(1989). Familial Hemophagocytic Lymphohistiocytosis: Diagnostic Problems and Differential Diagnosis. Pediatric Hematology and Oncology: Vol. 6, No. 3, pp. 219-225.
(1989). Natural Killer Cell Function and Interferon Production in Familial Hemophagocyticlymphohistiocytosis. Pediatric Hematology and Oncology: Vol. 6, No. 3, pp. 265-272.
(1989). Familial Hemophagocytic Lymphohistiocytosis: Therapy in the German Experience. Pediatric Hematology and Oncology: Vol. 6, No. 3, pp. 227-231.
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.
Cell-kinetic investigations were carried out in a 15-year-old girl, initially presenting bicytopenia and changing gradually to cALL. At three instances during this transitional stage of the disease, the proliferation kinetics were studied in the cALL blast cell population as well as in erythroblasts and myelocytes. The percentage of blasts in the bone-marrow was 39, 42 and 70%, respectively. The DNA synthesis time in individual cells was measured by using the technique of quantitative 14C-autoradiography.
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.
From 1963–1974, 62 cases of malignant lymphoma were treated: 24 patients with Hodgkin's lymphoma (HL) and 38 with non-Hodgkin's lymphoma (NHL). Relapse rate in children with HD before 1971 (pre-laparotomy data) was significantly higher than in the group after 1971, when explorative laparotomy, splenectomy and more aggressive radio- and chemotherapy were applied (1015 vs 29). Since 1970 all children with NHL were entered in a modified leukaemia protocol combining radiotherapy to the primary site, prophylactic C.N.S.-irradiation and aggressive multiple drug chemotherapy. In this group 2326 patients achieved complete remission vs 712 before 1970, when therapy consisted of low dose irradiation to the primary site, and/or single agent chemotherapy. Median survival in the group before 1970 was 5 months compared with 34+ months after 1970. Only 1 patient receiving prophylactic C.N.S.-irradiation developed C.N.S.-leukaemia vs 512 in the group before 1970.