Biomedical subjects
Publications and source records attributed to T Eckschlager.
IgG, IgA and IgM levels were examined in children treated for different malignant tumors. Chemotherapy decreased IgG and mainly IgM. There was increased incidence of infectious complications in IgG deficiency.
A mixed germ cell-sex cord stromal tumor developed in the right ovary of a 4-year-old girl. The patient's cells contained sex chromatin and the karyotype was 46,XX. Clinically, she exhibited mild isosexual pseudopuberty. Some of the tumor cells showed differentiation to Sertoli cells, whereas others had characteristics of germ cells. The neoplasm was composed of solid cords consisting of a mixture of the two tumor cell types and surrounded by a delicate connective-tissue network. In one small area, the tumor contained heterologous development in the form of glands and cysts lined by columnar mucinous epithelium containing numerous goblet cells and occasional argyrophilic neuroendocrine cells. Normal ovarian tissue was present at the periphery of the tumor. Electron microscopic study confirmed the presence of both Sertoli-like and germ cell-type cells. Immunohistochemical studies demonstrated vimentin positivity in both cell types (with preference for the Sertoli-like cells) and cytokeratin positivity in the Sertoli-like cells. The patient was symptom free 4 years after right oophorectomy, radiotherapy, and chemotherapy.
A tumor of the tongue with features consistent with the diagnosis of fetal cellular rhabdomyoma was seen in an 18-month-old infant. The tumor recurred 10 and 22 months after initial resection. The histologic condition of the first recurrence was similar to the original tumor except for some increased nuclear irregularities and mitotic activity. No adjunctive therapy was administered initially or after the first recurrence. The second recurrence showed mixed embryonal/alveolar rhabdomyosarcoma. This case represented a unique model of a highly differentiated striated muscle tumor converting to a moderately differentiated rhabdomyosarcoma, and illustrated differential diagnostic difficulties in distinguishing between fetal cellular rhabdomyoma and differentiated rhabdomyosarcoma. The patient was compared with eight children and adolescents with primary sarcomas of the tongue who entered the Intergroup Rhabdomyosarcoma Studies (IRS) I, II, and III protocols. The tumors of all eight arose at the base of the tongue. There were five embryonal, one alveolar, and one mixed embryonal/alveolar rhabdomyosarcomas, and one undifferentiated myxoid sarcoma. Five rhabdomyosarcomas were poorly differentiated, and two had a moderate degree of myogenesis. These sarcomas of the tongue represent approximately 0.34% of all cases entered in IRS studies.
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Normal human B lymphocytes are sensitive to the growth-inhibitory action of transforming growth factor beta1 (TGFbeta1) whereas malignant B lymphoma cells are mostly resistant to TGFbeta1 effects. We examined the phosphorylation status of retinoblastoma protein and the activity of G(1) cyclin-dependent kinases (cdk) in TGFbeta1-sensitive malignant follicular lymphoma cells during the TGFbeta1 treatment. The kinase activity of cdk2, cdk4, and cdk6 was significantly reduced and hypophosphorylation of pRb on serine 795 (S795) and threonine 373 (T373) was observed. We examined the composition of cdk complexes and the level of cdk inhibitors to explain the inhibitory action of TGFbeta1 toward cdk activity. Both cdk4 and cdk6 were notably dissociated from cyclin D cofactors, while cyclin E-cdk2 complexes remained coupled in TGFbeta1-treated cells. TGFbeta1-induced growth arrest was associated with notably increased binding of p21(WAF1) to cdk4 and cdk6. No induction of cdk-inhibitor molecules of INK family was observed in TGFbeta1-treated DoHH2 cells. As shown, TGFbeta1-induced growth arrest of malignant B cells was associated with the activation of CIP/KIP family members of cdk inhibitors.
Clinical impact of s.c. administration of IL-2 and/or IFN alpha was studied in 23 pediatric patients with Hodgkin lymphoma (IFN alpha group) and sarcoma, non-Hodgkin lymphoma, peripheral neuroepitelioma, neuroblastoma, and embryonic carcinoma (IL-2 + IFN alpha group) after autologous PBSC transplantation. Expression of CD3, CD4, CD8, CD25, CD38, CD56, CD71, CD122, and HLA-DR antigens, serum level of the soluble IL-2R alpha, and NK activity against K562 cell line were evaluated in 11 patients representative for both types of immunotherapy. T and, more markedly, NK cell proliferation, induction of activation markers on the surface of T and NK subsets, and elevation of sIL-2R alpha concentrations were seen in the IL-2 + IFN alpha subgroup. In the IFN alpha subgroup, the total number of lymphocytes and expression of activation markers remained unchanged, but the number of CD8+ T cells increased at the expense of CD4+ T and NK cells during the therapy. Cytotoxic activity against K562 cells was not influenced by the immunotherapy in either subgroup. No significant clinical benefit of the immunotherapy was seen in these patients compared to 27 control patients with relevant diagnoses who did not receive immunotherapy.
Natural killer (NK) cell activity and lymphocyte proliferation to mitogens might to have diagnostic implications that will help to distinguish between benign and malignant lymphadenopathy in children. In addition, we suggest that NK activity and especially the lowering of NK activity after chemotherapy might be of prognostic value in NHL children.
To evaluate the significance of the peripheral lymphocyte count in the prognostication of childhood cancer, 173 children with cancer (neuroblastoma, non-Hodgkin's lymphoma, malignant lymphogranuloma, nephroblastoma, Ewing's sarcoma, and rhabdomyosarcoma) were studied. All patients with the above-mentioned diagnoses admitted for the first time between 1985 and 1987 without prior treatment and acute infection were eligible for the study. Elevated peripheral lymphocyte count seems to be an independent indicator of survival from neuroblastoma but not from other tumors.
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