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  • 1
    ISSN: 1432-1076
    Keywords: Neuroblastoma ; 131-metaiodobenzylguanedine treatment ; Therapeutic effect ; Tolerance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Eleven children with neuroblastoma refractory to conventional therapy or relapse of neuroblastoma were treated with 131-metaiodobenzylguanedine (MIBG). The therapeutic results and the side effects were evaluated. In one patient with disseminated bone marrow involvement complete remission was obtained. Partial remission was observed in six patients and stable disease in another. Three patients did not respond to MIBG, in two of them the tumours did not accumulate a sufficient MIBG dose. Clinical and laboratory examinations revealed an excellent tolerance of MIBG in all patients. First attempts to continue cytostasis after MIBG therapy were made. MIBG has a good therapeutic efficacy is sufficiently incorporated into the tumour cell.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0584
    Keywords: Leukemia ; Childhood acute lymphoblastic of T-cell type ; Hand mirror variant ; Chromosome Translocation (11; 14)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three boys, 12, 15 and 5 years old are presented with acute lymphoblastic leukemia resp. Non-Hodgkin's lymphoma with leukemic transformation. Blast cells could be characterized as being of T-cell origin. Hand mirror variant was the predominant morphologic feature of the blast cells in two patients. Chromosome analysis of the leukemic blast cells revealed a pseudodiploid (modal chromosome number =46) karyotype in two patients and a pseudotetraploid (modal chromosome number =92) in one patient. A chromosome translocation (11; 14) with breakpoints at (p 13; q 13) (within the human T-cell receptor α chain locus!) was found in the leukemic cells of all three cases plus an additional t (7; 9) (q 22; p 13) in one patient.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0584
    Keywords: Acute childhood lymphoblastic leukemia ; T-cell immunophenotype ; Chromosome 14q11 anomaly
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Ten patients with T-cell acute lymphoblastic leukemia (ALL) and a chromosome anomaly involving band 14q11 are described. Mitotic index of bone marrow blasts was high in all patients (average 3.0%). Lymphoid morphology of the leukemic blasts, however, varied somewhat among the patients. The leukemic cells of 5 patients showed an immunophenotypic profile corresponding to early or common thymic differentiation stages whereas 5 children showed strong expression of CD3 suggesting a more mature thymic phenotype. Leukemic karyotypes revealed a modal chromosome number of 46 in 9 cases, 92 in one case. A chromosome translocation t(11; 14) (p13; q11) was found in 5 cases, a t(1; 14) (p32; q11) in 2 cases, a t(10; 14) (q24; q11) in one case, a (hitherto undescribed) t(12; 14) (q22; q11) in one case, and an inv(14) (q11 q32) in one patient. Additional abnormalities were t(3; 10), t(7; 9), dup (7q), del (6q), del (10q), and del (1 q). Of 32 cases with T-cell ALL successfully karyotyped in our laboratory 15 (=47%) had structural aberrations involving chromosomes 1, 3, 6, 7, 9, 10, 12, 14. Ten of these 15 patients (=67%) had a chromosome 14q11 anomaly. It is concluded that chromosome band 14q11, the gene locus of the T-cell receptor α-chain, is the most common site for structural chromosome aberrations in T-cell ALL.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0584
    Keywords: Acute leukemia ; (4; 11) chromosome translocation ; Early B-precursor cell origin ; Mixed lineage leukemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical and laboratory features of seven patients with acute leukemia associated with the (4; 11) chromosome translocation are presented. Leukemic blasts of these patients showed lymphoid morphology in 6 (although 1 was treated for monoblastic leukemia 3 years earlier) and monocytoid morphology in 1, were positive for TdT and HD 37 (CD 19) in 6 patients, whereas weak expression of CALLA was seen in only 1 patient and T-lineage-associated antigens in none. Leukemic blasts from four patients showed the simultaneous expression of B-lymphoid and myeloid antigens, suggesting leukemogenesis in a very early multipotent progenitor cell. In 2 patients an isochromosome of the long arm of No. 7 chromosome was found in the leukemic karyotypes in addition to t (4; 11) (q21; q23); in one instance present at diagnosis, in the other one occurring at relapse. In one other patient leukemia karyotype also demonstrated trisomy 8. Leukemic cells of three patients were investigated by molecular genetics and demonstrated immunoglobulin gene rearrangements for the Ig heavy chain sequences but not for the light chain constant regions and T cell receptor sequences. All patients were treated by intensive chemotherapy. Four of the 7 patients are in continuous complete remission. The longest event-free survival time (over 2 1/2 years) was seen in one patient who had also DOWN-syndrome. Including these 7 patients a clinical analysis of 71 patients with t (4; 11) acute leukemia was made, emphasizing the following characteristics at diagnosis: female sex (62%), age under 2 years (49%), leukocyte count over 100×109/1 (61%), splenomegaly (80%), CNS-disease (11%). Survival of over 2 years was reported in less than 15% of the patients. It remains to be seen if risk-adapted treatment can alter the course of this early B-precursor acute leukemia with hitherto very bad prognosis.
    Type of Medium: Electronic Resource
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