RESUMEN
A 46-year-old man with chronic myelogenous leukemia was found to have a new complex translocation. In chronic phase, all of the bone marrow cells had a rearrangement of a t(2;9;14;22) (p21;q34;q32;q11). Southern blot analysis of leukocyte DNA revealed rearrangement of the breakpoint cluster region (bcr) within the 5.8-Kb bcr. The patient eventually died in blast crisis 28 months later. The cytogenetic findings of bone marrow cells showed a 46,XY,t(2;9;14;22)(p21;q34;q32;q11),add(1p),del(3q) karyotype in blast crisis.
Asunto(s)
Leucemia Mielógena Crónica BCR-ABL Positiva/genética , Translocación Genética , Bandeo Cromosómico , Cromosomas Humanos Par 14 , Cromosomas Humanos Par 2 , Cromosomas Humanos Par 22 , Cromosomas Humanos Par 9 , Humanos , Cariotipificación , Masculino , Persona de Mediana EdadRESUMEN
A 74 year-old woman, who had been diagnosed as AML (M3) in poor condition, was treated with Retinol Palmitate (Chocola-A, 150,000 unit/m2 per os, after informed consent. An increase of white blood cells (neutrophil) counts was observed after 7 days. After 4 weeks, WBC counts were increased to 20,700/microliters (neutrophil counts 6,400/microliters) Maturation tendency of leukemic cells was also proved in the bone marrow. In vitro studies showed that morphological differentiation was recognizable in cultured leukemic cells treated with 10(-6)M all-trans retinoic acid after 6 days, but not in controls. Responses in the NBT reduction test were slightly less than in the clinical study. The administration of Retinol Palmitate may be a new regimen to treat AML (M3) in aged patients in poor condition.
Asunto(s)
Leucemia Promielocítica Aguda/tratamiento farmacológico , Vitamina A/análogos & derivados , Administración Oral , Anciano , Diferenciación Celular/efectos de los fármacos , Diterpenos , Femenino , Humanos , Leucemia Promielocítica Aguda/patología , Ésteres de Retinilo , Células Tumorales Cultivadas/efectos de los fármacos , Células Tumorales Cultivadas/patología , Vitamina A/administración & dosificación , Vitamina A/uso terapéuticoRESUMEN
A case of autoimmune hemolytic anemia associated with myasthenia gravis in a 33-year-old female is presented. The association of autoimmune hemolytic anemia and myasthenia gravis is a very rare event, with only eight cases reported. The known association of these two diseases is reviewed. To our knowledge, this is the first reported case in which autoimmune hemolytic anemia preceded systemic myasthenia gravis.
Asunto(s)
Anemia Hemolítica Autoinmune/complicaciones , Miastenia Gravis/complicaciones , Adulto , Femenino , Humanos , Miastenia Gravis/patología , Miastenia Gravis/cirugía , Timectomía , Timo/patología , Factores de TiempoRESUMEN
The inhibitory activity of T cells on autologous erythroid colony-forming units (CFU-E) (T cell inhibitory activity) in patients with aplastic anaemia (AA) was investigated. In 11 (32.4%) out of 34 AA cases, T cell inhibition on autologous CFU-E growth was greater than that in normal individuals. In order to evaluate the mechanism of this inhibitory activity, T cell surface markers, interferon (IFN) production in peripheral blood mononuclear cell (PBMNC) liquid culture, and cytokine levels such as IFN and tumour necrosis factor-alpha (TNF-alpha) in CFU-E clot cocultured with T cells, were measured in a portion of the patients. In five patients investigated for IFN production in PBMNC liquid culture, all produced statistically more IFN activity than normal individuals under phytohaemagglutinin (PHA-P) stimulation (P less than 0.01) with no relation to T cell inhibitory activity. In only one patient whose T cells displayed increased CD8 and HLA-DR antigen (CD8+HLA-DR+) and inhibitory activity, a significant amount of IFN-gamma was observed in CFU-E clot cocultured with T cells, and the addition of anti-IFN-gamma antibody to the coculture resulted in recovered CFU-E colony growth. These results suggest that IFN-gamma production by T cells may explain, at least in part, the pathogenesis of haematopoietic defects in AA. In other patients however, T cell inhibitory activity neither correlated to the T cell subpopulations (CD4+/CD8+, CD8+HLA-DR+), IFN production in PBMNC liquid culture, nor to IFN and TNF-alpha levels in CFU-E clot culture. The roles played by cytokines other than IFN and TNF-alpha on haematopoietic precursor cells require further evaluation in a larger sample of patients with AA.
Asunto(s)
Anemia Aplásica/inmunología , Eritropoyesis/inmunología , Interferón gamma/inmunología , Linfocitos T/inmunología , Factor de Necrosis Tumoral alfa/inmunología , Adolescente , Adulto , Anciano , Anemia Aplásica/sangre , Células Precursoras Eritroides/inmunología , Femenino , Humanos , Interferón gamma/sangre , Masculino , Persona de Mediana Edad , Subgrupos de Linfocitos T , Factor de Necrosis Tumoral alfa/análisisRESUMEN
A 48-year-old woman was referred to Tohoku University Hospital in November 1981 because of leukocytosis pointed out in a group examination. At that time white blood cell count was 26.8 x 10(3)/microliters with no blasts, platelet count 268.0 x 10(4)/microliters and hemoglobin 11.4 g/dl. Bone marrow aspirates showed marked increase of megakaryocytes (15,900/microliters). Bone marrow chromosome analysis revealed 46, XX, -18, +mar without Ph1 chromosome, and DNA analysis showed no bcr rearrangement. She was diagnosed as having essential thrombocythemia and was treated with busulfan. On November 1986, she developed remarkable leukocytosis with leukemic blasts. White blood cells reached 153 x 10(3)/microliters with 33% blasts. Her blasts were positive for peroxidase staining, but negative for platelet peroxidase on electron microscopic study and platelet specific glycoproteins. A diagnosis of acute myeloblastic leukemia (M2) was made. The patient received various combination chemotherapy, which was ineffective, and she died due to pneumonia on June, 1989. In Japan, there has been reported only 8 cases of essential thrombocythemia transformed to acute leukemia. The clinical pictures of these 9 cases were discussed.