Treatment of acute idiopathic thrombocytopenic purpura of childhood with intravenous immunoglobulin G: comparative efficacy of 7S and 5S preparations.

A prospective randomized study comparing 7S immunoglobulin G to a 5S IgG preparation for therapy of acute idiopathic thrombocytopenic purpura was conducted. The 5S preparation differed from the 7S preparation in that it lacked part of the Fc portion of the IgG molecule. Both groups were given 400 mg IgG/kg body weight over 5 days. All patients had platelet counts less than or equal to 30 X 10(9)/L before IgG infusion. Nine of the 10 patients in the 7S treatment group, compared with three of 10 patients in the 5S treatment group, responded to therapy with an increment in platelet counts greater than 100 X 10(9)/L within 4 days of completing the infusion treatment. Furthermore, the rate of increase of the platelets was more rapid in the 7S group. The results emphasize the efficacy of partial Fc for management of acute idiopathic thrombocytopenic purpura.

Bone metastasizing renal tumor (clear cell sarcoma) of childhood with epithelioid elements.

A case of bone metastasizing renal tumor (clear cell sarcoma) of childhood (BMRTC) is presented. Histologic examination demonstrated the typical histopathologic features of this type of tumor. In addition, epithelial cells were present which occurred in groups, either around a central eosinophilic core or occasionally around a small central lumen. To the authors' knowledge, the presence of epithelial cells in BMRTC has not been reported before. The histogenetic implication of this finding is discussed.

[Malignant pheochromocytoma in childhood and adolescence--clinical case and review of the literature]. / Maligne Phäochromozytome im Kindes- und Jugendalter--Kasuistik und Literaturübersicht.

In regard to the diagnostic and therapeutic dilemma concerning rare neoplasms in childhood, the presented paper includes the comprehensive description of a malignant pheochromocytoma in a 14-year-old girl on the one hand and the detailed analysis of a collected series of 21 equal cases in pediatric literature on the other hand. In our patient there was in particular a lack of hypertension in spite of proven hypercatecholaminemia, an initially good effect of the post-operative aggressive chemotherapy according to the neuroblastoma study NBL 79 (4b) resulting in a transient reduction of the pulmonary metastases, furthermore a diminuation of the total tumour mass induced by the last applied treatment with the radiopharmaceutical 131I-metaidiobenzylguanidine. The analysis of 21 case reports revealed a peak between 10 and 15 years of age, a striking prevalence of the female sex in all age groups, and a high frequency of metastases (75%) at the time of diagnosis. Two thirds of the primary tumours arose in extra-adrenal sites, about one third occurred in the adrenal gland. The regional lymph nodes, the bone system and the lungs were the main locations of metastatic lesions. The association with a group of other conditions, particularly hypoxemic congenital heart malformations has proved to be a remarkable feature, but not only in the malignant forms.(ABSTRACT TRUNCATED AT 250 WORDS)

[Leukocyte count and granulocyte function of children under lithium carbonate therapy treated with cytostatic drugs. A pilot study]. / Leukozytenzahl und Granulozytenfunktion zytostatisch behandelter Kinder unter Lithiumcarbonat. Eine Pilotstudie.

The usefullness of lithium carbonate for attenuation of leukopenia due to chemotherapy was studied in a prospective randomized trial. Chemotherapy for solid tumors was applied in a paired manner 24 times without and 24 times with lithiumcarbonate. Simultaneously, nucleotid-dependent functions of granulocytes were examined. An attenuation of leukopenia by lithium was observed. Toxic side effects within the therapeutic range of the serum level (0.3-1.4 mol/l) were not detected. Lithium did not impair adherence, chemotaxis and degranulation of neutrophile granulocytes. The influences of lithium on stem-cell maturation and granulocyte function are discussed.

[Helper T-cell deficiency in infantile genetic agranulocytosis (M. Kostmann)]. / Helfer-T-Zellmangel bei Infantiler Genetischer Agranulozytose (M. Kostmann).

A female turkish infant with congenital infantile genetic agranulocytosis was examined for its distribution of lymphocyte subclasses. Imbalances of B cells and T-cell subpopulations were found. Compared to the normal control group a relative as well as an absolute deficiency of T helper cells and an increase of the absolute number of T suppressor cells were the most prominent findings. The importance of immunoregulatory T-cell subsets in the pathogenesis of infantile genetic agranulocytosis, disorders of hematopoiesis and granulocyte production, as well as primary and secondary immunodeficiencies is discussed.

A new working formulation of non-Hodgkin's lymphomas. A retrospective study of the new NCI classification proposal in comparison to the Rappaport and Kiel classifications.

Two hundred thirty cases of malignant non-Hodgkin's lymphomas were reclassified in a retrospective study according to the New Working Formulation for Clinical Usage of the NCI as compared to the Rappaport and Kiel classifications. The reproducibility for the individual schemes this study was 81% (Rappaport), 79% (Kiel), and 85% (New Working Formulation). In keeping with the results of the NCI international study, all lymphomas were subdivided into 3 prognostic groups: (1) low-grade malignancy (6.0 years median survival); (2) intermediate-grade malignancy (3.5 years median survival); and (3) high-grade malignancy (1.4 years median survival). The NCI-proposed New Working Formulation for Clinical Usage is thus recommended as practical and unprejudicing classification scheme for general application; however, its usefulness as tool for translating one classification scheme into another appears limited.

[Pre- and retrorenal neuroblastoma without kidney displacement]. / Prä- und retrorenale Neuroblastome ohne Nierenverlagerung.

Pre- and retrorenal growing neuroblastomas did not produce any displacement of the kidney or any obstruction in the intravenous pyelogram. The diagnosis was done by ultrasound-scanning as well as biochemically before surgery. The reported cases show that sonography and determination of the catecholamine metabolites are essential in the diagnostic evaluation of abdominal tumors in childhood.

[Diagnostic-therapeutic trial in infantile genetic agranulocytosis with leucorecruitin]. / Infantile genetische Agranulozytose (IGA). Leukorecrutin im diagnostisch-therapeutischen Versuch.

Subject of this report is the usefulness of a biologically specific mediator of the leucocytosis reaction (leucorecruitin) in the diagnostic evaluation of granulopenic diseases. According to our trial the application of leucorecruitin in healthy humans resulted in a significant increase of granulocytes in circulating blood. The therapeutic effect depends on the neutrophil storage pool. In a diagnostic-therapeutic trial in infantile genetic agranulocytosis with depleted storage pools, leucorecruitin failed to lift the low number of circulating granulocytes to a normal level. No side effects could be observed so far.

[Membranes properties of granulocytes in Job's-Syndrome with E. coli-septicemia (author's transl)]. / Membraneigenschaften der granulozyten bei einem Hiob-Syndrom mit E. coli-Sepsis.

Since the age of nine weeks a red haired girl suffered from purulent dermatitis and recurrent, systemic E. coli infections. She had an excessive hyperimmunoglobulinemia E, as well as impaired granulocyte adherence and chemotaxis. Though a sepsis was evident, the granulocytes exhibited a random FITC-Concanavalin A fluorescence. In spite of intensive treatment with various antibiotics and several granulocyte transfusions the child died at the age of 2 years and 11 months. As shown by the FITC-Concanavalin A distribution, the hyperimmunoglobulinemia E may have caused a decreased membrane fluidity causing the impaired adherence and chemotaxis. This could explain the pathophysiology of the Job's Syndrome.

Lack of association between HLA specificities and Wilms' tumour.

HLA antigen distribution was determined in thirty patients with Wilms' tumour, and their frequencies compared with those of an ethnically matched control population. No statistically significant association was found between any single HLA antigen and Wilms' tumour disease. The value of prospective HLA typing studies, with special respect to genetic aspects, histopathological subgrouping and survival rate of Wilm's tumour patients is discussed.

Lack of association between the HLA-A10 (A25), B18 and C2o haplotype and anaphylactoid purpura (AP).

In 41 patients with AP and 134 healthy relatives HLA haplotypes and total hemolytic complement and C2 concentrations were determined. Though complement levels were found in the lower normal range in none of the individuals studied, a genetically determined C2 deficient state could be established. No association between low C2 levels and the HLA haplotype A10(A25), B18 or an isolated A25 or B18 was observed. A comparison of phenotype frequencies of AP patients with controls showed no significant difference. Contingency table analysis of patients and control haplotypes showed a definite close association with HLA-A1, Bw22, A2, Bw16, and A29, B12.

[Analysis of serum immunoglobulins and complement factors in children with chronic ITP and after reversible postinfectious thrombocytopenia (author's transl)]. / Serumimmunglobulin- und Komplementfaktorenanalyse bei Kindern mit chronischer idiopathischer thrombopenischer Purpura (ITP) und nach reversibler postinfektiöser Thrombopenie.

Levels of immunoglobulins G, A and M and complement factor C3 and C4 were determined by single radial immunodiffusion techniques in a group of 26 children with a history of postinfectious thrombocytopenic purpura and in another group of 6 children with chronic ITP. The statistical evaluation of the data of patients with chronic ITP did not show any significant deviation from normal. In patients with previously acute, reversible ITP, levels of IgG and IgM were elevated. Whether this elevation was significant or not depended upon the statistical analysis applied. C3 levels tended to be above the average normal mean, C4 levels, in contrast, to be below. The importance of increased IgG and IgM concentrations in patients with the history of postinfectious ITP is discussed. The raised IgG and IgM levels are interpreted as an expression of a certain type of hyperimmune state rather than as an impairment of immunoregulatory mechanisms.

HLA frequencies and haplotypes in children with idiopathic thrombocytopenic purpura (ITP).

26 patients with an acute reversible ITP and 6 with chronic ITP were tissue typed, together with their healthy first-degree relatives. The HLA frequencies of the different groups were compared with those of a normal control population. The only significant difference between the groups was an increase in the frequency of Aw32 in acute ITP patients. HLA-Aw32 was present in 26.9% of patients, but in only 0.8% of the controls (corrected P = 0.000027). The possible importance of associations between antigens of the HLA-A locus with certain diseases are discussed. Family analyses and haplotype determinations proved to be unproductive because no familial clustering of ITP was found.

Combined immune deficiency syndromes with primary T-cell defect and partial B-cell reactive hyperactivity. Immunological and morphological analysis of two unusual cases.

Two cases of combined immunodeficiency with lymphopenia, thymic dysplasia, and defective immunoglobulin production are reported. Both show selective hypo-gammaglobulinemia (IgG and IgA respectively) and selective hyper-gammaglobulinemia (both IgE, IgA, and IgM respectively). The cases are classified, by correlation of clinical and histopathological data as a variant of Fireman's disease.