ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

We report two children with leukemia who were born to diethylstilbestrol (DES)-exposed mothers. The children were 2 and 6 years old at diagnosis and the lymphoblasts expressed the unusual terminal deoxynucleotidyl transferase negative phenotype. Both children completed induction and maintenance therapy and are alive 6 and 4 years from diagnosis. The role of DES as a transplacental carcinogen is reviewed, and a hypothesis for the target cell interaction is proposed.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Leukemic blasts from patients with acute lymphoblastic leukemia, (ALL) were tested initially for the following surface markers: sheep erythrocyte receptors at 4°C and 37°C; receptor for the C3 fraction of complement; mouse erythrocyte receptor; and surface immunoglobulins. We found that 73% of the ALL were devoid of these markers, 9% had C3 receptor only, 2% were B-ALL, and 15% were T-ALL. Only one of 147 cases tested expressed receptors for mouse erythrocyte receptor. C3-ALL predominated in girls and the prognosis was similar to that of the ALL devoid of markers. T-ALL was associated with some high-risk factors, such as high initial leukocyte counts, and a predominance in boys older than 5 years of age. Further studies were done in which cytoplasmic immunoglobulins were evaluated and surface antigens were identified by the following monoclonal antibodies: OKT1, OKT3, OKT4, OKT6, OKT8, OKT9, OKT10, OKT11a, OKIa1, and J5 (anti-CALLA). Less than 1% were B-ALL, 15% were T-ALL, and approximately 85% were non-T, non-B ALL. Of the latter group, 69% were common-ALL, 16% were pre-B ALL, and 15% were null-ALL. Within the T-ALL, the expression of the T antigenic mosaic was heterogeneous. Results by a multivariate analysis demonstrated that sex, age, initial leukocyte count, and T-cell phenotype were independent variables with a negative prognostic value (p < 0.01), and the only combination with significant interaction was between T-cell phenotype and leukocyte counts.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Wedge biopsy of the testis was performed in 46 children who had received long-term chemotherapy for acute lymphoblastic leukemia. Occult testicular infiltration was noted in three children (6.5%). Two of three children with biopsy-proven infiltration died of systemic disease in spite of local irradiation and reinduction chemotherapy. Six of 43 children shown to be negative by testicular biopsy relapsed 11 months to 15 years later, and 3 of 6 patients died of systemic disease, but none of the cases developed testicular disease. Chemotherapy-induced gonadal damage was observed in 30 of 46 children, and tubular damage was occasionally still seen 4 years after cessation of treatment. Although gonadal damage usually depends on the cumulative dosage of cyclophosphamide, intact tubular fertility index was found in several children who had received a greater dose of cyclophosphamide intermittently. Induction and maintenance chemotherapy for acute lymphoblastic leukemia had little influence on hormonal function. Testicular biopsy at the time of cessation of chemotherapy seems to be worthwhile for the subsequent strategy of treatment, and long-term surveillance for gonadal damage of long-term survivors will be required.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

To confirm an impression that many survivors of acute lymphoblastic leukemia (ALL) are overweight or obese, we retrospectively examined the medical records of 414 patients for height and weight at diagnosis, at completion of treatment, and at annual intervals thereafter. The body mass index, weight/height2, was used as a measure of fatness; population norms for the index were established from 9,003 people between the ages of 1 and 30 years who were examined in a national health survey. The percentile of each patient's index was determined at each observation date. At diagnosis, the study sample was skewed toward leanness; however, at cessation of therapy, the fatness distribution resembled population norms. Statistically significant increases in fatness occurred during the first year off therapy, at the end of which 35% of the children were above the 80th percentile (i.e., overweight) and 12% were above the 95th percentile (i.e., obese). Only 12% were below the 20th percentile. This skewed distribution persisted during the subsequent 4-year follow-up period. Cranial irradiation was associated with a large increase of fatness in one group available for comparisons. Our findings indicate that the first year following cessation of therapy is a time of excessive weight gain among pediatric ALL patients.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Ten children, four males and six females, with malignant histiocytosis were treated from July 1980 to July 1984. None of them had an affected sibling with a similar disorder. Septic-type fever, hepatosplenomegaly, lymphadenopathy, pulmonary infiltration, and disseminated intravascular coagulation were common signs and symptoms, and convulsion occurred in four cases. The diagnosis was made from bone marrow smears in all cases. In five cases, biopsy or autopsy specimens confirmed the diagnosis. In five cases studied, proliferating histiocytes in lymph nodes were demonstrated to be S100 protein-positive. All patients were treated with adriamycin, cyclophosphamide, vincristine, and prednisone (ACOP). Complete response was achieved in four patients after two to three courses of ACOP, and another case attained complete remission after further drug treatment. The five complete responders are now alive without evidence of disease after 23–48 months from the onset. Among partial and no responders, four died within 3 months and one has been alive with disease for 2 months. Bone marrow aspiration is useful for prompt diagnosis, and early treatment with intensive combination chemotherapy improves the prognosis of malignant histiocytosis in childhood.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

The authors describe a 6 1/2-month-old boy with a large hemangioma involving the left buttock associated with a severe consumptive coagulopathy. Cosmetic improvement and resolution of the coagulation defect followed occlusion of the nutrient vessels to the hemangioma with polyvinyl alcohol. This is the first report of successful treatment of this condition with therapeutic embolization.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Central nervous system hemorrhage is a well-recognized complication of neonatal alloimmune thrombocytopenia attributed to perinatal trauma from passage through the birth canal. That central nervous system (CNS) hemorrhage can occur in utero is not as well recognized, and congenital CNS lesions have only circumstantially been linked to thrombocytopenia. We report two cases of intrauterine CNS hemorrhage shown to have occurred prenatally, resulting in porencephaly. The second case is unique in that the necropsy finding of a porencephalic cyst arising from an old hemorrhagic site pathologically confirms that the etiology of congenital CNS lesions in alloimmune thrombocytopenia is due to hemorrhage. This second case received close prenatal monitoring and yet died as a result of hemorrhage that was not detected until emergency operative delivery. A review of the literature revealed 10 cases, including two other pairs of siblings, who had CNS damage attributable to intrauterine hemorrhage. These findings indicate that congenital CNS lesions in alloimmune thrombocytopenia are due to intrauterine hemorrhage that careful obstetric and prenatal care may not identify or prevent. This fact should be included when genetic counseling is offered to alloimmunized mothers.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

The risk of significant bleeding in patients with severe Factor VII (FVII) deficiency is well documented. However, controversy regarding management exists not only for the choice of FVII replacement product, but also for the desired hemostatic level of FVII. Two case reports are presented where fresh frozen plasma (FFP) and several Factor IX (FIX) complex concentrates were used to achieve hemostasis. Significantly higher FVII levels were achieved with Proplex (FIX Complex, Hyland Therapeutics) than with other products. Although this and other reports indicate that minor bleeding can be controlled with levels of FVII under 15%, life-threatening hemorrhages require higher FVII levels that can be attained only by using a product with a significant FVII content. It is important to know the FVII content of FIX concentrates in order to plan appropriate therapy for patients with FVII deficiency.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

A 4-year-old Japanese girl had a congenital disorder that was characterized by recurrent thrombocytopenia, hemolytic anemia, hematuria, and proteinuria, which were repeatedly improved by the infusion of factor VIII concentrate. She developed the similar symptoms within 1 h after 1-desamino-8-D-arginine vasopressin (DDAVP) administration. Coagulation studies 30 and 60 min after DDAVP infusion showed a disappearance of large factor VIII:von Willebrand factor (VIII:vWF) multimers, which was the same abnormality that was observed at acute episodes. There were no significant changes in the plasma levels of 6-keto-prostaglandin F1α and thromboxane B2 before and after DDAVP infusion. These results provide further support that VIII:vWF is directly involved in the pathogenesis of this congenital disorder.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID