ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Marrow hematopoiesis was examined in two infants with erythroleukemia (EL) using cell culture colony assays. At diagnosis, marrow from both yielded normal to increased numbers of granulocytic colonies (CFU-C), ery-throid bursts (BFU-E), and mixed colonies (CFU-GEMM), which contrasted sharply with the reduced growth in eight other newly diagnosed patients with acute leukemia. BFU-E proliferation and hemoglobinization proceeded normally in culture and was entirely erythro-poietin-dependent. CFU-C colony cellular composition showed normal granulopoiesis in various stages of development. Despite low numbers of morphologically recognizable blasts in the aspirates, they were readily identified in a blast colony assay because of their high plating efficiency and high index of self-renewal on replating. Blast cells in all cultures had myeloblastic morphology, were peroxidase positive, and expressed the granulo-cytic-specific My-1 antigen. Monosomy 7 was seen in fresh and cultured blast cells but not in lymphocytes, indicating a clonal proliferation. After chemotherapy-induced remission, blast colonies and monosomy 7 could no longer be detected. It appears that the integrity and function of the normal hematopoietic progenitor pool were preserved in these patients with EL. The abnormal myeloblastic proliferation was expressed actively in colony assays and was useful diagnostically in these cases because marrow morphology was nondiagnostic. In these patients, EL seems to be a misnomer since the findings are suggestive of acute myeloblastic leukemia with secondary erythroid and granulocytic hyperplasia.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

A 3-year-old girl presented with bilateral renal masses. The histologic diagnosis was lymphoblastic lymphoma. Cell surface markers showed a cytotoxic/suppressor T cell phenotype. The patient was treated with APO therapy. She has remained continuously disease-free for 30 months and has been off all therapy for 6 months. This rare case supports the value of precise classification (by histologic and immunologic methods) as a guide to appropriate tumor therapy.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Hemoglobin Hammersmith, a rare unstable hemoglobin. was diagnosed in a 4-year-old Chinese girl living in Los Angeles. She presented with the typical manifestations of this disorder, including neonatal hyperbilirubinemia. followed by increasing hepatosplenomegaly, jaundice, bili-rubinuria, and a severe hemolytic anemia exacerbated by mild infections. The most prominent manifestations of the peripheral smear were polychromasia, normoblas-temia, and basophilic stippling. The diagnosis was confirmed by several techniques.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

A 17-year-old previously healthy girl is reported who developed acute infectious mononucleosis followed by progressive ill health over 20 months, associated with pan-cytopenia and a polyclonal B-lymphoproliferation, terminating in acute lymphoblastic leukemia (ALL). Epstein-Barr virus (EBV) was recovered from the patient's nasopharyngeal secretions; serologic titers of antibodies to EBV-associated antigens were compatible with a chronic persistent EBV infection. Plasma interferon levels were markedly elevated. EBV-specific cell-mediated immunity, as well as Natural killer (NK) cell activity were markedly deficient. Other studies of cell-mediated immunity revealed notable abnormalities, including abnormalities in T-cell subset ratios, and a serum blocker of autologous mitogen-induced lymphoprolifera-tion.Humoral (plasma)-mediated, but not cell-mediated, suppression of hemopoiesis was demonstrated using in vitro erythroid and myeloid colony culture techniques.Immunophenotyping of the patient's bone marrow cells preterminally was consistent with ALL. Autopsy revealed pathologic changes of ALL in marrow and multiple organs. We conclude that our patient developed an EBV-driven lymphoproliferative disorder, with associated defective cell-mediated immunity and hemopoiesis. Ultimately, the patient's documented polyclonal lymphoproliferative state was superimposed by acute lymphoblastic leukemia.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

The interpretation of small numbers of lymphoblasts or of nonspecific pleocytosis in the cerebrospinal fluid (CSF) of children with lymphoid malignancies is difficult. In this prospective study of 204 patients, 15 had white cell chamber counts of ≤10 cells/μl, with one or more blasts on a cytocentrifuged smear. Only 3/15 subsequently developed meningeal leukemia, and 4/15 remain in continuous complete remission. Among 53 patients with a chamber count >10 WBC/μl and no blasts, pleocytosis was associated with central nervous system (CNS) prophylaxis in half. Less common were viral syndromes, immune recovery, subsequent meningeal leukemia, active hematologic leukemia, the somnolence syndrome, leu-koencephalopathy, or reaction to a previous traumatic lumbar puncture.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

To determine the value of the direct platelet associated IgG (PAIgG) level as a prognostic indicator in childhood idiopathic thrombocytopenia purpura (ITP), 18 children with ITP were studied. Ten of the 18 had PAIgG levels measured at diagnosis, before any therapy. Of these 10 patients, six (Group I) had an acute course, with a mean initial platelet count of 15 ± 109/liter and a mean initial PAIgG level of 330.9 fg/plt. Four patients (Group II) had a chronic course, with a mean initial platelet count of 11 ± 109/liter and a mean initial PAIgG level of 38.3 fg/plt. There was no significant difference between the mean initial platelet count of Groups I and II (p > 0.10), but the initial PAIgG levels in those patients with an acute course were significantly higher than the levels in those patients with a chronic course (p < 0.05). Of the original 18 patients, nine were splenectomized for chronic thrombocytopenia, with normalization of the platelet count in all instances. Of these splenectomized patients, five had platelet counts and PAIgG levels measured before and after splenectomy. All five had normal PAIgG levels following splenectomy. The PAIgG level is a good prognostic indicator for the clinical course of childhood ITP. A high PAIgG level suggests an acute course while a modestly elevated level suggests a chronic course. The PAIgG level normalizes in remission after splenectomy.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

A case of a congenital hepatic vascular malformation in a child complicated by disseminated intravascular coagulation and hemolytic anemia is presented. Examination of the peripheral blood disclosed the presence in the leukocytes of giant intracytoplasmic inclusions resembling those of the Chediak-Higashi anomaly. Ultrastructural analysis characterized those inclusions as phagocytosed red cell debris, a result of mechanical destruction of the red cells.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Acute childhood leukemia was found to be less common in black children than in white children in the Johannesburg area of South Africa. Of 195 consecutive patients, 78 were black and 117 white. The annual incidence was 0.8/100,000 black children and 3.3/100,000 white. The low incidence in black children was due to the very low incidence of acute lymphoblastic leukemia (ALL), which was 0.41/100,000 black children compared with 2.73/100,000 white children. The incidence of acute non-lymphoblastic leukemia (ANLL) was approximately the same for both ethnic groups; 0.38/100,000 black children and 0.57/100,000 white children. Remission rates for black children with ALL were lower than for white children and the cumulative proportion of black patients surviving at 60 months was only 32% compared with 72% of white patients (p = 0.0001). The most significant poor prognostic factors in ALL were ethnic group and age (p = 0.0006), CNS disease at onset (p = 0.006), FAB L2 and L3 morphology (p = 0.05). and irregular clinic attendance during maintenance therapy (p = 0.05). In ANLL, remission and survival rates were less favorable than in ALL but there were no significant differences between black and white patients. Black patients exhibited certain clinical features rarely seen in white patients, including chloromata, oropharyngeal lesions, and CNS involvement at onset. Karyotypic abnormalities were common. The most significant poor prognostic factors in ANLL were CNS disease at onset (p = 0.03), generalized lymphadenopathy (p = 0.0001), and FAB morphology classification M3-M6 (p = 0.05). Certain patients with FAB Ml and M2 morphology appeared to have the potential for long-term survival. The possible significance of these findings in relationship to ethnic and environmental factors is discussed.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

In a 4-year period 168 tumor specimens received from 119 pediatric patients were plated in a soft agar cloning system. Overall, 47 or 28% of the tumor specimens had growth adequate enough for drug sensitivity testing. Drug sensitivity testing was performed using a variety of standards as well as investigational anticancer agents. Overall, 129 evaluable in vitro drug sensitivity tests were performed with 33 (26%) of the tests showing sensitivity to an agent. The most active drugs in vitro included the standard agents doxorubicin and cis-platinum as well as the investigational agents mitoxantrone and m-AMSA. From these data it is clear the cloning assay can be used to study pediatric malignancies. However, before the cloning assay can be applied to clinical pediatric oncology practice, the assay must be improved in terms of tumor growth and validated with prospective clinical trials.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID