Delayed spontaneous remission in a child with primary acquired chronic pure red cell aplasia.

Abstract:

:The acquired form of pure red cell aplasia (PRCA) presents either as an acute self-limited disease, predominantly seen in children, or as a chronic illness more frequently seen in adults with rare spontaneous remissions. A 14-year-old boy presented with pallor, without hepatosplenomegaly, jaundice, lymphadenopathy, petechiae, or any other apparent abnormalities. Isolated anemia in the presence of normal white cell and platelet counts with a marrow of normal cellularity and absence of erythroblasts but normal myeloid cells and megakaryocytes revealed the diagnosis of PRCA. All possible investigations excluded secondary causes of PRCA. The patient required packed red cell transfusions every 2 to 3 weeks. He failed therapy with intravenous immunoglobulin, corticosteroids, cyclosporine A plus corticosteroids, antithymocyte globulin, anti-CD 20 (rituximab), and erythropoietin (EPO). He showed a severe, resistant, and transfusion-dependent PRCA. Spontaneous remission with normal hemoglobin and reticulocyte levels was dramatic 6.5 years after the diagnosis of PRCA and 3.6 years after his last treatment.

journal_name

Pediatr Hematol Oncol

authors

Khalil A,Elhasid R,Ben Barak A,Ben Arush MW

doi

10.3109/08880018.2010.493577

subject

Has Abstract

pub_date

2010-10-01 00:00:00

pages

574-80

issue

7

eissn

0888-0018

issn

1521-0669

journal_volume

27

pub_type

杂志文章
  • Redefined role of radiation in combined treatment of Ewing's sarcoma.

    abstract::Eighty-six patients with Ewing's sarcoma were analyzed as to the role of radiation therapy. Fifty-eight patients (P group) had removal of the involved part of the bone after preoperative chemotherapy, and 28 (NP group) had local treatment either at the same time or before chemotherapy. Thirty-six of 58 P-group patient...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880018609031206

    authors: Jereb B,Ong RL,Mohan M,Caparros B,Exelby P

    更新日期:1986-01-01 00:00:00

  • Efficacy and safety of human umbilical cord derived mesenchymal stem cell therapy in children with severe aplastic anemia following allogeneic hematopoietic stem cell transplantation: a retrospective case series of 37 patients.

    abstract::The treatment of pediatric severe aplastic anemia (SAA) with allogeneic hematopoietic stem cell transplantation (allo-HSCT), presents major challenges including the risks of graft failure, septic complications, and graft-versus-host disease (GVHD). Additive infusions of human umbilical cord derived mesenchymal stem ce...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880018.2013.867556

    authors: Si Y,Yang K,Qin M,Zhang C,Du Z,Zhang X,Liu Y,Yue Y,Feng Z

    更新日期:2014-02-01 00:00:00

  • A pilot trial of tandem autologous peripheral blood progenitor cell transplantation following high-dose thiotepa and carboplatin in children with poor-risk central nervous system tumors.

    abstract::This is a pilot study performed to determine the maximum tolerated number of courses of high-dose thiotepa and carboplatin with autologous peripheral blood progenitor cell (PBPC) transplantation in poor-risk pediatric central nervous system (CNS) tumor patients. Twelve patients were enrolled and a total of 24 PBPC tra...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/08880010490501060

    authors: Ozkaynak MF,Sandoval C,Levendoglu-Tugal O,Jayabose S

    更新日期:2004-10-01 00:00:00

  • Two CD34+ stem cells from umbilical cord blood enrichment methods.

    abstract::The authors describe the relation between clamping time and blood volume collected, and two enrichment systems of CD34+ stem cells from umbilical cord blood, to determine an excellent recovery with high proliferate ability and bone marrow reconstitution. After an obstetrician-based cord blood collection, the purificat...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/08880010252899406

    authors: Pafumi C,Bosco P,Cavallaro A,Farina M,Leonardi I,Pernicone G,Bandiera S,Russo A,Giardina P,Chiarenza M,Calogero AE

    更新日期:2002-06-01 00:00:00

  • MYCN amplification by differential PCR.

    abstract::A method is described to estimate MYCN (N-myc) oncogene amplification in neuroblastoma by the technique of differential polymerase chain reaction (PCR). The technique is quicker than conventional Southern blotting techniques and does not require radioactive materials. The ability to measure MYCN amplification from sma...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880019309016525

    authors: Huddart SN,Mann JR,McGukin AG,Corbett R

    更新日期:1993-01-01 00:00:00

  • Neutropenic enterocolitis in children with acute lymphoblastic leukemia.

    abstract::Neutropenic enterocolitis is an acute, life-threatening inflammation of the small and large bowel, often seen in children with malignancies during periods of prolonged or severe neutropenia. During the period 1990-1995, 180 children were treated at the authors' center for acute lymphoblastic leukemia using a standard ...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/088800100276712

    authors: Jain Y,Arya LS,Kataria R

    更新日期:2000-01-01 00:00:00

  • Management of nephroblastoma with intracardiac extension: three case reports and review of the literature.

    abstract::A single institution's experience of three patients with nephroblastoma extending via the inferior vena cava to the right heart is described. The case reports and the pertinent literature illustrate that preoperative failure to detect the intracardial extension increases the risk of right outflow obstruction and pulmo...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章,评审

    doi:10.3109/08880019109028807

    authors: Kremens B,Sadony V,Goepel M,Hellinger A,Havers W

    更新日期:1991-10-01 00:00:00

  • Moyamoya syndrome associated with severe iron deficiency anemia in a young child.

    abstract::A 3-year-old boy presented with recurrent strokes and pallor. Hematological investigations revealed severe iron deficiency anemia without thrombocytosis. The magnetic resonance angiogragraphy findings were suggestive of moyamoya syndrome. The association of moyamoya syndrome with severe iron deficiency anemia has not ...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880018.2011.650838

    authors: Meena SS,Ramkumar TV,Sharma S,Aneja S,Kumar A

    更新日期:2012-05-01 00:00:00

  • Enteroviral meningoencephalitis in immunocompromised children after matched unrelated donor-bone marrow transplantation.

    abstract::Two children are described who presented with fever and generalized seizures, days 50 and 200, respectively, after matched unrelated donor-bone marrow transplantation. Upon antiepileptic treatment the seizures vanished but somnolence and fever remained. Magnetic resonance imaging (MRI) of the brain was performed and r...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/08880010050034337

    authors: Fischmeister G,Wiesbauer P,Holzmann HM,Peters C,Eibl M,Gadner H

    更新日期:2000-07-01 00:00:00

  • Nesiritide in a pediatric oncology patient with renal insufficiency and myocardial dysfunction following septic shock.

    abstract::Nesiritide is a recombinant formulation of B-type natriuretic peptide used most commonly in the treatment of adults with decompensated congestive heart failure. The physiologic effects of BNP include natriuresis, diuresis, and smooth muscle relaxation. These physiologic effects result in its beneficial therapeutic eff...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/08880010590935248

    authors: Wheeler AD,Tobias JD

    更新日期:2005-06-01 00:00:00

  • Immunocompetent cells and lymphocyte reactivity to mitogens in levamisole-treated brain tumor children.

    abstract::This study investigated the influence of levamisole therapy on immunocompetent cells and lymphocyte reactivity to mitogens in 25 children with brain tumor. Eleven (11/25) patients were receiving chemotherapy and immunomodulating drug levamisole 3 months after neurosurgery, during maintenance chemotherapy, 2.5 mg/kg of...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/088800199277164

    authors: Hajnzić TF,Kastelan M,Lukac J,Hajnzić T

    更新日期:1999-07-01 00:00:00

  • Changes in cerebral blood flow in children with sickle cell disease after splenectomy.

    abstract::The study assessed changes in cerebral blood flow and need for chronic blood transfusions in sickle cell disease children after splenectomy. A retrospective chart review of 40 children splenectomized between 1999 and 2014 was performed. The mean time-average maximum velocity before splenectomy was 129 cm/sec; which in...

    journal_title:Pediatric hematology and oncology

    pub_type: 临床试验,杂志文章

    doi:10.3109/08880018.2015.1014589

    authors: Siddiqui AH,Soh PB

    更新日期:2015-05-01 00:00:00

  • Vincristine and irinotecan in children with relapsed hepatoblastoma: a single-institution experience.

    abstract:PURPOSE:This study was to determine the efficacy of vincristine and irinotecan in children with relapsed hepatoblastoma (HB). METHODS:A total of 10 patients with relapsed HB were enrolled. Three patients were excluded. Patients received irinotecan 50 mg/m(2)/day, day 1-5 and vincristine 1.5 mg/m(2)/day, day 1, repeate...

    journal_title:Pediatric hematology and oncology

    pub_type: 临床试验,杂志文章

    doi:10.3109/08880018.2014.909913

    authors: Zhang YT,Feng LH,Zhong XD,Wang LZ,Chang J

    更新日期:2015-02-01 00:00:00

  • Red blood cell distribution width is not a reliable biomarker for low iron stores in children with cystic fibrosis.

    abstract::Low iron stores in children, absolute iron deficiency (AID), can lead to impaired neurodevelopment and requires iron therapy. In the presence of infection/inflammation, like in cystic fibrosis (CF), serum ferritin (SF) is not a reliable biomarker for AID. Red blood cell distribution width (RDW) is a promising alternat...

    journal_title:Pediatric hematology and oncology

    pub_type: 临床试验,杂志文章

    doi:10.1080/08880018.2016.1268226

    authors: Akkermans MD,Uijterschout L,Nuijsink M,Hendriks DM,van Goudoever JB,Brus F

    更新日期:2017-02-01 00:00:00

  • Hematologic phenotype of the mutations IVS1-n6 (T-->C), IVS1-n110 (G-->A), and CD39 (C-->T) in carriers of beta-thalassemia in Greece.

    abstract::The hematologic phenotype was characterized in heterozygotes for three of the most common beta-thalassemia mutations in the Greek population. The study included 17 carriers of beta++ IVS1-n6 (T-->C), 21 carriers of beta+ IVS1-n110 (G-->A), and 17 carriers of beta 0 CD39 (C-->T). The 55 beta-thalassemia heterozygotes w...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880019409141689

    authors: Stefanis L,Kanavakis E,Traeger-Synodinos J,Tzetis M,Metaxotou-Mavromati A,Kattamis C

    更新日期:1994-09-01 00:00:00

  • Spontaneous remission of acute lymphoblastic leukemia with mediastinal mass.

    abstract::Spontaneous remission/regression of cancer is defined as partial or complete disappearance of malignant disease temporarily or permanently in the absence of medical treatment. This event is named as spontaneous regression for solid tumors and spontaneous remission for leukemia. The authors report the case of a girl ag...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/08880010801938132

    authors: Yoruk A,Erguven M,Celiker E,Aki H,Timur C,Yuksel E,Ozkan H

    更新日期:2008-04-01 00:00:00

  • Percutaneous transhepatic biliary drainage in an infant with obstructive jaundice caused by neuroblastoma.

    abstract::Neuroblastoma presenting with obstructive jaundice is a rare event. Management of this condition includes surgery, chemotherapy, radiotherapy, temporary cholecystostomy tube, endoscopic retrograde cholangiopancreatography (ERCP), and internal biliary drainage (IBD). We herein describe our experience with one infant af...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880018.2014.981901

    authors: Saettini F,Agazzi R,Giraldi E,Foglia C,Cavalleri L,Morali L,Fasolini G,Spotti A,Provenzi M

    更新日期:2015-04-01 00:00:00

  • Primary diffuse leptomeningeal glioneuronal tumors of the central nervous system: Report of three cases and review of literature.

    abstract::Primary diffuse leptomeningeal glioneuronal tumors (DLGNT) are rare tumors, recently recognized as a unique entity based on their unique pathologic and clinical characteristics. We report three cases of DLGNT and compare their clinical characteristics and presentation with other reported cases, and with primary leptom...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章,评审

    doi:10.1080/08880018.2019.1711270

    authors: Abongwa C,Cotter J,Tamrazi B,Dhall G,Davidson T,Margol A

    更新日期:2020-04-01 00:00:00

  • Infantile hepatic hemangioendothelioma with elevated serum alpha-fetoprotein.

    abstract::Infantile hemangioendothelioma is the most common hepatic vascular tumor in infants less than 6 months of age, with a prevalence of 1%. Serum alpha-fetoprotein levels have been used as an important tumor marker for hepatoblastoma, hepatocellular carcinoma, and germ cell tumors. It is rarely elevated in hepatic hemangi...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章,评审

    doi:10.1080/08880010600954397

    authors: Sari N,Yalçin B,Akyüz C,Haliloglu M,Büyükpamukçu M

    更新日期:2006-12-01 00:00:00

  • Central nervous system (CNS) tumors in the first six months of life: the Children's Hospital Los Angeles experience, 1979-2005.

    abstract:BACKGROUND:The authors report the experience at the Children's Hospital Los Angeles with brain tumors diagnosed before 6 months of age, describing the characteristics of the patients, their tumors, treatment strategies, and prognostic factors. METHODS:Thirty-three children who were identified between 1979 and 2005 wer...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880010903447342

    authors: Serowka K,Chiu Y,Gonzalez I,Gilles F,McComb G,Krieger M,Dhall G,Britt B,Ji L,Sposto R,Finlay JL

    更新日期:2010-03-01 00:00:00

  • Etiology, drug sensitivity profiles and clinical outcome of bloodstream infections: A retrospective study of 784 pediatric patients with hematological and neoplastic diseases.

    abstract::Bloodstream infections (BSI) represent one of the most serious complications in patients in the hematology-oncology unit. In this study, the prevalence, distribution, drug sensitivity profiles, and clinical outcome of BSI were analyzed in pediatric patients with hematological malignancies. Patients admitted to the ped...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/08880018.2019.1667462

    authors: Chen S,Liu S,Yuan X,Mai H,Lin J,Wen F

    更新日期:2019-11-01 00:00:00

  • Molecular characterization of homozygous (high HbA2) beta-thalassemia intermedia in Greece.

    abstract::Homozygous beta-thalassemia is usually characterized by severe anemia requiring regular blood transfusion for survival. For homozygous patients with milder clinical manifestations and no dependence on transfusion therapy, the term thalassemia intermedia is usually applied. Genetic mechanisms that may ameliorate the cl...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880019509029526

    authors: Kanavakis E,Traeger-Synodinos J,Tzetis M,Metaxotou-Mavromati A,Ladis V,Kattamis C

    更新日期:1995-01-01 00:00:00

  • Autologous bone marrow transplantation in pediatric solid tumors.

    abstract::During the last 5 years massive chemotherapy and autologous bone marrow transplantation have been increasingly explored in the treatment of pediatric solid tumors, mainly for neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, Wilms' tumor, germ cell tumors, osteosarcoma, and retinoblastoma. Although the disease course ...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章,评审

    doi:10.3109/08880019009034318

    authors: Yaniv I,Bouffet E,Irle C,Negrier S,Biron P,Favrot M,Philip I,Brunat-Mentigny M,Philip T

    更新日期:1990-01-01 00:00:00

  • Primary benign erythrocytosis with high erythropoietin levels and an early erythropoietin-sensitive population in the peripheral blood.

    abstract::Primary erythrocytosis diagnosed in a 10-month-old female and followed for 12 years is described. The erythrocytosis was associated with an abnormally elevated set point of erythropoietin production in which the sensitivity fluctuated independently, but corresponded to the alterations in the oxygen-carrying capacity o...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880019109033455

    authors: Kalmantis T,Kalmanti M

    更新日期:1991-07-01 00:00:00

  • Neutrophil apoptosis in patients with beta-thalassemia major.

    abstract::Increased susceptibility to infection is reported in patients with beta-thalassemia major due to toxic effect of iron on neutrophil functions and reticuloendothelial system dysfunction. This study investigated the association between the neutrophil apoptosis and frequency of infection episodes, desferrioxamine treatme...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/713842280

    authors: Oren H,Sahin B,Irken G,Ateş H,Duman M,Yilmaz S,Türker M,Atabay B,Yaprak I

    更新日期:2003-04-01 00:00:00

  • Inflammatory pseudotumor of the lung in children.

    abstract::Two children with inflammatory pseudotumor (IPT) of the lung are reported. Symptomless "cystic" lesions were present on routine chest x ray. Morphological study of these peculiar lesions included light microscopic, immunohistochemical, and ultrastructural analysis. Histologic appearance of the lesion varied from the f...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880019109033439

    authors: Vujanić GM,Dojĉinov D

    更新日期:1991-04-01 00:00:00

  • Experience with cefepime versus meropenem as empiric monotherapy for neutropenia and fever in pediatric patients with solid tumors.

    abstract::A prospective, open-label, randomized, comparative study in pediatric cancer patients was conducted to evaluate the efficacy and safety of cefepime and meropenem in the empiric therapy of febrile neutropenic patients. Febrile episodes were classified as microbiologically documented infection, clinical documented infec...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章,随机对照试验

    doi:10.1080/08880010500506867

    authors: Oguz A,Karadeniz C,Citak EC,Cil V,Eldes N

    更新日期:2006-04-01 00:00:00

  • Angioimmunoblastic lymphadenopathy with dysproteinemia: report of a case in infancy with review of literature.

    abstract::A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinica...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章,评审

    doi:10.3109/08880018909014579

    authors: de Terlizzi M,Toma MG,Santostasi T,Colella R,Ceci A,De Benedictis G

    更新日期:1989-01-01 00:00:00

  • A variant of myelokathexis with hypogammaglobulinemia: lymphocytes as well as neutrophils may reverse in response to infections.

    abstract::A 7-year-old boy with prolonged and marked leukopenia diagnosed at 6 months of age is described. The polymorphonuclear cells presented no hypersegmented nuclei or concentrated nuclear chromatin, although vacuolated myeloid cells appeared in bone marrow smears. Neutrophils reversed in response to administration of G-CS...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.1080/088800100276532

    authors: Arai J,Wakiguchi H,Hisakawa H,Kubota H,Kurashige T

    更新日期:2000-03-01 00:00:00

  • Case of a child with paroxysmal nocturnal hemoglobinuria diagnosed by flow cytometry.

    abstract::A case of a child with paroxysmal nocturnal hemoglobinuria (PNH) is characterized by an increased sensitivity of the erythrocyte to hemolytic action of complement. The widely used Ham test may not always be reliable. Recently, a panel of monoclonal antibodies has become available to detect various glycosylphosphatidyl...

    journal_title:Pediatric hematology and oncology

    pub_type: 杂志文章

    doi:10.3109/08880019309029514

    authors: Kinugawa N,Shichishima T,Nishimura J,Kitani T

    更新日期:1993-10-01 00:00:00