Departamento de Innovación del Hospital Sant Joan de Déu / Department of Innovation of the Hospital Sant Joan de Déu

No disponible

[An increase in gamma-delta T-lymphocytes in the peripheral blood of cystic fibrosis patients]. / Incremento de linfocitos T gamma-delta en sangre periférica de pacientes afectos de fibrosis quística.

The function of the T gamma-delta cells of the human immune system is not well known at present. Only 3-10% of the T cells express the heterodimer composed of the gamma-delta chains. Recent studies have demonstrated a role of the T gamma-delta cells in the immunopathogenesis of autoimmune and infectious diseases. The present study was designed to evaluate the quantity of T gamma-delta cells in patients with cystic fibrosis with P. aeruginosa infections. These results were compared to blood levels of T cells found in patients with acute pulmonary infections, chronic pulmonary infections and healthy control patients. The cellular phenotype was determined by flow cytometry. Monoclonal antibodies against the different cell types studied were employed. The means of each group were compared by a Student's T test of Mann Whitney. We found that the percentage of T gamma-delta cells (TCR 1+) was significantly increased in patients with cystic fibrosis when compared to the pathological controls and healthy children. We conclude that our results demonstrate that children with cystic fibrosis infected with Pseudomonas aeruginosa demonstrate and increase in the subclass of T cells with the gamma-delta receptor.

Neonatal mixed lineage acute leukaemia.

We report here an uncommon case of neonatal acute leukaemia that presented concomitant with serological evidence of rubella infection. The clinical course was aggressive and the patient died 5 days after diagnosis from septicaemia. Leukaemic blasts had a mixed lineage immunophenotype co-expressing a constellation of B-lymphoid (CD19, cytCD22, TdT) and myeloid (CD13, CD33, CD14, anti-MPO) markers, as well as multiple adhesion molecules and markers associated with early lympho-myeloid progenitor cells (CD34, CD7, HLA-DR). A previously unrecorded discordant expression of different CD10 and CD34 epitopes was identified using different monoclonal antibodies. The karyotype was 46,XX t(4;11)(q21;q23) and molecular analysis confirmed rearrangement of the trithorax-related oncogene HRX at 11q23. There was a clonal biallelic rearrangement of the immunoglobulin heavy-chain gene. The features of this rare case have implications for possible aetiological events leading to leukaemia.

[A little known entity: aggressive fibromatosis]. / Una entidad poco estudiada: la fibromatosis agresiva.

Aggressive fibromatosis is an unfrequent and little known entity, which in spite of being a histologically benign tumoration with scarce mitosis and without metastasis at distance, frequently presents with a high degree of local malignancy that can cause serious functional and aesthetical disturbance for the patient and even lead to death if infiltration of vital organs is presented, above all in cases of abdominal or maxillo-facial mass localization. The authors present their experience with 17 cases of aggressive fibromatosis observed in our centre: four of abdominal localization, six in extremities, five in the maxillo-facial mass, one in the torax and one in the lumbo-sacral region. Histological diagnosis, either by puncture or biopsy, is complemented by studies of extension of the tumour based on ecography and TAC. All cases were treated according to the classical criteria of ample resection of the lesion, always when practicable, except in one infant case and in the torax, in which only a biopsy was effected. Of the 15 cases resected, nine cases had local relapses, six of which remained free of disease with a second operation, another two required a third operation and the remaining case needed five interventions. In six children chemotherapy was applied with vincristina, cyclophosphamide and adriamicina. A follow up was carried out in 14 patients, one of which died and the remaining 13 are free of disease. In spite of the fact that progestagene receptors were not evidenced in two of our cases, one presented complete remission of the tumor after treatment with medroxyprogesterone. In this case the coincidence of Gardner's syndrome arises in the family history.(ABSTRACT TRUNCATED AT 250 WORDS)

[Non-Hodgkins lymphoma in childhood, preliminary results of the LSA2 L2 protocol modified by high-dose methotrexate]. / Linfoma no Hodgkin en la infancia, resultados preliminares del protocolo LSA2 L2 modificado con metotrexate a altas dosis.

From january 1982 to january 1986 we treated 23 pediatric patients who had non Hodgkin's lymphoma, with a new therapeutic protocol. This protocol is based on the LSA2 L2, modified during the induction phase with high-dose methotrexate and increasing of intrathecal therapy. Nineteen patients (83%) were in stages III and IV and the 47.4% were in stage IV. The distribution according to histology was: 10 Burkitt's undifferentiated lymphomas; 2 non-Burkitt's undifferentiated lymphomas; 5 lymphoblastic lymphomas convoluted-cells; 5 lymphoblastic lymphoma non-convoluted cells and 1 indeterminate lymphoma. The follow-up oscillated between 6 months and 4 years. The total survival rate is 82.6% with a 100% survival in stages I, II and III and 63.5% in stage IV. The survival according histology is 91.7% for undifferentiated lymphomas and 70% for lymphoblastic lymphomas; the actuarial survival rates in the two groups at 30 months are 87% and 55% respectively (p greater than 0.10). We concluded that adding high-dose methotrexate to the LSA2 L2 protocol improve the survival rate in undifferentiated lymphomas and in a less extent in lymphoblastic lymphomas, without significant toxicity. This new protocol is mainly indicated in undifferentiated and indeterminate non-Hodgkin's lymphomas.

[Neuroblastoma IV-S. A series of 7 cases]. / Neuroblastomas IV-S. Serie de siete casos.

A review of the findings in seven cases of stage IV-S neuroblastoma, that have been observed between 1966 and 1984. Patients under one year stage IV-S neuroblastoma have a favorable prognosis; survival rate was 71%. Primary tumor in some may be relatively small. Chemotherapy and radiation therapy may not be necessary in the management of certain children.