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Biomedical subjects

R J Haas

Publications and source records attributed to R J Haas.

At least 55 records · Page 3Linked to original sources
PubMed1983

Therapy of acute lymphocytic leukemia in childhood with intermediate dose methotrexate and CNS irradiation. A report of the ALL 77-02 study group.

One hundred and eight children with acute lymphocytic leukemia (ALL) were admitted to a prospective therapeutic regime. Remission induction was achieved in 94% of the cases with vincristine, L-asparaginase, adriamycine and prednisone. One hundred and one patients received three intermediate dose methotrexate (MTX) infusions combined with intrathecal MTX, followed by L-asparaginase 24 h later. High risk (HR) patients (n = 50) were treated in addition with high dose cyclophosphamide and Ara-C over 3 weeks. One hundred and one patients received cranial irradiation (1,800 rads standard risk (SR)-patients, 2,400 rads HR-patients) and intrathecal MTX. Maintenance therapy was performed with the usual two drug combination of daily 6 mercaptopurine (6 MP) and weekly MTX orally. Based on phenotyping 67% of patients had common type ALL, and pre-T or T-cell type in 18%. Six per cent of the patients had leukemic blasts expressing both common ALL and T-cell markers (c/T-type); 9% had acute undifferentiated leukemia (AUL). Out of 108, 101 achieved a complete remission, 6 patients died during induction therapy, 1 was a non-responder and 9 patients relapsed. Of these Four patients died in continuous complete remission (CCR). For 101 patients the 30 months probability of CCR is 0.85 (+/- 0.05). For 51 patients with standard risk CCR probability is 0.98 (+/- 0.03), for 50 patients with high risk indices it is 0.65 (+/- 0.11). Patients with c-ALL have a CCR survival of 0.85 (+/- 0.07), those with T- or pre-T-ALL 0.88 (+/- 0.09), all 5 patients with c/T-ALL alive in CCR. In our study pediatric AUL patients have the most unfavourable prognosis.

Acute Disease↗
PubMed1982

[Embryonal rhabdomyosarcoma with infiltration of the orbit and the paranasal sinuses (author's transl)].

The authors report on a case of an orbital embryonal rhabdomyosarcoma in a 12-year-old boy. Treatment consisted of radiotherapy, chemotherapy, and local excision of the tumor, the excision, however, without any mutilating surgery. Altogether, seven children have been treated in the same way at the pediatric clinic of the University of Munich. All seven are quite well and none of them show any signs of recidivism. The longest follow-up observation period is now four years.

Child↗
PubMed1982

[So-called histiocytosis X and malignant histiocytosis].

A retrospective study of 12 cases of so called histiocytosis-X and 3 cases of malignant histiocytosis was done. It was possible to establish the differences in clinical, morphologic and cytochemical findings of both diseases. The diagnosis of histiocytosis-X can be confirmed by multinucleated histiocytes interrupted by eosinophils and plasmacells. The histopathology of malignant histiocytosis is different and is characterized by atypical histiocytes. Erythrophagocytosis throughout the tissues is seen. Typical histochemistry (acid phosphatase and naphtol-AS-acetat-esterase) findings are also helpful for diagnosis. The treatment of both diseases should be continued at least six months after disappearance of clinical apparent lesions. Combination chemotherapy with vinblastine and prednisone is suggested. In cases of histiocytosis-X in isolated lesions curettage or irradiation may be adequate. Long term remissions and presumed cures of histiocytosis-X are possible in over 70% of the cases. A strict correlation between the prognosis and the degree of involvement is confirmed. Even in cases of malignant histiocytosis, previously reported as rapidly fatal disease, combination chemotherapy may produce complete long term remissions.

Adolescent↗
PubMed1982

[Improved prognosis in Wilms' tumor due to adjuvant combination drug therapy].

78 children with Wilms' tumor stage I--IV diagnosed since 1960 are presented. There were two groups of patients: one group consisting of 35 patients which received adjuvant chemotherapy for 1 year, the other group consisting of 43 patients which received no chemotherapy. Surgical excision and irradiation was identical in both groups. The prognosis was greatly improved by adjuvant chemotherapy: 4-year survival rates increased from 25 to 69%. Late side effects from radiation and chemotherapy were noted: 15/78 patients suffered from scoliosis and all patients treated by chemotherapy had a decreased lymphotoxin activity over years. The following factors appear to be related to prognosis: the extent of disease in patients determined by better techniques, histopathology of the tumor, and the nature of treatment.

Age Factors↗
PubMed1982

Craniofrontonasal dysplasia in a three-generation kindred.

Frontonasal dysplasia, a nonspecific defect in a developmental field complex, and craniosynostosis usually occur as isolated sporadic events. Cohen recently described a syndrome that includes both of these defects, which he called "craniofrontonasal dysplasia." We report a three-generation family in which five individuals (four females, one male) have varying degrees of frontonasal dysplasia and craniosynostosis. The mode of inheritance is unclear and possible explanations include autosomal dominant with sex-influenced expression, X-linked dominant, and metabolic interference. This family and others reported in the literature represent a subpopulation of patients with frontonasal dysplasia who are at high risk for recurrence.

Adult↗
PubMed1981

[Bone marrow transplantation for aplastic anaemia (author's transl)].

From March 1975 until May 1980 twelve patients with severe aplastic anemia were grafted with bone marrow from HLA-identical siblings by the Munich Cooperative Group for Bone Marrow Transplantation. Six patients are alive between 10 months and more than 5 years after grafting with normal blood values and marrow. One patient is treated as an out patient for chronic localized graft-versus-host disease (GvHD), five patients are well and without treatment. Six patients have died, one patient with a cerebral hemorrhage the day before transplantation, three patients following rejection of grafts 32, 40 and 55 days after grafting, one patient with severe GvHD 85 days after grafting and one patient, probably with interstitial pneumonia, following cerebral hemorrhage. Three of 6 patients who were conditioned with Cyclophosphamide (CY) only died following rejection of the graft. Two adults who were conditioned with CY and "total lymphoid irradiation" and three children, who wer given unirradiated leukocyte concentrates from the marrow donor after grafting, did not reject their grafts. The results of the Munich-Cooperative Group for Bone Marrow Transplantation are comparable to those of large, specialized centers for bone marrow transplantation, they indicate possibilities of cure of severe aplastic anemia by marrow grafts from HLA-identical siblings. They confirm that better results are obtained with earlier transplantation in the course of the disease.

Adolescent↗
PubMed1981

Sinus histiocytosis with massive lymphadenopathy and epidural involvement.

Sinus histiocytosis with massive lymphadenopathy (SHML) was recognized as a new clinical-pathological entity in 1969. Up to the present 134 cases have been described. The disease is characterized by prominent cervical lymph node enlargment. Microscopic features include marked dilatation of sinuses with intrasinusal histiocytes and lymphophagocytosis. About 70% of the patients reported were affected during the first 2 decades of life. The disease is held to be benign on account of spontaneous resolution in some patients. A follow-up survey of 72 patients showed disappearance of the symptoms 10 years after the original diagnosis in 24 patients. In 42 patients the disease still persisted 6 months to 21 years later. Six patients died, but only one of them as a result of the disease. Extranodal involvement was seen in the orbit, eyelid, respiratory tract, skin, bone, salivary glands, and testis. In two cases, one of which will be reported here, paraparesis resulted from infiltration of the epidural space. Treatment with prednisolone was tried in some cases with excellent results. In our case treatment with prednisolone and vinblastine resulted in the disappearance of the neurological symptoms.

Adolescent↗
PubMed1981

Familial lymphohistiocytosis.

Familial lymphohistiocytosis is a genetically transmitted disease affecting infants and very young children with usually a fatal outcome. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Histologic examination shows infiltration of all organs with phagocytosing histiocytes and lymphocytes as well as atrophy of the normal lymphoid tissue. The distinction from other histiocytic disorders, i.e., Letterer-Siwe disease or malignant histiocytosis, may be difficult. However, the familial occurrence and characteristic findings in the coagulation system and lipid pattern make familial lymphohistiocytosis a sufficiently distinct clinical entity. This report review 79 cases fron the literature and adds four of own observations.

Blood Coagulation↗
PubMed1981

[Bone marrow transplantation in acute leukemias: antibody treatment for suppression of graft-versus-host disease (author's transl)].

Anti-human-thymocyte globulin (AHTZG) was applied to prevent GvHD in clinical bone marrow transplantation. AHTZG produced by absorption with several cell preparations reacted specifically with T-lymphocyte populations and was no longer inhibitory to human CFUc and bone marrow growth in diffusion chambers. Marrow grafts of 14 patients with ALL were incubated in vitro with AHTZG and transferred to the recipients conditioned with antileukemic chemotherapy and total body irradiation of 1000 rad. Ten patients were transplanted after relapse, four patients during remission. The patients tolerated the marrow without side effects and a hemopoietic engraftment was seen in 12 cases. Three patients showed signs of GvHD on the skin, two of them showed later on also manifestations in the liver. In the other cases no GvHD could be detected. Five out of 14 patients are still alive between 144 and 964 days post transplantation in remission.

Acute Disease↗
PubMed1981

Intravenous and subcutaneous desferrioxamine therapy in children with severe iron overload.

Ten children with transfusion dependent anemias (thalassemia, sideroblastic anemia, congenital pure red cell aplasia) received either intravenous desferrioxamine (DF) in increasing doses up to 450 mg/kg at the time of transfusion or daily subcutaneous DF up to 110 mg/kg on an outpatient basis. No patient on intravenous DF reached a negative iron balance. All children with a subcutaneous DF dose of more than 60 mg/kg obtained a negative iron balance with a net iron excretion (transfusion iron already substracted) between 206 to 810 mg (mean 496 mg) monthly. The effectiveness of regular subcutaneous DF on liver storage iron could be confirmed in 4 patients by liver biopsy, showing a decrease between 40-60% iron after 12-14 months of chelation therapy. So far the daily iron excretion has remained constant with a given dose of DF over a period up to 15 months. Even if poor compliance in some patients is taken into account, it is possible with this method of treatment to prevent further accumulation of iron in chronically transfused children.

Adolescent↗
PubMed1981

[Psychic development of children under conditions of longterm isolation (author's transl)].

Three children with severe combined immunodeficiency have been isolated for 19-22 months. Two of the children have been placed into the isolation system directly after delivery; on child entered the isolation system at the age of 5 months. The influence of this situation on the psychic development of the children was studied by the means of direct observation as well as by psychometric test methods. An additional child which has been isolated between the age of 6 and 32 months was retested at the age of 4 and 6 years. The reported observation which include those of the parents and the team allow the conclusion, that a normal development is possible under these extreme conditions, when an optimal clinical and psychological support is maintained.

Child Development↗