RESUMO
Acute lymphoblastic leukemia (ALL) in the elderly is characterized by its ominous prognosis. On the other hand, imatinib has demonstrated remarkable, although transient, activity in relapsed and refractory Philadelphia-positive acute lymphoblastic leukemia (Ph+ ALL), which prompted us to assess the use of imatinib in previously untreated elderly patients. ALL patients aged 55 years or older were given steroids during 1 week. Ph+ve cases were then offered a chemotherapy-based induction followed by a consolidation phase with imatinib and steroids during 2 months. Patients in complete response (CR) after consolidation were given 10 maintenance blocks of alternating chemotherapy, including two additional 2-month blocks of imatinib. Thirty patients were included in this study and are compared with 21 historical controls. Out of 29 assessable patients, 21 (72%, confidence interval (CI): 53-87%) were in CR after induction chemotherapy vs 6/21 (29%, CI: 11-52%) in controls (P=0.003). Five additional CRs were obtained after salvage with imatinib and four after salvage with additional chemotherapy in the control group. Overall survival (OS) is 66% at 1 year vs 43% in the control group (P=0.005). The 1-year relapse-free survival is 58 vs 11% (P=0.0003). The use of imatinib in elderly patients with Ph+ ALL is very likely to improve outcome, including OS.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Metilprednisolona/uso terapêutico , Cromossomo Filadélfia , Piperazinas/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgia , Pirimidinas/uso terapêutico , Resultado do Tratamento , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Benzamidas , Intervalo Livre de Doença , Humanos , Mesilato de Imatinib , Metilprednisolona/administração & dosagem , Piperazinas/administração & dosagem , Pirimidinas/administração & dosagem , Transplante de Células-TroncoAssuntos
Antineoplásicos/uso terapêutico , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/tratamento farmacológico , Síndromes Mielodisplásicas/induzido quimicamente , Piperazinas/uso terapêutico , Pirimidinas/uso terapêutico , Adulto , Antineoplásicos/efeitos adversos , Benzamidas , Células Clonais/metabolismo , Células Clonais/patologia , Humanos , Mesilato de Imatinib , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologia , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/patologia , Masculino , Síndromes Mielodisplásicas/patologia , Segunda Neoplasia Primária/induzido quimicamente , Segunda Neoplasia Primária/patologia , Cromossomo Filadélfia , Piperazinas/efeitos adversos , Pirimidinas/efeitos adversosRESUMO
INTRODUCTION: Niemann-Pick disease is an autosomal recessive disorder due to partial or total deficit in sphingomyelinase. EXEGESIS: We report a case of type B Niemann-Pick disease revealed by pneumonia and splenomegaly associated with blue histiocyte syndrome. Investigations that were done 2 years prior to diagnosis had shown the existence of isolated chronic lipid pneumonia which is specific of overloading. CONCLUSION: Though diagnosis is based on biochemistry, the morphology of alveolar histiocytes after simple bronchoalveolar lavage is of value.
Assuntos
Pulmão/patologia , Doenças de Niemann-Pick/diagnóstico , Pneumonia/etiologia , Esplenomegalia/etiologia , Adulto , Líquido da Lavagem Broncoalveolar/citologia , Doença Crônica , Feminino , Humanos , Doenças de Niemann-Pick/complicações , Pneumonia/patologia , Esplenomegalia/diagnósticoAssuntos
Anti-Inflamatórios/uso terapêutico , Difosfonatos/uso terapêutico , Interferon-alfa/uso terapêutico , Mastocitose/tratamento farmacológico , Osteoporose/tratamento farmacológico , Anti-Inflamatórios/farmacologia , Densidade Óssea/efeitos dos fármacos , Difosfonatos/farmacologia , Humanos , Interferon-alfa/farmacologia , Masculino , PamidronatoRESUMO
We report the case of a 71-year old male patient with a chronic lymphoid leukaemia of 10 years' duration who abruptly suffered deterioration of this general condition and haematological disturbances suggesting worsening of this blood disease (Richter's syndrome). Blood cultures demonstrated a Campylobacter coli septicaemia. Treatment with antibiotics resulted in a return to the previous clinical and biological situation. The various visceral manifestation of the infection are discussed.