Pentasomy 21 characterizing spontaneously regressing congenital acute leukemia.

Pentasomy 21 was found to characterize the proliferating cells in a case of transient congenital acute leukemia (or congenital acute leukemia) with spontaneous remission. The patient was phenotypically normal, and cytogenetically no evidence could be found for the existence of a mosaic with a normal cell line and one with more than two No 21 chromosomes. The importance of these findings is discussed.

[Skeletal changes in children with acute lymphoblastic leukemia]. / Skeletafwijkingen bij kinderen met acute lymfoblastenleukemie.

Children with acute lymphoblastic leukemia frequently show skeletal changes. We examined 50 children with acute lymphoblastic leukemia with reference for skeletal pain as well as the nature and the degree of skeletal changes. 14 children had bone pain. The pain did not correlate with the roentgenographic findings. Some children had characteristic bone changes without bone pain. Roentgenographic findings were observed in 35 of the 50 children (70%). The major lesions observed were: transverse metaphyseal lucent bands, osteolytic lesions, osteosclerotic lesions, periosteal reaction and osteoporosis. Different pathologic findings in leukemia have been given a prognostic value. This was also done for the bony changes but the literature is not coherent on this point. In the group of 17 children with evident skeletal changes, 15 had a non B- non T-cell leukemia. Also the age of the patient, apart from the immunological cell type, seems to be an important factor in the incidence of initial skeletal roentgenographic findings.

Malignant otolaryngological tumors in children.

The details of 37 children presenting a malignant tumor in the ENT-region (period 1950-1983) are presented and discussed. The prevalence was higher in boys than in girls and the most common tumors were non-Hodgkin lymphoma (n = 14) and rhabdomyosarcoma (n = 13). The most common primary sites were the nasopharynx (9 cases), the paranasal sinuses (7 cases) and the soft tissue in the parotid region (7 cases). In 23 children the presence of a painless tumor was the first symptom, in 11 pain was predominant. Only a minority (5 children) presented a limited tumor; in 20 children there was a local extension of the tumor and in 10 children metastases were present at the first visit. The beneficial role of chemotherapy and the necessity of a good teamwork in the treatment of these children is outlined. Of the 37 children, 14, (38%) are surviving; of these 14 children, 12 are off therapy and cured. It is demonstrated that the prognosis improved considerably since the last 14 years.

Meningeal leukaemia in the blastic phase of chronic granulocytic leukaemia.

A 51/2-year-old boy is presented with chronic granulocytic leukaemia and blastic transformation whose clinical course is complicated by meningeal leukaemia. In the authors' opinion prophylactic central nervous system therapy should be part of the initial therapy of blastic transformation.

Oral BVDU treatment of varicella and zoster in children with cancer.

In the period June 1980-April 1983, 21 children with malignant disease were admitted because of an intercurrent varicella or zoster infection. They were treated with the new antiviral drug BVDU [(E)-5-(2-bromovinyl)-2'-deoxyuridine]. The drug was administered orally at a dose of 15 mg/kg per day for 5 days. All our patients responded promptly to BVDU treatment and recovered completely from their varicella or zoster infections without complications. No toxic side-effects due to the drug were observed.

Comparison of chemotherapy with immunotherapy for maintenance of acute lymphoblastic leukemia in children and adults.

Two hundred and seventeen patients, 1-50 yr old, with acute lymphoblastic leukemia in complete remission were randomized to receive a 1-yr consolidation chemotherapy of either type P, comprising 7 different drugs, or type M, consisting of methotrexate interspersed with prednisone and vincristine. Thereafter, they were randomized a second time to receive a 4-yr maintenance of either chemotherapy or immunotherapy, comprised of allogeneic blasts and bacillus Calmette-Guérin (BCG). Consolidation P caused more toxicity than consolidation M. However, comparison between the consolidation therapies P and M showed no significant difference, neither for disease-free interval nor for duration of survival. Chemotherapy showed more lethal toxicity in adults than in children. Comparison between chemotherapy (C) and immunotherapy (I) as maintenance treatment showed a significant (p = 0.016) superiority of C for disease-free interval (DFI). The difference was even more pronounced (p = 0.009) in the group with less than 8 g/dl of hemoglobin (Hb) at diagnosis before therapy. On the other hand, for patients with more than 8 g/dl Hb at diagnosis, presumably those with T-ALL, no difference in DFI was seen. No difference has been seen so far between maintenance therapies I and C concerning the duration of survival. The patients who were receiving maintenance I when they relapsed and who were consequently retreated by chemotherapy, survived longer from relapse than those patients retreated for relapse while receiving maintenance C.

Treatment of acute lymphoblastic leukemia in children with the BFM protocol. A cooperative pilot study.

From January 1981 through July 1983, 141 children with newly diagnosed acute lymphoblastic leukemia were registered in a cooperative clinical study whose objective was to evaluate the toxicity and the feasibility of the German Berlin-Frankfort-Münster (BFM) protocol. The results were comparable with those reported by the BFM group. For the 133 patients (94%) who achieved complete remission, the actuarial disease-free survival was 67% at 4 years. These results were obtained in spite of a high rate of deaths in complete remission during the initial year of the study. Subsequently, probably as a result of improved expertise in the handling of the protocol, the proportion of toxic deaths declined sharply. Although the current BFM protocol adjusts for aggressiveness of therapy according to the volume of the liver and spleen, splenomegaly (but not hepatomegaly) remained of prognostic significance. Moreover, for patients with bad risk features, an initial high hemoglobin level was found to represent an additional factor of negative significance.