RESUMEN
Thirteen patients with Langerhans cell histiocytosis (LCH) have been treated in hospital Brno Masaryk University during the last 15 years. In 4 cases of this total amount, the diagnosis was made in childhood and these young adults were referred to our department from Pediatric cancer center. In 9 cases, the diagnosis has been made in people elder than 18 years. The disease recidived in 3 patients with LCH diagnosed in childhood, in one case with neurodegenerative impairment of brain. In 4 patients from the total of 9 with LCH diagnosed in age over 18 years, the disease had aggressive course with several recidives. In one case it was pulmonal and multifocal osseal manifestation, in two cases multifocal osseal disease. Isolated pulmonal form of LCH was diagnosed only in one patient. By the first patient, high dose melphalan and etoposide with peripheral blood stem cell transplantation was performed after failure ofvincristin and prednison therapy and failure of etoposide therapy. The first remission after this high dose therapy lasted only 2.5 years. For relapse second cycle of the same high dose therapy was administered, but the next remission was much shorter. By the two patients with multifocal recidives in bones 2-chlordeoxyadenosine was administered as initial therapy. These patients are followed up for more then 24 months and they are without relapse of this disease. The 2-chlordeoxyadenosine is very efficient in multifocal bone form of LCH and has potential to reach long remission. Therefore in a case of aggressive multifocal disease we would prefer 2-chlordeoxyadenosine therapy as therapy of the first choice.
Asunto(s)
Histiocitosis de Células de Langerhans/diagnóstico , Adulto , Femenino , Histiocitosis de Células de Langerhans/complicaciones , Histiocitosis de Células de Langerhans/terapia , Humanos , Masculino , RecurrenciaRESUMEN
Accurate prognostic evaluation of patients with multiple myeloma (MM) is required for their stratification for more adequate therapy. Chromosomal G-banding and interphase fluorescence in situ hybridization (FISH) on cell-nonspecific samples and on myeloma cells selected by magnetic-activated cell separation (MACS) were used to study 13 samples from 12 multiple myeloma (MM) patients. Bone marrow (BM) samples were analysed using three approaches. Standard mitotic samples were prepared and analysed after G-banding. Interphase FISH was performed to detect the 13q14 deletion in unselected BM cells. In parallel, myeloma cells were selected from the BM using the CD138-specific antibody. The high-purity myeloma cell suspension was then analysed by interphase FISH for the 13q14 deletion. Magnetic separation yielded enriched myeloma cell suspensions with the mean viability of 98.0% (range: 97.0%-99.0%), and the purity of 97.6% (range: 87.2%-99.2%) as detected morphologically, and 85.2% (range: 44.8%-98.4%) as detected by immunophenotyping for CD138+ cells. Interphase FISH revealed the 13q14.3 deletion in 5 of 13 (38.5%) of cell-nonspecific samples and in 9 of 13 (69.2%) of enriched myeloma cell suspensions. In conclusion, interphase FISH on immunomagnetically selected MM cells increases the detection of the 13q14 deletion in BM samples from the patients with MM.
Asunto(s)
Aberraciones Cromosómicas , Cromosomas Humanos Par 13 , Separación Inmunomagnética , Mieloma Múltiple/genética , Células Cultivadas , Bandeo Cromosómico , Eliminación de Gen , Humanos , Inmunofenotipificación , Hibridación Fluorescente in Situ , Magnetismo , Glicoproteínas de Membrana/biosíntesis , Mitosis , Mieloma Múltiple/sangre , Proteoglicanos/biosíntesis , Sindecano-1 , SindecanosRESUMEN
We report our results with high-dose chemotherapy in previously untreated multiple myeloma patients (4 courses of VAD chemotherapy, collection of PBSC after priming with cyclophosphamide, 5 g/m2, high-dose chemotherapy with melphalan, 200 mg/m2). Second transplantation was indicated only for patients who did not achieve remission after the first high-dose therapy (paraprotein lower than 25% of the pretreatment value). For the second transplantation melphalan (200 mg/m2) with methylprednisolone (1.5 g for 5 days) were used as conditioning regimen. After high-dose therapy all patients were randomized into two arms of maintenance therapy: interferon alpha-2b or sequential maintenance therapy (interferon alpha-2b for 3 months followed after 4 week pause by 40 mg of dexamethasone days 1-4, 10-13 and 20-23. The administration of interferon alpha was resumed four weeks after the last dexamethasone for next three months. The maintenance therapy continued for 48 months or until the progression. Fifty-five patients were enrolled in the study from January 1996 to August 1997. Thirty-five patients have undergone the first transplantation and 57% of them reached complete remission. There were 10% of non-responders after the first high-dose regimen. The mean time to reach white blood cell count above 1 x 10(9)/L after the application of high dose melphalan and platelets more than 50 x 10(9)/L were 12.2 (range 6-16 days) and 12.4 (range 0-25 days), respectively. Grade 4 mucositis according to SWOG classification requiring total parenteral nutrition was presented in 40% of the patients. The mean number of 1 unit of platelets and 2 units of packed red blood cells transfusions were given within the posttransplant period. Early transplant related mortality was 3%. This paper describes the response and tolerance of each particular step of therapy. The follow-up has been too short to evaluate event-free and overall survivals.