Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden.

Abstract:

BACKGROUND:Primary hemophagocytic lymphohistiocytosis (HLH) represents a group of inherited hyperinflammatory immunodeficiencies, including familial HLH (FHL), Griscelli syndrome type 2 (GS2), and X-linked lymphoproliferative syndrome (XLP). We previously reported an annual incidence of suspected primary HLH in Sweden 1971-1986 of 0.12 per 100,000 children. Here, we determined if the incidence had increased with concomitant awareness. PROCEDURE:Children <15 years old presenting with HLH 1987-2006 in Sweden were identified through the national mortality registry as well as by nation-wide inquiries to all pediatric centers. HLH was diagnosed according to the HLH-2004 diagnostic guidelines (in case of missing data of at least three of the eight diagnostic criteria, fulfillment of four was sufficient for inclusion). We defined primary HLH as patients presenting with HLH requiring transplantation or dying of disease. RESULTS:Remarkably, the minimal annual incidence rate of primary HLH remained 0.12 per 100,000 children, equating to 1.8 per 100,000 live births. Notably, an increased overall survival was observed in 1997-2006, relative to the period 1987-1996. During the subsequent 5-year period, 2007-2011, the incidence of genetically and/or functionally verified primary HLH was 0.15 per 100,000 children per year, suggesting that new assays may aid the identification of patients with primary HLH. CONCLUSION:The annual incidence of primary HLH in Sweden is 0.12-0.15 per 100,000 children per year. Pediatr Blood Cancer 2015;62:346-352. © 2014 Wiley Periodicals, Inc.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Meeths M,Horne A,Sabel M,Bryceson YT,Henter JI

doi

10.1002/pbc.25308

subject

Has Abstract

pub_date

2015-02-01 00:00:00

pages

346-352

issue

2

eissn

1545-5009

issn

1545-5017

journal_volume

62

pub_type

杂志文章
  • Physician Perspectives on Palliative Care for Children With Neuroblastoma: An International Context.

    abstract:BACKGROUND:Studies have shown that children with cancer globally lack access to palliative care. Little is known regarding physicians' perceptions of palliative care, treatment access, and self-reported competence in providing palliative care. PROCEDURE:Members of the Global Neuroblastoma Network (online tumor board) ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.25900

    authors: Balkin EM,Thompson D,Colson KE,Lam CG,Matthay KK

    更新日期:2016-05-01 00:00:00

  • Long-term results of the AIEOP LNH-97 protocol for childhood lymphoblastic lymphoma.

    abstract:BACKGROUND:Treatment intensification was considered a suitable strategy to increase the cure rate of lymphoblastic lymphoma (LBL) in children. PROCEDURE:The AIEOP LNH-97 trial was run between 1997 and 2007 for newly diagnosed LBL in patients aged less than 18 years. Treatment schedule was based on the previous, LSA2-L...

    journal_title:Pediatric blood & cancer

    pub_type: 临床试验,杂志文章

    doi:10.1002/pbc.25469

    authors: Pillon M,Aricò M,Mussolin L,Carraro E,Conter V,Sala A,Buffardi S,Garaventa A,D'Angelo P,Lo Nigro L,Santoro N,Piglione M,Lombardi A,Porta F,Cesaro S,Moleti ML,Casale F,Mura R,d'Amore ES,Basso G,Rosolen A

    更新日期:2015-08-01 00:00:00

  • Treatment of the X-linked lymphoproliferative, Griscelli and Chédiak-Higashi syndromes by HLH directed therapy.

    abstract:BACKGROUND:Griscelli syndrome type 2 (GS2), the X-linked lymphoproliferative (XLP) and the Chédiak-Higashi (CHS) syndromes are diseases that all may develop hemophagocytic syndromes. We wanted to investigate whether the treatment protocols for hemophagocytic lymphohistiocytosis (HLH) can also be used for these syndrome...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.21790

    authors: Trottestam H,Beutel K,Meeths M,Carlsen N,Heilmann C,Pasić S,Webb D,Hasle H,Henter JI

    更新日期:2009-02-01 00:00:00

  • Children with HbSβ0 thalassemia have higher hemoglobin levels and lower incidence rate of acute chest syndrome compared to children with HbSS.

    abstract:BACKGROUND:Based on the presumed clinical similarity between the two most severe sickle cell disease (SCD) genotypes, hemoglobin (Hb) Sβ0 thalassemia and HbSS, randomized controlled trials (RCTs) have included both genotypes. Our group has demonstrated that healthcare providers inadequately distinguish the two diagnose...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.27352

    authors: Day ME,Rodeghier M,DeBaun MR

    更新日期:2018-11-01 00:00:00

  • Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP).

    abstract:BACKGROUND:Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline ...

    journal_title:Pediatric blood & cancer

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1002/pbc.28332

    authors: Virgone C,Andreetta M,Avanzini S,Chiaravalli S,De Pasquale D,Crocoli A,Inserra A,D'Angelo P,Alaggio R,Opocher G,Cecchetto G,Ferrari A,Bisogno G,Dall'Igna P

    更新日期:2020-08-01 00:00:00

  • Physician perceptions and beliefs of phase I trials in pediatric oncology.

    abstract::The identification and referral of candidate patients for phase I trials relies heavily on pediatric oncologists who must balance their own perceptions of phase I trials with the desires of the patient and his/her family. A survey was sent to 419 physicians practicing pediatric oncology at 30 different institutions. R...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1002/pbc.24522

    authors: Gilliam MB,Madan-Swain A,Adams JM,Pressey JG

    更新日期:2013-08-01 00:00:00

  • A family with Hoyeraal-Hreidarsson syndrome and four variants in two genes of the telomerase core complex.

    abstract::We describe an African American family with Hoyeraal-Hreidarrson syndrome (HHS) in which 2 TERT mutations (causing P530L and A880T amino acid changes) and two in the DKC1 variants (G486R and A487A) were segregating. Both genes are associated with dyskeratosis congenita and HHS. It was important to determine the import...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.24389

    authors: Vogiatzi P,Perdigones N,Mason PJ,Wilson DB,Bessler M

    更新日期:2013-06-01 00:00:00

  • Pediatric residents' perceived barriers to opioid use in sickle cell disease pain management.

    abstract:OBJECTIVE:Current guidelines recommend high-priority treatment of severe sickle cell disease (SCD) pain with opioids; however, patients with SCD have historically been undertreated. We used mixed methods to assess pediatric residents' perceptions toward opioid use in SCD pain management. METHODS:We distributed a surve...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.27535

    authors: Fearon A,Marsh A,Kim J,Treadwell M

    更新日期:2019-02-01 00:00:00

  • Prevention and management of central venous catheter occlusion and thrombosis in children with cancer.

    abstract:BACKGROUND:The views and clinical practice of children's cancer units were surveyed regarding management of central venous catheter (CVC) occlusion (CVC-occlusion), CVC-related thrombosis (CVC-thrombosis) and thromboembolism (CVC-thromboembolism). PROCEDURE:A questionnaire was sent to all 22 United Kingdom Children's ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,多中心研究

    doi:10.1002/pbc.21332

    authors: Skinner R,Koller K,McIntosh N,McCarthy A,Pizer B,United Kingdom Children's Cancer Study Group (UKCCSG).,Paediatric Oncology Nursing Forum (PONF) Supportive Care Group.

    更新日期:2008-04-01 00:00:00

  • Successful antifungal combination therapy with voriconazole and caspofungin.

    abstract::A 12-year-old boy in third remission of an acute lymphoblastic leukaemia developed infection of lung and paranasal sinuses with Aspergillus flavus in neutropenia. Because of the high risk of leukaemia-relapse bone marrow transplantation (BMT) from a matched unrelated donor was carried out despite invasive pulmonary as...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.20302

    authors: Schuster F,Moelter C,Schmid I,Graubner UB,Kammer B,Belohradsky BH,Führer M

    更新日期:2005-06-15 00:00:00

  • A critical assessment of transcranial doppler screening rates in a large pediatric sickle cell center: opportunities to improve healthcare quality.

    abstract:BACKGROUND:Transcranial Doppler ultrasound (TCD) has been demonstrated to be a powerful predictor of stroke risk due to sickle cell disease (SCD) in pediatric populations. Little is known about how this healthcare innovation has disseminated into preventive care for SCD. The objective of this study was to determine TCD...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.21677

    authors: Raphael JL,Shetty PB,Liu H,Mahoney DH,Mueller BU

    更新日期:2008-11-01 00:00:00

  • Ongoing remission after intensive ALL-type chemotherapy in pediatric intestinal T-cell lymphoma.

    abstract::A rare case of primary intestinal T-cell lymphoma (ITL) of an 8-year-old boy is reported. Medium- to large-sized tumor cells were betaF1+, CD3+, CD8+. TIA-1+, but CD4-, CD5-, CD30-, CD56-, CD20-, CD79a-, TdT-, consistent with an intraepithelial lymphocyte (IEL) origin. They showed monoclonal rearrangement of the T-cel...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.22330

    authors: Friedrich C,Schrum J,Chott A,Janka-Schaub G,Kabisch H

    更新日期:2010-04-01 00:00:00

  • The associations between maternal factors during pregnancy and the risk of childhood acute lymphoblastic leukemia: A meta-analysis.

    abstract:BACKGROUND:Although genetic and environmental factors are considered to be the main causes of acute lymphoblastic leukemia, the associations between maternal factors during pregnancy and the childhood ALL is still unclear. PROCEDURE:In this study, meta-analysis was used. Medline, PubMed, and Web of Science were search...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,meta分析

    doi:10.1002/pbc.25443

    authors: Yan K,Xu X,Liu X,Wang X,Hua S,Wang C,Liu X

    更新日期:2015-07-01 00:00:00

  • Successful Propranolol Treatment of a Kaposiform Hemangioendothelioma Apparently Resistant to Propranolol.

    abstract::A newborn with unresectable kaposiform hemangioendothelioma associated with Kasabach Merritt phenomenon, unresponsive to vincristine and prednisone, received second-line treatment with propranolol at a dose of 2 mg/kg/day, starting at 2 months of life and continued for 13 months. There was only slight reduction in tum...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.25979

    authors: Filippi L,Tamburini A,Berti E,Perrone A,Defilippi C,Favre C,Calvani M,Della Bona ML,la Marca G,Donzelli G

    更新日期:2016-07-01 00:00:00

  • Medical and rehabilitation interventions in pediatric central nervous system radiation necrosis: A case report.

    abstract::Radiation necrosis is a potentially debilitating side effect of therapy necessary to treat pediatric central nervous system tumors. Clinical signs of cerebral radiation necrosis (CRN) are similar to symptoms of disease progression and require close monitoring. The case of an infant diagnosed with a malignant rhabdoid ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.28705

    authors: L'Hotta AJ,Thomas KM,Milgrom SA,Hemenway MS,Levy JMM

    更新日期:2021-01-01 00:00:00

  • Hematopoietic stem cell transplant referral patterns for children with sickle cell disease vary among pediatric hematologist/oncologists' practice focus: A Sickle Cell Transplant Advocacy and Research Alliance (STAR) study.

    abstract:BACKGROUND:Hematopoietic stem cell transplantation (HSCT) provides a curative therapy for children severely affected by sickle cell disease (SCD). Rejection-free survival after matched sibling donor (MSD) HSCT is very high, but adoption of HSCT as a curative SCD therapy has been slow. In this study, we assess providers...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.28861

    authors: Meier ER,Abraham AA,Ngwube A,Janson IA,Guilcher GMT,Horan J,Kasow KA

    更新日期:2021-03-01 00:00:00

  • Quality of life in young patients after bone tumor surgery around the knee joint and comparison with healthy controls.

    abstract:BACKGROUND:This study aimed to compare the health related quality of life (HRQoL) of children and adolescents after malignant bone tumor surgery of the leg with healthy controls. PROCEDURE:Patients between 8 and 25 years old were cross-sectional recruited. Patients under 16 years of age received the TNO (Netherlands O...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,多中心研究

    doi:10.1002/pbc.22439

    authors: Bekkering WP,Vliet Vlieland TP,Koopman HM,Schaap GR,Schreuder HW,Beishuizen A,Tissing WJ,Hoogerbrugge PM,Anninga JK,Taminiau AH

    更新日期:2010-05-01 00:00:00

  • Treatment late effects in long-term survivors of pediatric sarcoma.

    abstract:PURPOSE:To assess health and musculoskeletal function in survivors of pediatric sarcomas. PATIENTS AND METHODS:Thirty-two individuals treated for Ewing sarcoma family of tumors (ESFT), rhabdomyosarcoma (RMS), or non-rhabdomyosarcoma soft tissue sarcomas (NR-STS) with multi-modality therapy were enrolled on this cross-...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.20871

    authors: Mansky P,Arai A,Stratton P,Bernstein D,Long L,Reynolds J,Chen D,Steinberg SM,Lavende N,Hoffman K,Nathan PC,Parks R,Augustine E,Chaudhry U,Derdak J,Wiener L,Gerber L,Mackall C

    更新日期:2007-02-01 00:00:00

  • A comparison of pediatric, adolescent, and adult testicular germ cell malignancy.

    abstract:BACKGROUND:Testicular germ cell tumors (T-GCTs) occur from infancy to adulthood, and are the most common solid tumor in adolescent and young adult males. Traditionally, pediatric T-GCTs were perceived as more indolent than adult T-GCTs. However, there are few studies comparing these groups and none that specifically ev...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.24773

    authors: Cost NG,Lubahn JD,Adibi M,Romman A,Wickiser JE,Raj GV,Sagalowsky AI,Margulis V

    更新日期:2014-03-01 00:00:00

  • Pulmonary function abnormalities in children treated with whole lung irradiation.

    abstract:BACKGROUND:We sought to determine the prevalence of abnormal pulmonary function tests (PFTs) in a cohort of children who had received whole lung irradiation (WLI) for treatment of metastatic disease. PROCEDURE:This was a retrospective (1988-2003) chart review that included all children treated at our institution with ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.20457

    authors: Weiner DJ,Maity A,Carlson CA,Ginsberg JP

    更新日期:2006-02-01 00:00:00

  • Serologic response and clinical efficacy of influenza vaccination in children and young adults on chemotherapy for cancer.

    abstract:BACKGROUND:Influenza is a health risk to children receiving chemotherapy for cancer. An absolute lymphocyte count (ALC) >1,000 cells/mm(3) has been associated with the ability to produce an immune response to influenza vaccine during chemotherapy. However, clinical efficacy of influenza vaccination during chemotherapy ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.26110

    authors: Choi DK,Fuleihan RL,Walterhouse DO

    更新日期:2016-11-01 00:00:00

  • The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis.

    abstract:BACKGROUND:The identification of hemophagocytosis (HPC) in tissue or bone marrow (BM) represents only one of 5/8 criteria needed for the diagnosis of hemophagocytic lymphohistiocytosis (HLH). Yet, confirmation of HPC in bone marrow aspirates (BMA) is often relied upon to make therapeutic decisions. There is no standard...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.21564

    authors: Gupta A,Tyrrell P,Valani R,Benseler S,Weitzman S,Abdelhaleem M

    更新日期:2008-09-01 00:00:00

  • Complete durable response of a pediatric anaplastic oligodendroglioma to temozolomide alone: Case report and review of literature.

    abstract::Anaplastic oligodendroglioma (AO) is rare in children. Treatment typically consists of varying combinations of surgery, chemotherapy, and radiotherapy. We present a pediatric case of frontal lobe AO with periventricular subcallosal extension and local leptomeningeal involvement. The isocitrate dehydrogenase (IDH) wild...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,评审

    doi:10.1002/pbc.26708

    authors: Sorge C,Li R,Singh S,Reddy AT,Solomon DA,Perry A,Friedman GK

    更新日期:2017-12-01 00:00:00

  • Does dexamethasone induce more neuropsychological side effects than prednisone in pediatric acute lymphoblastic leukemia? A systematic review.

    abstract::Steroid-induced neuropsychological side effects impact quality of life in children with acute lymphoblastic leukemia. Dexamethasone induces more metabolic side effects than prednisone. To evaluate whether dexamethasone also leads to more neuropsychological side effects, we reviewed all available literature. Randomized...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,评审

    doi:10.1002/pbc.24988

    authors: Warris LT,van den Heuvel-Eibrink MM,den Hoed MA,Aarsen FK,Pieters R,van den Akker EL

    更新日期:2014-07-01 00:00:00

  • Information provision and information needs in adult survivors of childhood cancer.

    abstract:BACKGROUND:Knowledge about their past medical history is central for childhood cancer survivors to ensure informed decisions in their health management. Knowledge about information provision and information needs in this population is still scarce. We thus aimed to assess: (1) the information survivors reported to have...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.24762

    authors: Gianinazzi ME,Essig S,Rueegg CS,von der Weid NX,Brazzola P,Kuehni CE,Michel G,Swiss Paediatric Oncology Group (SPOG).

    更新日期:2014-02-01 00:00:00

  • Comparison of allergic reactions to pegasparaginase given intravenously versus intramuscularly.

    abstract:BACKGROUND:Pegasparaginase (PEG) is important for treatment of Acute Lymphoblastic Leukemia (ALL). Despite conjugation to polyethylene glycol to reduce immunogenicity, allergic reactions still occur and may be severe. Traditionally, PEG is given via intramuscular (IM) injection but recent protocols have shown it can be...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.23380

    authors: Pidaparti M,Bostrom B

    更新日期:2012-09-01 00:00:00

  • Childhood liver tumors in Argentina: Incidence trend and survival by treatment center. A report from the national pediatric cancer registry, ROHA network 2000-2015.

    abstract:BACKGROUND:Information on the epidemiology of pediatric liver tumors in Latin America is limited. PURPOSE:To describe the incidence of liver tumors in a pediatric registry in Argentina according to geographic region, national trends over 16 years, and survival related to stage, age, sex, and care center. METHODS:Newl...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.28583

    authors: Moreno F,Rose A,Chaplin MA,Cipolla MC,García Lombardi M,Nana M,Cervio G,Halac E,Viso M,Ayarzabal V,Bosaleh A,Liberto D,Sarabia E,Rizzi A,Morici M,Streitenberger P,de Dávila MTG

    更新日期:2020-11-01 00:00:00

  • "Living life as if I never had cancer": A study of the meaning of living well in adolescents and young adults who have experienced cancer.

    abstract:BACKGROUND:Cancer diagnoses pose challenges to adolescents' and young adults' (AYA) physical, mental, and emotional health, and developmental tasks. In order for AYAs, caregivers, clinicians, and other collaborators to optimize health outcomes (coproduction of health), understanding what living well means for AYAs who ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,随机对照试验

    doi:10.1002/pbc.28599

    authors: Schreiner K,Grossoehme DH,Friebert S,Baker JN,Needle J,Lyon ME

    更新日期:2020-10-01 00:00:00

  • A novel mutation of ABCG5 gene in a Turkish boy with phytosterolemia presenting with macrotrombocytopenia and stomatocytosis.

    abstract::Phytosterolemia is a rare autosomal recessive sterol storage disease caused by mutations in ABCG5 and ABCG8 genes. A 9-year-old Turkish boy who was presented with exclusively hematologic abnormalities had elevated plant sterol levels. Sequencing of ABCG5 and ABCG8 genes revealed a novel homozygous IVS10-1 G>T mutation...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.24934

    authors: Kaya Z,Niu DM,Yorulmaz A,Tekin A,Gürsel T

    更新日期:2014-08-01 00:00:00

  • Ultrasound-guided core needle biopsy for the diagnosis of rhabdomyosarcoma in childhood.

    abstract:BACKGROUND:Most commonly a tissue diagnosis of rhabdomyosarcoma (RMS) in children is made by biopsy as opposed to primary resection. Open surgical procedures are often recommended to obtain sufficient material for accurate and complete diagnostic work up. Our institution has routinely used image-guided needle biopsies ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章

    doi:10.1002/pbc.22059

    authors: Chowdhury T,Barnacle A,Haque S,Sebire N,Gibson S,Anderson J,Roebuck D

    更新日期:2009-09-01 00:00:00