxis of Iron-deficiency Anemia
We compared the efficacy of ferrous sulfate (divalent) and ferric polymaltose (trivalent) compounds for the prophylaxis of iron-deficiency anemia (IDA). Study infants included exclusively breast milk-fed term infants. Subjects were divided randomly into 2 groups at 4 months of age and group 1 (n=56) received divalent and group 2 (n=56) received trivalent iron (Fe) preparation at a dose of 2 mg/kg/d for 5 months. At 9 months of age, after a 5-month prophylaxis, a significant increase was observed in hemoglobin (Hb), hematocrit, serum Fe levels, and transferrin saturation in both groups. However, group 1 had significa...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Evans Syndrome as First Manifestation of Primary Immunodeficiency in Clinical Practice
Conclusions: These cases highlight the importance of detection of possible PID in the context of ES and the establishment of CVID treatment to control AC. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Original Articles Source Type: research

Immature Ovarian Teratoma: When to Give Adjuvant Therapy?
The question of giving or not adjuvant chemotherapy in pure ovarian immature teratomas (ITs) remains unsolved to date and illustrates differences in management between pediatric and adults oncologists. Because of the rarity of these tumors, this question has never been addressed through randomized trials. Standard of care for adult women with ovarian ITs is postoperative platinum based chemotherapy for all patients except FIGO stage IA, grade 1 tumors, whereas pediatric series concluded that surgery alone is curative for completely resected ovarian ITs, regardless of grade. Moreover the role of chemotherapy in incompletely...
Source: Journal of Pediatric Hematology Oncology - September 23, 2017 Category: Hematology Tags: Invited Review Source Type: research

Epstein-Barr Virus–associated Mucocutaneous Ulcer in a Patient With T-Cell Acute Lymphoblastic Leukemia: Importance of Accurate Diagnosis and Conservative Management
Epstein-Barr virus–associated mucocutaneous ulcer (EBV-MCU) is a recently characterized entity that falls under the spectrum of EBV-lymphoproliferative disorders. First described in 2010 by Dojcinov et al, it is an EBV-driven localized proliferation of B cells, occurring in mucocutaneous tissues including the skin, the oropharynx, and the gastrointestinal tract of immunosuppressed patients in the absence of an intact T-cell repertoire. Typically, it has been described in elderly patients with age-related immunosenescence and patients who are on immunosuppressive therapy. However, only 2 cases have been reported in pe...
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Thalassemia Major and Priapism: A Case Report of an Adolescent
Priapism is defined as a prolonged pathologic penile erection without sexual stimulation. In children, priapism secondary to sickle cell disease or hematological malignancy is a frequent condition. Appropriate treatment of priapism varies; the treatment is primarily etiological, conservative management. In the present report, we aimed to present a case of asplenic thalassemia major who developed priapism, improved with hydration and ibuprofen treatment. Clinicians should take into account that priapism can be encountered in patients with thalassemia major. To our knowledge this is the second publication reporting the assoc...
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Concomitant Use of Panobinostat and Reirradiation in Progressive DIPG: Report of 2 Cases
We present 2 cases of DIPG, who were treated with panobinostat at 22 to 25 mg/m2/dose, 3 times weekly for 2 weeks in 3-week cycles and concomitant reirradiation after disease progression. Two episodes of asymptomatic thrombocytopenia were observed in 1 patient. Hyperacetylation of histone H4 of peripheral blood mononuclear cells was evident following treatment. In our experience, panobinostat administered with reirradiation was well tolerated at a relatively higher dose than that used in adult studies. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Everolimus for Treatment of Pseudomyogenic Hemangioendothelioma
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. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Lemierre’s Syndrome as a Trigger for Secondary Hemophagocytic Lymphohistiocytosis
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. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Case of Familial Hemophagocytic Lymphohistiocytosis Type 4 With Involvement of the Central Nervous System Complicated With Infarct
Conclusions: Physicians managing patients with HLH must be vigilant about the possibility of central nervous system involvement including stroke. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Cytomegalovirus Cavitary Pneumonia in Nontransplant Pediatric Acute Lymphoblastic Leukemia: Case Report and Review of the Literature
Lung cavitary lesions are a rare finding in the nontransplant setting of a patient with acute leukemia. Among viral agents, cytomegalovirus (CMV) may cause pulmonary nodules and cavities in patients undergoing hematopoietic stem cell transplantation, but during pediatric acute lymphoblastic leukemia treatment, CMV disease is less common. Here we report the case of a boy affected by acute lymphoblastic leukemia who suffered severe cavitary CMV pneumonia during chemotherapy, and we review the current literature. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Central Nervous System Fungal Infection and Acute Lymphoblastic Leukemia in Children: What is the Optimal Duration of Antifungal Therapy?
Invasive fungal infections are rare in children with acute lymphoblastic leukemia. It compromises the antileukemia therapy should it happen. Central nervous system fungal infections carry high mortality. We here report the case of a 6.5-year-old girl who developed multiple central nervous system fungal abscesses possibly due to Aspergillus infection during induction therapy for acute lymphoblastic leukemia who has been successfully managed without compromising antileukemia therapy. She has been receiving antifungal therapy and maintenance chemotherapy for 20 months from diagnosis. We reviewed literature about the optimal d...
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Newborn With Familial Hemophagocytic Lymphohistiocytosis Complicated With Transfusion Associated Graft Versus Host Disease
Hemophagocytic lymphohistiocytosis (HLH) is characterized by activation of cytotoxic T and natural killer (NK) cells, and macrophages related to a spectrum of hyperinflammatory disorders. The clinical findings mainly include high fever, cytopenia, splenomegaly, phagocytosis, and proliferation of histiocytes in lymphoreticular tissue. To the best of our knowledge, transfusion-associated graft versus host disease (TA-GVHD) in a 13-day old male newborn with HLH is being reported first time in the literature. The aim of this report was to emphasize the importance of blood products irradiation in the prevention of the developme...
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Pantoea Species Bacteremia in a Child With Sickle Cell Disease: Looking for a Culprit
We describe the case of a 5-year-old girl with sickle cell disease with P. agglomerans bacteremia and review its potential causes. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Critically Ill Children With Hemophagocytic Lymphohistiocytosis: A Case Series of 14 Patients
In this study, we described the clinical characteristics of critically ill children with HLH and identify factors associated with poor clinical outcomes. Children who were diagnosed with HLH with emergent admission to Children’s Intensive Care Unit (CICU) between January 1, 2000 and October 31, 2015 were included. The primary outcome was CICU mortality. Over the 15-year period, there were 14 critically ill patients with HLH with 23 CICU admissions. Median age at HLH diagnosis was 8.2 years (interquartile range [IQR], 2.9 to 11.3). Overall CICU mortality was 8 of 23 CICU admissions (34.8%). Factors that were associate...
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Online Articles: Original Article Source Type: research

Mature Ovarian Teratoma After Treatment for Unilateral Retinoblastoma With Nonmutated RB1
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Letters to the Editor Source Type: research

Thalidomide in Transfusion Dependent Thalassemia: Hope or Hype
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Letters to the Editor Source Type: research

Clofarabine Dosing in a Patient With Acute Myeloid Leukemia on Intermittent Hemodialysis: Case Report and Review of the Literature
We describe our rationale for dosing, clofarabine plasma levels obtained, and discuss our findings in the context of other available literature. Consistent with previous findings, intermittent hemodialysis was not found to be a reliable method of removing clofarabine in patients with renal insufficiency. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Pediatric Oncology Clinic Care Model: Achieving Better Continuity of Care for Patients in a Medium-sized Program
Providing the best care in both the inpatient and outpatient settings to pediatric oncology patients is all programs goal. Using continuous improvement methodologies, we changed from a solely team-based physician care model to a hybrid model. All patients were assigned a dedicated oncologist. There would then be 2 types of weeks of outpatient clinical service. A “Doc of the Day” week where each oncologist would have a specific day in clinic when their assigned patients would be scheduled, and then a “Doc of the Week” week where one physician would cover clinic for the week. Patient satisfaction surv...
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Type 2B von Willebrand Disease: An Unusual Cause of Severe Neonatal Thrombocytopenia
An infant with presumed maternal immune thrombocytopenic purpura had persistent thrombocytopenia with platelet clumping. The patient had no significant bleeding symptoms in the first year of life and von Willebrand antigen and ristocetin cofactor activity were normal. Absent high molecular weight multimers ultimately led to a genetically proven diagnosis of type 2B von Willebrand disease (3964G>A VWF exon 28), highlighting the challenges of establishing this diagnosis in infants. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 25, 2017 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research