The American journal of pediatric hematology/oncology (J Pediatr Hematol Oncol )

Publisher: American Society of Pediatric Hematology/Oncology


Discontinued. Now published as the Journal of Pediatric Hematology/Oncology.

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    The American journal of pediatric hematology/oncology
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    Periodical, Internet resource
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    Journal / Magazine / Newspaper, Internet Resource

Publications in this journal

  • The American journal of pediatric hematology/oncology 01/2000; 22(4):392.
  • The American journal of pediatric hematology/oncology 01/1997; 19(4):383.
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    The American journal of pediatric hematology/oncology 01/1997; 19(1).
  • The American journal of pediatric hematology/oncology 01/1997; 19(2).
  • [Show abstract] [Hide abstract]
    ABSTRACT: A 3-year-old girl with acute lymphoblastic leukemia developed typhlitis immediately after diagnosis and before the institution of chemotherapy. Typhlitis is a necrotizing colitis that develops in immunodeficient patients with severe neutropenia. Most patients are leukemic children who are receiving or have received chemotherapy. Typhlitis in the absence of chemotherapy is rare. We report a fatal case of typhlitis in an untreated leukemic patient. This case emphasizes the multifactorial pathogenesis of typhlitis, which may occur in the absence of chemotherapy. Typhlitis should always be considered as a potential cause of acute sepsis and abdominal pain in a leukemic patient.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):348-51.
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    ABSTRACT: We describe a newborn baby boy with anemia in whom human parvovirus (HPV) antibodies were detected in his serum. Severe reticulocytopenia and anemia lasted until day 11 after his birth. Thereafter reticulocytosis was observed. Work-ups for immune hemolytic anemia, fetomaternal hemorrhage, placental hemorrhage, intrauterine infections were negative by clinical and laboratory analysis. HPV-specific IgM and IgG were positive on both the baby's and the maternal serum. Despite severe anemia, the baby remained clinically stable. His hemoglobin levels steadily increased along with ongoing reticulocytosis. He is now 14 months old with normal hemoglobin. Detection of HPV antibodies indicated its causal relation to the development of congenital pure red cell aplasia. The possibility of HPV infection should be considered in the assessment of anemia of the newborn.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):352-5.
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    ABSTRACT: After the harvesting of bone marrow for use in transplantation, pain control needs are greatest in the first 12 to 24 hours. This is also the time during which systemic side effects of parenterally administered narcotics are greatest as a result of the recent general anesthetic. We examined the role of regional anesthesia as an adjunct to pain management in pediatric patients undergoing bone marrow harvest. Ten pediatric bone marrow donors were treated with intraoperative caudal blocks using bupivacaine or a combination of bupivacaine and morphine. Donors who received regional anesthesia had a significant decrease in postoperative parenteral narcotic requirement and fewer systemic complications when compared with a control group of 10 age-matched children who did not receive the caudal block. Regional anesthesia with caudal block can decrease the morbidity associated with bone marrow harvest in donors.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):305-8.
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    ABSTRACT: We present the association of a lupus anticoagulant with hypoprothrombinemia in a 5-year-old girl, who presented with ecchymoses and a hematoma. This coagulopathy should be included in the differential of bleeding in the previously healthy children. Coagulation and immunology laboratory evaluation was performed at the time of presentation with bleeding and 2 months later, after complete clinical recovery. A 5-year-old girl presented with ecchymoses and a hematoma after after an upper respiratory illness. Laboratory evaluation showed prolongation of both the prothrombin time (PT) and activated partial thromboplastin time (aPTT) due to the presence of a strong lupus anticoagulant associated with a decreased level of prothrombin (15 U/dl). Hypocomplementemia was also detected. Bruising resolved spontaneously, and the PT and aPTT gradually normalized. Reevaluation 2 months later showed that the lupus anticoagulant had disappeared and the prothrombin deficiency was markedly improved. This case demonstrates that transient lupus anticoagulants must be included in the differential for bleeding in young children. Also, in children with lupus anticoagulants, neither the association of hypoprothrombinemia nor the presence of evidence of activation of the immune system appears to predict whether a patient will have or develop systemic lupus erythematosus.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):372-6.
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    ABSTRACT: Malignancy of the sphenoid sinus, especially metastatic involvement, is an extremely rare event at any age and is usually associated with a poor prognosis. We present a case of a 5-year-old boy who underwent allogenic bone marrow transplantation for stage IV neuroblastoma. Three years later, he presented with an isolated lesion, histologically proven to be neuroblastoma, in the sphenoid sinus. Partial excision of the tumor, followed by chemotherapy and radiotherapy, achieved a remission for 3 years.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):377-9.
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    ABSTRACT: Four children with prolonged emesis during brain tumor therapy were diagnosed with pancreatitis. All were exposed to medications or radiotherapy that potentially contributed to pancreatitis. Because recognition of pancreatitis may necessitate changes in supportive care, pancreatitis should be included in the differential diagnosis of vomiting in this population.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):301-4.
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    ABSTRACT: Erythropoietin-dependent pure erythrocytosis (EDPE) is a rare disorder caused by idiopathic hypererythropoietinemia. We describe a 13-year-old girl who developed an EDPE-like erythrocytosis after removal of an adrenal pheochromocytoma. As occurs in EDPE, this post-pheochromocytoma erythrocytosis was associated with a high serum erythropoietin (s-Epo) level that maintained physiological regulation. Phlebotomies produced a three- to sixfold increase of s-Epo, and a 6-week course of theophylline caused a decrease of both s-Epo and hemoglobin. We hypothesize that the intense and prolonged pheochromocytoma-induced renal ischemia before surgery could be the cause of this unique case of erythrocytosis.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):365-7.
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    ABSTRACT: We report a case of adrenal cortical carcinoma in an infant, which was incidentally discovered by renal sonography after a urinary tract infection. The previous death of a sibling after rhabdomyosarcoma in infancy prompted a search for a heritable p53 tumor suppressor gene mutation in this family. Starting with frozen adrenal carcinoma tissue, polymerase chain reaction (PCR) amplification followed by direct sequencing of exons 4-8 of p53 was used to search for a mutation. When a mutation was identified in exon 6 of the tumor p53 sequence, PCR amplification and direct sequencing of exon 6 alone was then performed on DNA from peripheral blood lymphocytes (PBLs) of all immediate family members to determine whether a germline mutation was present. A different set of primers was used by a second laboratory at our institution to independently confirm the presence of the mutation in the adrenal carcinoma and in paraffin-embedded rhabdomyosarcoma tissue of the deceased sibling. A C-to-T transition was identified at a CpG site in codon 196 resulting in a change from arginine to a stop codon (CGA to TGA). The identical mutation, present as the sole p53 allele in the tumor DNA samples and in the heterozygous state with wild type p53 allele in DNA from PBLs (germline), was found in the adrenal carcinoma, the rhabdomyosarcoma, and the PBLs of the tumor-bearing child and her healthy father and 5-year-old brother. This nonsense mutation of p53 has never before been reported in the germline. The extended pedigree showed only one known additional cancer. A novel germline p53 mutation was identified by investigation of a sibling pair with cancers associated with the Li-Fraumeni syndrome in a family with an otherwise negative history for cancer. The implications of this case for identification of carriers of p53 germline mutations and their clinical management are discussed.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):341-7.
  • The American journal of pediatric hematology/oncology 12/1994; 16(4):384-6.
  • The American journal of pediatric hematology/oncology 12/1994; 16(4):361-4.
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    ABSTRACT: We report a case of infection-associated hemophagocytic syndrome in the setting of disseminated histoplasmosis. A 6-year-old boy with chronic mucocutaneous candidiasis developed a fulminant hemophagocytic syndrome. Evaluation for an infectious cause included bacterial, mycobacterial, viral, and fungal cultures, serological assessment, and histological examination of bone marrow and bronchoalveolar lavage fluid. Culture of bone marrow aspirate, blood, and bronchoalveolar lavage showed disseminated histoplasmosis as the cause for this patient's hemophagocytic syndrome. The patient was treated with amphotericin B with complete resolution of his hemophagocytic syndrome. Disseminated histoplasmosis is another cause of IAHS in children with an underlying immunodeficiency. Diagnostic difficulties associated with the hemophagocytic syndromes of childhood are discussed.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):368-71.
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    ABSTRACT: Infection with human parvovirus B19 is the most common cause of transient aplastic crisis in patients with chronic hemolytic anemia. Multiple reports of children with simultaneous B19 infection and thrombocytopenia as well as the known association between experimental B19 infection and thrombocytopenia prompted us to hypothesize that B19 may be associated with childhood idiopathic, or immune, thrombocytopenic purpura (ITP). Because there is a paucity of evidence regarding a viral etiology for ITP, we performed a comprehensive study to explore its possible relationship to B19 infection. Thirty-five previously healthy children with ITP were studied prospectively. Bone marrow and peripheral blood were analyzed for B19 DNA using the polymerase chain reaction (PCR). Serum was analyzed for anti-B19 immunoglobulin (Ig) M and IgG antibodies using a B19 VP1 antigen-based enzyme-linked immunosorbent assay. Fourteen healthy children served as controls for peripheral blood PCR and serologic analyses. The presenting clinical and laboratory features of the study population were typical of classic ITP. Seventeen of the 35 patients (49%) had evidence of B19 DNA in the peripheral blood, bone marrow, or both. Six of 35 (17%) had anti-B19 IgM antibodies. Eight of 35 (23%) were anti-B19 IgG seropositive. The control group had no positive PCR or anti-B19 IgM specimens. Our results suggest that infection with human parvovirus B19 may be associated with childhood ITP. More investigation is warranted regarding the role of PCR methodology and serologic detection methods in defining B19 pathobiology as it relates to ITP.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):314-9.
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    ABSTRACT: We present a report of a child with subclinical immunodeficiency who became chronically infected with parvovirus resulting in pancytopenia and morphologic abnormalities in the bone marrow mimicking myelodysplastic syndrome (MDS). An 8-year-old boy presented with severe anemia, moderate thrombocytopenia and granulocytopenia. The patient showed hyper-immunoglobulin M (IgM) immunodeficiency but no increased susceptibility to infections. The bone marrow was hypercellular with dysplastic granulocytopoiesis and erythroblastopenia. Treatment with cyclosporine and i.v. Ig resulted in temporary normalization of the hemoglobin level. For several years it was assumed that the patient had MDS. A diagnosis of parvovirus infection was initially rejected due to the lack of specific antibodies and the absence of giant pronormoblasts in the bone marrow. When the polymerase chain reaction technique became available, parvovirus DNA was detected from the entire disease course. This case report expands our conception of the clinical spectrum of parvovirus infection and emphasizes that parvovirus must be considered as a differential diagnosis in MDS. We recommend performing a parvovirus DNA test despite negative serologic findings in patients with MDS, especially when associated with immunologic abnormalities.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):329-33.
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    ABSTRACT: To prospectively evaluate 50 patients with transient erythroblastopenia of childhood (TEC) at a single institution in order to compare those patients presenting with reticulocytopenia (group I) with those presenting in the recovery phase with reticulocytosis (group II); to further describe the clinical course of this common pediatric hematological disorder in a large number of patients, particularly the effect on the neutrophils; and to review the available literature regarding this disorder. Fifty patients presenting to the Children's Hospital from September 1983 to September 1991 were prospectively evaluated. Those patients with a reticulocytosis and in recovery at the time of diagnosis were included and compared with those with reticulocytopenia. All patients were followed through complete recovery. Thirty-six patients were reticulocytopenic (group I) and 14 had a reticulocytosis (group II). There was a high incidence of neutropenia (64%) in both groups and the resolution of this neutropenia was variable in relation to the resolution of the anemia, with 44% having the lowest ANC before, 9% simultaneous with, and 47% after the peak reticulocyte count. Our experience with a large group of patients with TEC suggests that neutropenia is an integral part of this disorder, and its recovery has no relation to the recovery of the anemia. A significant number of patients are described in the recovery phase for the first time, and this clarifies this group of patients in order to aid in their diagnosis, particularly in the differentiation from a hemolytic process. Some previously described associations of TEC are not supported in this study of a large number of patients.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):320-4.
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    ABSTRACT: Despite the excellent prognosis for 90% of patients with Wilms' tumor, survival remains poor among those with recurrent or advanced disease or tumors of unfavorable histology. We sought to identify a chemotherapy regimen for this subset of patients that offers potential efficacy with minimal nephrotoxicity. Through a review of patients' medical records, we compared the efficacy and nephrotoxicity of ifosfamide, cisplatin, cisplatin/etoposide, and ifosfamide/carboplatin/etoposide (ICE) regimens in 32 patients with recurrent (n = 23), refractory (n = 1), or metastatic (n = 8) Wilms' tumor, including six with tumors having unfavorable histologic features. Single-agent ifosfamide was minimally nephrotoxic and induced responses in three of 11 patients, but none have survived. Cisplatin with or without etoposide induced responses in six of 18 patients with recurrent Wilms' tumor (there is one long-term survivor). Seven of eight patients with newly diagnosed extensive metastatic disease responded to cisplatin/etoposide plus vincristine, dactinomycin, adriamycin, and radiotherapy. This regimen produced three long-term survivors, but was associated with significant nephrotoxicity. The ifosfamide, carboplatin, and etoposide regimen induced responses in four of five patients treated, and had minimal nephrotoxicity. Two remain free of disease progression 22 months after recurrence. Although long-term survival remains to be determined, the ICE combination appears to be effective against recurrent Wilms' tumor without endangering the patients' single remaining kidney. Myelotoxicity can be ameliorated by administering growth factors. We suggest that ICE chemotherapy be considered for the primary treatment of high-risk patients with Wilms' tumor.
    The American journal of pediatric hematology/oncology 12/1994; 16(4):296-300.
  • The American journal of pediatric hematology/oncology 12/1994; 16(4):387-8.