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Biomedical subjects

R J Haas

Publications and source records attributed to R J Haas.

At least 91 records · Page 5Linked to original sources

[Neuroblastoma in children. Clinical staging and management (author's transl)].

Sixty-four children with neuroblastoma stage I to III c are presented. The coordinated management utilizing surgical excision, irradiation (2--5.000 rad) in stage II and III and multiagent chemotherapy is described. Favorable sites were abdomen (30) and thorax (22). In ten cases the primary site was unknown. The prognosis is influenced by several factors: Patients under 1 year of age without evidence of bone or bone marrow metastases have a favorable outcome (13/15). Nearly all patients with lesions of bone or generalized tumor in bone and/or bone marrow (stage III b and III c) failed to attain long term disease free survival despite combination chemotherapy and the use of radiation therapy (33/34). No relation between histological or biochemical characteristics and prognosis could be found. Biochemical determinations however are useful as an index of response to treatment. Prognosis was independent from sex. A review of the literature and on attempt to improve the therapeutic efficancy in stage III are reported.

Abdominal Neoplasms↗

Effects of CCNU on hematopoiesis in rats.

The effect of orally administered CCNU on proliferating and cytokinetically "resting" bone marrow cells was studied in rats. The resting bone marrow cells were selectively labeled with 3H-thymidine by the "complete 3H-thymidine labeling method." The results show a reduction in the number of absolute bone marrow cells to about 40% of the original value in 3 days. Regeneration was completed only after 22 days. No difference was found in the destructive or the regenerative pattern of CFU-C, myelopoietic, erythropoietic, lymphatic, or megakaryocytic cells. Resting and proliferating cells of the bone marrow were equally affected by CCNU.

Animals↗

[Non-Hodgkin's lymphoma in children: improved prognosis through aggressive multiple drug combination and irradiation (author's transl)].

From 1964-1975 43 children with non-Hodgkin's lymphoma (NHL) were treated. 60% of the patients had far advanced disease at diagnosis. Therapy before 1970 consisted of low dose irradiation to the primary and single agent chemotherapy; no C.N.S. irradiation to prevent meningeal recurrence was given. Median survival in this group was 5 months; all patients died. Since 1970 all children with NHL were entered into a modified leukaemia protocol regardless of stage or primary site. Therapy comprised an aggressive multiple drug combination, high dose local irradiation and prophylactic C.N.S. irradiation with intrathecal methotrexate. 41% of the patients treated since 1970 survive in continuous complete remission with a median observation time of 31+ (1-93+) months. All relapses occurred within 30 months after diagnosis. Stage of disease was the most important prognostic factor in our patients. Risk of a primary C.N.S. relapse in the total group was 30% for patients without prophylactic C.N.S. therapy compared to only 6% for patients with treatment.

Adolescent↗

Lymphoblast cell size and prognosis in acute lymphoblastic leukemia in childhood.

The significance of cell size as a prognostic indicator in acute lymphoblastic leukemia (ALL) is controversial. Accuracy in measurement of cell size can be improved by determination of cell areas instead of single cell diameters. In the present study cell areas of 200 cells were determined in pretreatment bone marrows of 35 children with ALL. For better comparison with other studies which had used cell diameters only, the measured area was expressed as circle area from which the circle diameter was calculated. Cells with a diameter of greater than 12 micron were defined as macrolymphoblasts (MLB). Several clinical characteristics considered to be risk factors in ALL were ascertained for each patient. The duration of first complete remission was used to assess the prognostic significance of cell size and of number of risk factors. In contrast to previous reports patients with more than 25% MLB had longer remissions. However, nearly all patients of this group had no or one risk factor only. When patients with more than one risk factor were excluded from statistical analysis, the group with more than 25% MLB had no longer a better prognosis compared to the group with 25% MLB or less. Thus, in this study the percentage of MLB was not an independent prognostic indicator for risk of relapse in ALL.

Age Factors↗

[ALL-Associated antigen: occurrence on normal blood cells, cell-lines and leukaemic cells (author's transl)].

The specificity of an antiserum against ALL-cells lacking B- and T-cell markers was characterized. The antiserum was used in several indicator systems to investigate the expression of leukaemia-associated antigens on normal lymphocytes, lymphoid cell lines and various leukaemia cells. The relevance of such antisera for the detection and classification of leukaemic cell populations is discussed.

Antigens, Neoplasm↗

Combination chemotherapy and radiotherapy for Hodgkin's disease in children.

31 children with Hodgkin's disease were treated at the Children's Hospital, University of Munich, between 1963-1976. Before 1971 the diagnosis of abdominal involvement was based solely on physical findings and radiological studies. Treatment consisted of involved-field radiation; chemotherapy was added in selected cases. Of 15 children treated between 1963-1971, 5 are still in their first remission and 2 in their second remission. Since 1971 exploratory laparotomy and splenectomy have been performed. Radiation therapy was changed from involved- to extended-field therapy and combination chemotherapy was added in all the children. No patient from this group of 16 children relapsed during a median observation time of 24 months (range 12-55 months). The toxicity of the combined treatment does not seem to be prohibitive.

Adolescent↗

Sinus histiocytosis with massive lymphadenopathy and paraparesis: remission with chemotherapy. A case report.

A case of sinus histiocytosis with massive lymphadenopathy (SHML) in which cranial nerve function was impaired is described. Severe paraparesis due to an epidural block at the C2 and C5-T2 levels was also present. Treatment with cytotoxic agents resulted in a dramatic decrease in the size of the involved lymph nodes and disappearance of neurological symptomatology. A review of the literature revealed that, although the disease is benign in its clinical course, the process is not restricted to the lymph nodes. The case reported here is the second case in which the epidural space was affected.

Adolescent↗

[Premonitory blood count changes in the relapse of juvenile acute lymphatic leukemia].

Investigation was done in 80 children with acute lymphocytic leukemia (ALL) with respect to premonitory changes of peripheral blood cell counts before a hematologica relapse. 38 patients suffered from one or more relapses. Evaluation was done by t-test and Friedmann test. 9 weeks before a relapse there was a significant decrease in thrombocytes numbers demonstrable. Granulocytes numbers and monocytes numbers declined 6 and 3 weeks before the relapse, respectively. The lymphocytes counts had already increased 6 weeks before a relapse. The blood cell counts during intercurrent infections did not interfere with the typical pattern of blood cell counts before a relapse. Also the changes in blood cells found are different from those seen in untreated cases of ALL.

Blood Platelets↗

[Juvenile rhabdomyosarcoma. Diagnosis and new therapeutic possibilities].

Seventeen children with rhabdomyosarcoma stage I to III diagnosed since 1973 are presented. The coordinated management utilizing surgical excision, irradiation (5000 rad) and systemic adjuvant multiagent chemotherapy is described. The favorable sites were head and neck sites and genitourinary region. The mean survival is at present 19 months. 14 children are alive and well. 13 children had no tumor recidivation, 1 child had 8 months after beginning of the therapy lung metastases which after irradiation with 2000 rad disappeared. 3 children (stage III) died 9, 14 and 20 months after diagnosis during therapy by metastases. Acute and late effects on normal tissues from radiation and chemotherapy were noted in 12 cases. A review of the literature and therapeutic alternatives in the future are indicated.

Child↗

[Diagnostik significance of cell membrane markers in childhood ALL (author's transl)].

Acute lymphoblastic leukemia (ALL) in childhood is not a homogeneous disease. By membrane phenotyping 6 groups of ALL in 66 children could be differentiated: Group I (38%) common ALL (anti-cALL+. Group II (32%) common ALL with T cell marker in addition (anti-cALL+, anti-T+). Group III (14%) T. cell ALL with E-rosette marker (anti T+, E+). Group IV (9%) T cell ALL without E-rosette marker (anti T+, E-). Group V (6%) undifferentiated ALL (anti-cALL-, anti-T). Group VI (1%) B cell typ (SmIg+). 19 or 20% of children in groups I and II opposed to 67 or 100% of children in groups III and IV had a white cell count at diagnosis of greater than 25,0 x 10(3)/mm(3) Mediastinal involvement was typical for groups III and IV. No significant difference was found so far for age or sex in the groups.

Age Factors↗

Congenital immunodeficiency and agranulocytosis (reticular dysgenesia).

A patient is presented who manifested the typical clinical and pathological features of congenital immunodeficiency and agranulocytosis (reticular dysgenesia). Treatment under gnotobiotic conditions enabled the measurement of immunogical parameters up to the 17th week of life with the following results: negative skin test, low response to phytohaemagglutinin, weak response in the mixed leukocyte culture and very few E rosettes. Peripheral lymphocytes and lymphocytes in the lymphatic tissues were markedly decreased. Humoral immunoglobulins and plasma cells in the organs were decreased. The in vitro culture of hemopoietic cells showed a diminished content of myelopoietic progenitor cells ("committed stem cells"). It is concluded that the disease may be primarily a defect of stem cells with regard to differentiation in myelopoiesis or lymphopoiesis.

Agranulocytosis↗

Specific absorbed antithymocyte globulin for incubation treatment in human marrow transplantation.

The experimental data show that absorption of ATG with liver-kidney homogenate and CLL and LCL cells stepwise removed the hemopoietic toxicity, whereas the specific activity against T lymphocytes remained. Although the mode of action of absorbed ATG could not be tested in the first clinical case, the successful experiments in rodents together with the fact that the incubation treatment was tolerated by the patient may provide a new way of preventing fatal GVH reactions in man.

Animals↗

[Autoimmune hemolytic anaemia in childhood. Review and report of one case (author's transl)].

Autoimmune hemolytic anaemia (AIHA) in childhood is associated with antibodies produced by the patient himself, which coat his red cells causing their hemolysis. Although in some cases no underlying disease could be found, in the majority of children a virus etiology is apparent. There are very few reports regarding the use of treatment with immunsuppressive agents and the possible benefit. The article reports one case of AIHA. The patient developed at age 6 months a prolonged chronic form of AIHA complicated by thrombopenic purpura. The recent knowledge about pathogenesis, clinical phenomena, serology and prognosis is discussed. Treatment with corticosteroids, azathioprine or cyclophosphamide failed to benefit. Splenectomy and 6-mercaptopurin therapy (3 months) resulted in a complete remission.

Anemia, Hemolytic, Autoimmune↗

Experimental analysis of developing hemopoiesis in fetal bone marrow.

1. We have used fetal rats to study the following aspects of the development of hemopoiesis: (a) content of hemopoietic stem cells in fetal bone marrow, liver, and peripheral blood and (b) origin of hemopietic cells in the developing mammalian bone marrow. 2. In the studies we utilized the diffusion chamber technique to study the content of stem cells committed to granulopoiesis. The number of myelopoietic stem cells in liver peripheral blood and in "bone marrow" of 18-day-old rats is nearly identical. Since in "bone marrow" a considerable number of peripheral blood cells are present in the vessels at that time, whereas extravascular cells consist only of mesenchymal cells, one might assume that these peripheral blood cells give rise to granulocytic precursors in the cultures. Morphologically these cells are "blast" cells and lymphocytes. 3. Based on cell labeling indices of radioautograms, derived from continuous infusion of pregnant rats with [3H]thymidine, it could be shown that in the perichondral area mesenchymal cells of the fetus and newborn have a slow rate of DNA turnover whereas "bone marrow" cells are in an active state of profileration. 4. In further support of this it was also shown that injections of hydroxyurea (an agent which destroys cells in DNA synthesis) has vitrually no effect on perichondral mesenchymal cells whereas "bone marrow" cells were completely blocked in their ability to support myelopoietic differentiation in the diffusion chamber implants. 5. The conclusions would therefore be that (a) local perichondral cells, i.e., mesenchymal cells, do not contribute to marrow hemopoiesis, (b) matrix cells of the developing bone marrow cannot reconstitute hemopoiesis, and (c) hemopoietic bone marrow cells develop from migrating peripheral "stem cells", one of the sources being the liver.

Animals↗