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Summary. Hereditary motor and sensory neuropathy type 1 (HMSN-1) is an autosomal dominant disorder, which is usually not associated with neoplastic diseases. The disease predisposes to severe vincristine neurotoxicity. We report a 31-year-old women with recurrent Hodgkin's lymphoma and unrecognized HMSN-1 who developed severe motor neuropathy 3 weeks after the first cycle of treatment including 2 mg of vincristine. HMSN is diagnosed in most cases retrospectively, usually suggested by the observation of foot abnormalities or family history. Recognizing early signs of HMSN, such as areflexia and pes cavus deformity, can prevent severe neurotoxicity of polychemotherapy by avoiding vincristine.Keywords: vincristine, neurotoxicity, hereditary motor and sensory neuropathy, Charcot±Marie±Tooth syndrome.Vincristine is an essential part of the chemotherapeutic regimens used for Hodgkin's and non-Hodgkin's lymphoma and acute lymphocytic leukaemia, and plays an important role in the treatment of several solid tumours. Neurotoxicity, most frequently symmetric peripheral neuropathy caused by axonal degeneration, is well-known as a dose-limiting sideeffect of vincristine (Weiss et al, 1974). A general predisposition for vincristine-related neuropathy has been observed in people with a history of neurological disorders, such as Guillain±Barre  syndrome, childhood poliomyelitis and hereditary neuropathies. Hereditary motor and sensory neuropathy (HMSN), also known as Charcot±Marie±Tooth syndrome, is one of the most common types of familiar polyneuropathy with a prevalence of 20±40 in 100 000. Most patients suffer from the demyelinating type of HMSN, also termed HMSN type 1, an autosomal dominant disorder that is associated predominantly with a 1´5 Mb tandem duplication in chromosome 17p11.2±12 (Timmerman et al, 1990;Lupski et al, 1991). Although previous reports have pointed out a particular sensitivity to vincristine among patients with HMSN, knowledge of this association is not widespread. We would like to describe the clinical course of a female patient with unrecognized HMSN type 1, who developed severe neuropathy after vincristine treatment. CASE REPORTA 31-year-old woman was referred to our hospital with relapse of nodular sclerosing Hodgkin's lymphoma. Staging revealed clinical stage IVA with osseous manifestation. At this time the patient felt well without any subjective symptoms. Her initial family history was unremarkable. After informed consent she was enclosed in the BEACOPP protocol which included cyclophosphamide 650 mg/m at three-weekly intervals. Three weeks after a 2 mg dose of vincristine, the patient developed extreme general muscle weakness. She was hardly able to cope with activities of daily life. Physical examination revealed pareses in feet, legs and hands, particularly marked peroneus palsy, generalized areflexia and bilateral pes cavus deformity (Fig 1). Electrophysiological studies showed marked reductions in the median motor conduction velocity to 14´4 m/s (normal, . 50 m/s) and the peroneal nerve m...

Summary:High-dose chemotherapy with autologous transplantation of in vivo purged PBSC is a new and interesting therapeutic option for CML patients not eligible for allogeneic transplantation. We investigated the feasibility and toxicity of this approach in 57 patients with Ph-positive CML. For mobilization of Ph-negative PBSC, patients were treated either with '5 + 2/7 + 3'-type chemotherapy or with 'mini-ICE/ICE' chemotherapy followed by administration of G-CSF. Fourteen patients were in early chronic phase, 30 patients in late chronic phase and 13 patients in accelerated phase (AP) or blast crisis (BC). Cytogenetic responses in the PBSC harvests were dependent on both disease stage and type of chemotherapy: in late chronic phase and AP/BC, a complete or major cytogenetic response could be obtained in nine out of 13 patients treated with 'mini-ICE/ICE' but only in three out of 23 patients treated with '5 + 2/7 + 3' chemotherapy. However, in early chronic phase a Ph-negative autograft could be obtained in three out of eight patients upon mobilization with '5 + 2' chemotherapy. Thirty-one patients underwent PBSC transplantation and all of them successfully engrafted. Post-transplant cytogenetic analysis was available on 21 cases, of whom seven achieved a complete or major cytogenetic response, with two minor cytogenetic remissions. One patient (1/57) in blast crisis died during mobilization therapy (1.8%). Transplantation related mortality was 0%. This study demonstrates that mobilization of Ph-negative PBSC after myelosuppressive chemotherapy is feasible in CML patients and is associated with acceptable toxicity. Autologous transplantation of in vivo purged PBSC is a safe procedure with rapid and complete hematopietic recovery.

Objective: We investigated the health-related quality of life (HRQoL) of patients with gastrointestinal stromal tumours (GIST). Methods:In the multicentre PROSa study, the HRQoL of adult GIST patients was assessed between 2017 and 2019 using the European Organisation for Research and Treatment of Cancer HRQoL questionnaire (EORTC QLQ-C30). We performed group comparisons and multivariate linear regressions.

This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. AbstractObjectives: R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) is the standard therapy for patients with previously untreated diffuse large B-cell lymphomas (DLBCL). Dose-dense two-weekly 'R-CHOP-14' was not superior over three-weekly 'R-CHOP-21' in randomised clinical trials (RCTs). We present realworld data on effectiveness of R-CHOP-14 and R-CHOP-21 in patients with DLBCL treated in German routine practice. Methods: We identified 582 patients with DLBCL treated with R-CHOP-14 or R-CHOP-21 in 92 sites from the prospective clinical cohort study Tumour Registry Lymphatic Neoplasms. Patients' schedules were classified by (a) length of the initial first cycle and (b) length of cycles 1-4.Results: About 55% of patients received R-CHOP-21, 45% R-CHOP-14, in median 6 cycles. 51% and 55% of patients, respectively, were able to continue their initial R-CHOP-14 and R-CHOP-21 schedule. While most characteristics between the patient cohorts were similar, patients receiving R-CHOP-21 presented slightly more often with tumour stage I and lower IPI risk. 3-year overall survival of patients with R-CHOP-14 and R-CHOP-21 did not differ: 84% vs 84% (first cycle), 87% vs 89% (cycles 1-4). Conclusions:Patients with DLBCL in Germany are slightly more likely to receive R-CHOP-21 than R-CHOP-14. Both schedules are similarly effective in routine practice confirming the results from RCTs. K E Y W O R D Scohort studies, cyclophosphamide, doxorubicin, diffuse large B-cell lymphoma, outpatients, prednisone, prognosis, registries, rituximab, vincristine | 461 KNAUF et Al.