摘要:

Glucocorticoid receptors were quantified in circulating lymphoblasts from a child with relapsed acute lymphocytic leukemia refractory to multiple glucocorticoid containing drug regimens. The number of receptor sites in this patient's lymphoblasts was found to be similar to the number of receptor sites in lymphoblasts from a group of newly diagnosed children with acute lymphocytic leukemia. This patient differs from previous reported cases of relapsed acute lymphocytic leukemia in that all previous relapsed patients with normal receptor numbers obtained complete remissions when glucocorticoid containing treatment regimens were employed.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

Fetal hydantoin syndrome (FHS), a characteristic pattern of altered growth and development, has been well described in recent years in offsprings of epileptic mothers taking phenytoin or other hydantoin anticonvulsants during the gestational period. Recent reports of neuroblastoma in three patients with the FHS further raise the questions of the “oncogenic effect” of hydantoin compounds. A case of melanotic neuroectodermal tumor of infancy (MNTI) has been studied clinically and pathologically including light microscopy, histochemistry, and electron microscopy. This case strengthens the evidence for the teratogenic and oncogenic effects of hydantoin compounds and we believe that it represents the first reported case of FHS associated with MNTI. It would be most important from a clinical standpoint to carefully scrutinize individuals with the FHS for neoplasias. Furthermore, detailed gestational drug history in children with neuroblastoma and other neoplasias should be carefully searched for, with the hope of clarifying the definitive oncogenic effect of hydantoin compounds.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

A patient with leukemic optic nerve infiltration is presented. It occurred as an isolated manifestation of leukemia 17 months after therapy for acute lymphocytic leukemia was discontinued. Therapy with prednisone and vincristine was effective in reducing optic nerve infiltration in a short period of time. This observation leads to the conclusion that the optic nerve does not belong to the pharmacological sanctuary.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

A case of Philadelphia (Ph‘) chromosome negative chronic myeloid leukemia (CML) in a child is reported. The age of onset (7 years) and duration of survival (40 months) are not typical of juvenile CML and the relatively low leukocyte and hemoglobin F concentrations are more akin to the findings in Ph’ negative CIVIL in adults. There was refractoriness to chemotherapy with the exception of a dramatic response to a combination of prednisone and vincristine, despite the absence of terminal transferase activity in circulating blood cells. Splenic irradiation, splenectomy, and leukaphoresis were ineffective. Transition to a terminal aggressive phase caused a marked change in the growth characteristics of peripheral blood cellsin vitro.This patient may represent a new variant in the spectrum of childhood CML.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

A patient with ataxia telangiectasia presenting with a severe recurrent bleeding diathesis characterized by a prolonged bleeding time, normal platelet counts and clot retraction, absent platelet aggregation, and normal platelet factor 3 availability is described. These findings are indicative of a thrombasthenic-like pattern associated with multiple membrane receptor site defects. Chromosomal studies revealed a 14/14 tandem translocation involving chromosomal band 14q32 in peripheral T-lymphocytes; this chromosomal marker was not found in peripheral B-lymphocytes, direct bone marrow preparations, or skin fibroblasts.We postulate that the platelet functional defect demonstrated in this patient occurred in a clone of abnormal platelet stem cells possibly containing the chromosomal marker. This defect could be analogous to the situation in chronic myelogenous leukemia in which similar platelet functional disorders have been noted and in which the marker Philadelphia chromosome has been present on megakaryocytes. Our patient would also appear to be at high risk for the development of a T-cell malignancy.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

The present report describes two young males with clinically diagnosed infectious mononucleosis (IM) who subsequently were diagnosed as having malignant B-cell lymphoma (i.e., immunoblastic sarcoma of B-cells). Despite these apparent similarities, there were fundamental differences between the two cases. The first patient, whose lymphoma was diagnosed 9 months after IM, was one of a well-described kindred with the X-linked lymphoproliferative syndrome (XLP) in which affected young males lack the ability to mount an effective immune response to primary infection with the Epstein-Barr virus (EBV) (i.e., infectious mononucleosis), and subsequently develop fatal lymphoproliferative disorders of the B-cell type. This was in contrast to a second patient, also a young male, who did not have the X-linked lymphoproliferative syndrome, who did develop specific antibodies to the Epstein-Barr virus and whose malignant lymphoma was closely associated in time (i.e., 5 weeks) with the clinical diagnosis of infectious mononucleosis. The comparative immunologic and virologic features are discussed as well as the importance of careful clinicopathologic correlation in young adults and children developing malignant lymphoma both following and in association with infectious mononucleosis.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

Morphological diagnostic criteria for the various “histiocytoses” must be reevaluated in the context of new concepts of lymphology and the characteristics of a unique histiocyte, the Langerhans' cell. Indeed, many diseases categorized as histiocytoses are now known to be proliferative disorders featuring the lymphocyte with a histiocytoid appearance. The term lymphoreticulosis is preferred in reference to the various disorders featuring proliferation of lymphoid and reticuloendothelial cells considered in this paper.There are numerous reactive syndromes resulting in lymphoreticulosis. Careful evaluation of the patient is required for the identification of etiological agents and therapy should be directed in light of etiology.Patients with deficiency of defense systems may present with lymphoreticulosis, the signs and symptoms of which may overshadow the primary host defense deficiency state.A group of diseases featuring primary proliferation of Langerhans' cells, unique histiocytes with characteristic ultrastructural morphology, is the major subject of this seminar and encompasses the histiocytosis × spectrum of disease (eosinophilic granuloma, multifocal, and systemic disease).Disorders within this category, mainly the multifocal and systemic diseases, pose serious problems in terms of variation of clinical course and prognostic indicators which make clinical management difficult.An entity not to be confused with primary Langerhans' cell proliferative disorder is a highly lethal and most often familial disease designated lymphohistiocytic reticulosis with phagocytosis. Specific diagnostic criteria are posed and include bone marrow, cerebrospinal fluid, and hepatic morphological changes.Other lymphoreticuloses not readily classifiable are described briefly.It is suggested that the rarity and complexity of these disorders demand that children manifesting signs and symptoms of the lymphoreticuloses be promptly referred to a sophisticated pediatric hematology-oncology center for proper patient care and productive investigation.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

It is now clear that the prognosis in children with histiocytosis × has improved considerably over the past few years. To be sure, patients with solitary lesions have an excellent prognosis. Whereas the outlook for patients with significant visceral involvement is not as good as those with bone lesions only, the outlook is by no means hopeless, as was once thought.A number of prognostic factors have been reviewed here. The most significant of these factors at the present time would appear to be age of onset of the disease, extent of involvement, the rapidity of progression of the disease, and, in particular, the presence or absence of dysfunction of such crucial organ systems as liver, lung, and hemopoietic system. Further studies of the significance of histologic features and immunologic findings are clearly needed to further our understanding of this disorder.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

The most commonly reported familial variant of histiocytosis is familial hemophagocytic lympho-histiocytosis. Clinically, this may be distinguished from other infantile forms by the absence of skin involvement and high incidence of leptomeningeal involvement.Eosinophilia, erythematous skin rash, alopecia, and opportunistic pulmonary infections suggest combined immunodeficiency with histiocytic response.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID