摘要:

A generalized lymphadenopathy syndrome (GLS) occurs in persons at high risk for development of acquired immunodeficiency syndrome (AIDS). The natural history and immunologic status of patients with GLS are not fully known, although in some persons GLS may progress to full AIDS. We present the clinical and immunologic findings in two children with severe hemophilia A with nonprogressive GLS for 18–24 months. The functional activity in vitro of lymphocytes from both peripheral blood and biopsied lymph nodes were compared. The peripheral blood lymphocytes responded normally to both mitogens and antigens: lymph node lymphocytes failed to respond to antigens, but did respond to mitogens. The implications of these abnormalities for understanding the pathogenesis of GLS are discussed.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Neuroblastoma (NB) is a tumor usually arising in children and young adults showing different degrees of malignancy. Recently, the presence ofN-mycamplification in neuroblasts has been associated with a poor outcome in the late stages of disease (Evans's Stage IV). Until now no amplification ofN-mycgene has been observed in Stage IV-S, usually considered to have a favorable prognosis. In this paper we report a case of a child affected by NB Stage IV-S showing mildN-mycgene amplification.The finding ofN-mycamplification in our patient shows that such alteration of theN-myconcogene is not necessarily correlated with a poor prognosis: in this light the role ofN-mycamplification in the neoplastic process should be reconsidered.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Superoxide (O2−) generation of leukemic cells was investigated in 28 children with acute leukemia. Leukemic cells of seven with acute myelomonocytic leukemia and one with acute monocytic leukemia produced various amounts of O2−(0.13–0.87 nmol/min/106cells) when stimulated with wheat germ agglutinin (WGA) and phorbol myristate acetate (PMA). Other types of leukemia cells exhibited no such functions. Leukemic cells of two patients diagnosed as unusual cases of acute lymphoblastic leukemia generated significant amounts of O2−when stimulated with WGA and PMA. Both patients went into complete remission while on acute nonlymphoblastic leukemia treatment. Thus O2−generating activity can serve as a functional marker of leukemic cells in myelomonocytic lineage.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Twenty-four girls were studied following long-term treatment (X:50 months) for acute lymphoblastic leukemia: 14 were prepubertal and 10 pubertal. Follow-up during endocrine studies ranged from 2 months to 6.7 years (X: 2.3 years). Five of 14 prepubertal patients started clinical pubertal development at a normal age and were reevaluated during puberty, increasing the pubertal group to 15 patients. Thirteen of 15 pubertal patients had received cranial radiotherapy. Ten of 15 pubertal patients started menses during the endocrine study. Although age of menarche was normal, in nine patients it was below the normal mean. Except for the remaining patient, all had received cranial cobalt therapy. In 6 of 19 patients bone age was significantly accelerated. Serum gonadotrophin response to LH-RH was normal in 13 prepubertal patients and in 10 pubertal patients. In 3 of 10 pubertal patients follicle-stimulating hormone (FSH) values were temporarily elevated. Only one pubertal patient had oligoamenorrhea. Five patients were studied by measuring serum progesterone on days 19–22 of the cycle to determine corpus luteum function. Three of them showed progesterone levels compatible with adequate corpus luteum function (6, 19, and 12 ng/ml, respectively) and two presented low progesterone levels (2 ng/ml), probably because of their short gynecological age (0.24 and 0.3 years, respectively). This study suggests that neither the disease nor the long-term antileukemia therapy seems to injure gonadal function in girls. A tendency to early sexual development was observed, which may be related to cranial cobalt therapy.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

A 15-year-old boy with lymphadenopathy and marked leukocytosis was evaluated. At 2 years old, he was diagnosed to have malignant testicular carcinnoma for which radiotherapy and cytotoxic drugs were administered. On admission, the peripheral blood showed an increase of all stages of the granulocytic cell line and atypical monocytoid cells. Cervical lymph node biopsy showed infiltration by immature granulocytic cells. Eleven percent of the peripheral leukocytes was positive for combined staining by α-naphthyl butyrate esterase and naphthol-ASD-chloroacetate esterase activity. The leukocyte alkaline phosphatase score was low. Serum and urinary lysozyme levels were markedly elevated. Philadelphia chromosome was not detected. Although chronic myelomonocytic leukemia (CMML) has been described in elderly patients, the findings in this pediatric patient were most compatible with a diagnosis of CMML.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

A newborn infant was diagnosed as having congenital basophilic leukemia, an extremely rare disorder, by examination of cord blood. An ultrastructural cytochemical study was conducted on the blasts of the infant. Granules of basophil blasts showed ultrastructural characteristics of immature basophil granules and were similar in size to those of normal basophil granules but were fewer in number in each profile. Histochemical studies showed weak reactions for acid mucopolysaccharides and neutral glycoconjugates in the granules, but they were negative for peroxidase. Acid phosphatase was markedly positive. The findings of congenital basophilic leukemia are different from those of basophils in chronic myelocytic leukemia. The patient underwent induction therapy with vincristine and prednisone and has been in complete remission for 21 months.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Microcomputer assisted data management techniques, utilized in the oversight of two multiinstitutional trials for children with previously untreated acute lymphoblastic leukemia and unfavorable prognostic features, are presented. In the first study, such oversight hastened identification of unexpectedly prolonged delays, so that treatment could be successfully modified. In the second study, inferiority of one therapy was rapidly demonstrated, and after careful review that therapy was halted. Performance monitoring is discussed in terms of (1) consideration of the overall quality of a clinical trial, namely, how complete are the data submitted and how precisely was protocol therapy administered; (2) measurement of the quality of institutional participation, and (3) identification of deficiencies in reporting or performance in the records of individual patients.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

The use of cytochemical, electron microscopic, immunofluorescent, and tissue culture techniques has led to important advances in our understanding of the mechanisms underlying the pathogenesis of the Chediak—Higashi syndrome (CHS). This rare and fatal autosomal recessive disorder is clinically characterized by partial albinism, frequent pyogenic infections, and an accelerated lympho-histiocytic phase. The pathological hallmark of CHS is the presence in all white blood cells of massive lysosomal inclusions, which are formed through a combined process of fusion, cytoplasmic injury, and phagocytosis. The abnormal inclusions exhibit both azurophilic and specific granular markers, and are probably responsible for most of the impaired leukocyte and other cell functions in CHS patients. In addition, a selective profound natural killer (NK) cell function and platelet storage pool deficiencies have been described in these patients. Impaired microtubule assembly and functions, mediated by abnormal intracellular cyclic nucleotide levels, which could be corrected by treatment with ascorbic acid, were suggested to be the pathophysiological basis for CHS abnormalities. However, some recent studies have questioned this cyto-skeletal model, which is suggested to be rather a secondary manifestation of CHS.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Defining cure for a child with cancer is not simple. Such terms as “long-term disease-free survival” or “no evident disease” are expressions of our current clinical inadequacies. An equally important problem, however, is that the disease and its treatment are frequently associated with sequelae that prevent complete restoration of baseline health status or that alter subsequent growth and developmental patterns. This article describes the nature of cures that have been achieved for childhood cancer and reassesses current treatment goals, taking into consideration altered realities of survival. Tasks remaining include the construction of medical treatment regimens that minimize acute and late effects, the design of intervention strategies to reduce the psychological and social impact of the disease on children and families, and the modification of society's attitudes so that cancer survivors are more accepted in the community.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID