Numerous studies have reported that monoclonal antibody (mAb) FMC7 detects an antigen present on only a subset of circulating B lymphocytes. In particular, this mAb may distinguish typical B-cell chronic lymphocytic leukemia (FMC7 negative) from other types of B-cell non-Hodgkin lymphoma (B-NHL; FMC7 positive). We treated patients with B-NHL with Rituxan, a chimeric CD20 mAb, and observed abrogation of staining not only with prototype CD20 mAb B-1 but also with mAb FMC7. To investigate the relation between antigens CD20 and FMC7, we performed mutual blocking studies that showed mutual inhibition of FMC7 and CD20. In addition, The antigen detected by FMC7 has not been characterized in detail, and only one study using immunoblot analysis found that this antigen is a 105-kDa molecule (9).In the diagnostic setting, FMC7 has been widely used to distinguish FMC7-negative B-cell chronic lymphocytic leukemia (B-CLL) from other varieties of B-cell non-Hodgkin lymphoma (B-NHL) (10 -13). Matutes et al. (14) established a scoring system for the proper diagnosis of typical B-CLL, and one criterion is lack of FMC7 expression. In the studies cited, the cases considered FMC7 negative were classified as such, even though a substantial proportion of cells stained with FMC7. Thus, cases in which up to 30% of all monoclonal B cells stained with FMC7 were considered FMC7 negative (14). Hübl et al. (15) reported that CD20 expression and FMC7 expression were parallel, but they did not discover the exact relation of these antigens. Specifically, they did not find mutual inhibition of binding between mAbs CD20 and FMC7. In contrast, D'Hautcourt and Isaac (16) reported that phycoerythrin (PE)-labeled CD20 mAb substantially reduced binding of fluorescein isothiocyanate (FITC)-labeled FMC7 mAb, and they hypothesized that poorly defined steric inhibition was the cause.Because we were treating B-NHL patients with the chimeric CD20 mAb Rituxan, we investigated the relation of antigens CD20 and FMC7. The prototype CD20 mAb B-1 modulated the phenotypic expression of other surface antigens by inducing enzymatic cleavage within 4 h in an experiment using CESS cells and CD23 antigen (17). In view of that finding we performed flow cytometric, short-
This paper describes the successful mobilization of peripheral blood stem cells for autologous transplantation in three children with malignant diseases by using plerixafor (Mozobil™; Genzyme Corporation, Cambridge, MA) and granulocyte-colony stimulating factor (G-CSF) after failed previous mobilizations. A median sixfold increase in the number of circulating CD34+ cells after plerixafor treatment as compared with the baseline level was observed. An optimal CD34+ cell count for transplantation with one or two leukapheresis sessions was achieved. Mobilization using plerixafor was found to be safe with no adverse events. Therefore, the combination of G-CSF and plerixafor in children results in effective increases in peripheral CD34+ cell counts and reduces the risk of mobilization failure.
We report a rare case of cerebellar degeneration as a paraneoplastic syndrome in an 8-year-old boy with Hodgkin lymphoma that presented during first-line treatment. Antibodies against Purkinje cells (anti-Tr antibodies) were detected in the serum of the patient. After successful treatment of the lymphoma, the cerebellar symptoms resolved partially. Childhood presentation of paraneoplastic cerebellar degeneration is extremely rare, with only a few reports in the literature. For this reason, the description of all such cases contributes to the enrichment of the medical knowledge and will improve the diagnosis and the treatment of this complication.
Allogeneic haematopoietic SCT (allo-HSCT) from a matched sibling donor is a curative treatment option for patients with high-risk ALL. Congenital or acquired diseases of the donor are potentially transmissible to the recipient.1,2 There are reports of immune thrombocytopenic purpura (ITP) after allo-HSCT associated with GVHD, 3 anti-plt allo-Abs, 4 autoimmune pancytopenia and thrombocytopenia, 5 and of acquired thrombocytopenia from a donor with preceding chronic ITP. 6,7 We report here a case of ITP transmission through allo-HSCT.A 13-year-old boy with high-risk ALL failed to achieve remission after standard induction therapy. CR was achieved after salvage therapy combination and the patient was considered appropriate for an upfront allo-HSCT from his HLA-identical 17-year-old sister. The prospective donor had a 4-year history of chronic ITP with plt counts ranging between 20-30
Background: During the last four decades the prognosis of childhood acute myeloid leukemia (AML) has been substantially improved due to an increase in complete remission (CR) rates, event-free survival (EFS) and reduced early mortality. The relapsed AML still remains a therapeutic challenge. Aim: To report the AML treatment results of the Bulgarian pediatric oncohematological centers. Materials and methods: Retrospective analysis of the treatment results of children and adolescents (age from 0 to 20 years) with primary AML. Unifi ed AML BFM-backbone type treatment protocol is used. Results: This study included 97 newly diagnosed patients (44 girls and 53 boys) with AML in Bulgaria between 2003 and 2016. The median age at diagnosis was 10.2 years. The most frequent FAB-morphologic subtype was М2 followed by М4. First complete remission (CR1) was achieved in 83 patients (85.6%). The 13-year EFS was 49%, while the overall survival (OS) was 54.6%. Twenty seven (27.8%) patients relapsed, with only 5 of them being still alive towards the end of the study period. Conclusion: The EFS and OS for the children with AML in Bulgaria are comparable with those reported by other European groups. The prognosis of relapsed AML remains still unfavorable for the past 13 years. BACKGROUND
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