Assuntos
Neoplasias do Sistema Nervoso Central , Infiltração Leucêmica , Sarcoma Mieloide , Neoplasias da Medula Espinal , Medula Espinal/patologia , Feminino , Humanos , Leucemia Promielocítica Aguda/patologia , Leucemia Promielocítica Aguda/terapia , Infiltração Leucêmica/patologia , Infiltração Leucêmica/terapia , Pessoa de Meia-Idade , Indução de Remissão , Sarcoma Mieloide/patologia , Sarcoma Mieloide/terapia , Neoplasias da Medula Espinal/patologia , Neoplasias da Medula Espinal/terapiaRESUMO
The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues (2001) defined a provisional entity named refractory anemia with ringed sideroblasts associated to marked thrombocytosis (RARS-MT). Diagnosis of RARS-MT requires more than 15% of ringed sideroblasts in bone marrow aspirate and the existence of a thrombocytosis in blood, with a platelet count above 600 x 10(9)/L. Nevertheless, controversy exists regarding this platelet count "cut-off" value and, when RARS-MT was defined, the JAK2 mutation and its importance in the study of myeloproliferative disorders was unknown. We present the results of a Spanish retrospective multicentric study, which includes 76 cases of RARS with associated thrombocytosis (platelet count above 400 x 10(9)/L) at diagnosis (RARS-T), 36 of them with a platelet count above 600 x 10(9)/L. Our aim was to analyze their clinical, analytical and morphological characteristics, and to establish correlations with the JAK2 mutational status.
Assuntos
Anemia Refratária/genética , Anemia Refratária/patologia , Janus Quinase 2/genética , Mutação de Sentido Incorreto , Trombocitose/genética , Trombocitose/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Substituição de Aminoácidos , Anemia Refratária/sangue , Células da Medula Óssea/metabolismo , Células da Medula Óssea/patologia , Feminino , Humanos , Janus Quinase 2/metabolismo , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Estudos Retrospectivos , Trombocitose/sangueRESUMO
Agranulocytosis is one of the most serious side effects to drugs. From January 1991 to June 1996 were diagnosed 19 cases of agranulocytosis associated with drugs at our hospital (incidence rate: 9.4 over million hab. per year). The average age was 62 and 11 cases were women. The drugs most commonly involved were metamizol and ticlopidine. In 15 of the patients fever blew up and 16 presented some infectious location. In 9 of the cases some positive microbiological culture was obtained, gram-negative bacilli being the commonest. G-CSF was used in 13 of the patients, observing a quicker haematological recovery (5.7 days vs 9.1, p = 0.07), though without any difference in mortality, which was of 0%. All this leads to the following conclusions: a high incidence of agranulocytosis in our environment and the important role of metamizol and ticlopidine in its origin.
Assuntos
Agranulocitose/induzido quimicamente , Adulto , Idoso , Idoso de 80 Anos ou mais , Agranulocitose/tratamento farmacológico , Dipirona/efeitos adversos , Feminino , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Ticlopidina/efeitos adversosRESUMO
A child with AIDS is presented who developed L1-ALL while being treated for L3-AL. After achievement of complete remission of the former, he suffered a relapse with L3 morphology. Although the possibilities of a shift in both the morphology and the immunophenotype of ALL in relapse are well known, the rarity of L1-ALL in association with AIDS is stressed by the authors, along with the factors related with the morphologic and phenotypic changes shown by these patients.
Assuntos
Síndrome da Imunodeficiência Adquirida/complicações , Linfoma de Burkitt/complicações , Segunda Neoplasia Primária , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Síndrome da Imunodeficiência Adquirida/sangue , Síndrome da Imunodeficiência Adquirida/tratamento farmacológico , Síndrome da Imunodeficiência Adquirida/imunologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medula Óssea/patologia , Linfoma de Burkitt/sangue , Linfoma de Burkitt/patologia , Pré-Escolar , Evolução Fatal , Humanos , Imunofenotipagem , Masculino , Segunda Neoplasia Primária/sangue , Segunda Neoplasia Primária/patologia , Células-Tronco Neoplásicas/imunologia , Células-Tronco Neoplásicas/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/sangue , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Zidovudina/uso terapêuticoRESUMO
We report the case of a 2-year-old boy with hypereosinophilia who presented with fever, muscle pain and heart, CNS and skin involvement. He was treated with steroids with partial response. Six months after diagnosis of hypereosinophilia, several scalp nodules were surgically removed and Hypoderma bovis larvae were found, which led to the diagnosis of cutaneous myiasis. Following removal of the larvae, the clinical and hematological manifestations returned to normal.
Assuntos
Eosinofilia/etiologia , Hipodermose/complicações , Animais , Pré-Escolar , Dípteros , Humanos , MasculinoRESUMO
The authors reviewed 92 patients diagnosed as ALL. Age range was 1 to 12 years (x = 5 years), 50 males and 42 females. The patients were classified as high risk, intermediate and low risk according to the following prognostic factors: sex, age at diagnosis, visceromegaly, adenopathy, initial WBC, HB, immunoglobulins, and platelets, FAB morphological classification, and bone marrow response to initial therapy, immunological markers, CNS infiltration and mediastinal mass. The previous factors were analyzed individually and also the results obtained with each risk group according to the treatment applied.
Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras/classificação , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Prognóstico , Fatores de RiscoRESUMO
Megaloblastic anaemia is very rare in the first weeks of life and it is related to impaired metabolism of folic acid or vitamin B12. One of this disorders is the congenital transcobalamin II deficiency. The case of a three month old infant, with vomiting, diarrhoea and severe anaemia is presented. Both parents and the child had very low or undetectable levels of serum TC II, respectively. Using i.m. hydroxycobalamin at high doses, the clinical and laboratory responses have been satisfactory.