RESUMO
Correct definition of the level of risk of invasive fungal infections is the first step in improving the targeting of preventive strategies. We investigated the potential relationship between pre-hospitalization exposure to sources of fungi and the development of invasive fungal infections in adult patients with newly diagnosed acute myeloid leukemia after their first course of chemotherapy. From January 2010 to April 2012, all consecutive acute myeloid leukemia patients in 33 Italian centers were prospectively registered. Upon first admission, information about possible pre-chemotherapy risk factors and environmental exposure was collected. We recorded data regarding comorbid conditions, employment, hygienic habits, working and living environment, personal habits, hobbies, and pets. All invasive fungal infections occurring within 30 days after the first course of chemotherapy were recorded. Of the 1,192 patients enrolled in this study, 881 received intensive chemotherapy and were included in the present analysis. Of these, 214 developed an invasive fungal infection, including 77 proven/probable cases (8.7%). Of these 77 cases, 54 were proven/probable invasive mold infections (6.1%) and 23 were proven yeast infections (2.6%). Upon univariate analysis, a significant association was found between invasive mold infections and age, performance status, diabetes, chronic obstructive pulmonary disease, smoking, cocaine use, job, hobbies, and a recent house renovation. Higher body weight resulted in a reduced risk of invasive mold infections. Multivariate analysis confirmed the role of performance status, job, body weight, chronic obstructive pulmonary disease, and house renovation. In conclusion, several hospital-independent variables could potentially influence the onset of invasive mold infections in patients with acute myeloid leukemia. Investigation of these factors upon first admission may help to define a patient's risk category and improve targeted prophylactic strategies. (Clinicaltrial.gov: NCT01315925)
Assuntos
Antifúngicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Hospedeiro Imunocomprometido , Leucemia Mieloide Aguda/complicações , Micoses/etiologia , Adulto , Idoso , Estudos de Casos e Controles , Feminino , Seguimentos , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/imunologia , Masculino , Pessoa de Meia-Idade , Micoses/tratamento farmacológico , Estadiamento de Neoplasias , Prognóstico , Estudos Prospectivos , Fatores de Risco , Taxa de SobrevidaAssuntos
Leucemia Promielocítica Aguda , Micoses , Sistema de Registros , Adulto , Idoso , Feminino , Humanos , Itália/epidemiologia , Leucemia Promielocítica Aguda/complicações , Leucemia Promielocítica Aguda/epidemiologia , Masculino , Pessoa de Meia-Idade , Micoses/epidemiologia , Micoses/etiologia , Fatores de RiscoAssuntos
Cromossomos Humanos Par 8/ultraestrutura , Cromossomos Humanos Par 9/ultraestrutura , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Sistema de Sinalização das MAP Quinases , Proteína Quinase 1 Ativada por Mitógeno/fisiologia , Proteína Quinase 3 Ativada por Mitógeno/fisiologia , Proteínas de Fusão Oncogênica/fisiologia , Translocação Genética , Adulto , Cromossomos Humanos Par 8/genética , Cromossomos Humanos Par 9/genética , Eritropoese , Células-Tronco Hematopoéticas/patologia , Humanos , Cariotipagem , Leucemia Mielogênica Crônica BCR-ABL Positiva/sangue , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologia , Masculino , Células Neoplásicas Circulantes , Células-Tronco Neoplásicas/patologia , Proteínas de Fusão Oncogênica/genética , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Fator de Transcrição STAT3/análise , Fator de Transcrição STAT5/análiseRESUMO
BACKGROUND: To analyze the efficacy of antifungal prophylaxis (AFP) with posaconazole and itraconazole in a real-life setting of patients with acute myeloid leukemia (AML) during the first induction of remission. METHODS: From January 2010 to June 2011, all patients with newly diagnosed AML were consecutively registered and prospectively monitored at 30 Italian hematological centers. Our analysis focused on adult patients who received intensive chemotherapy and a mold-active AFP for at least 5 days. To determine the efficacy of prophylaxis, invasive fungal disease (IFD) incidence, IFD-attributable mortality, and overall survival were evaluated. RESULTS: In total, 515 patients were included in the present analysis. Posaconazole was the most frequently prescribed drug (260 patients [50%]) followed by fluconazole (148 [29%]) and itraconazole (93 [18%]). When comparing the groups taking posaconazole and itraconazole, there were no significant differences in the baseline clinical characteristics, whereas there were significant differences in the percentage of breakthrough IFDs (18.9% with posaconazole and 38.7% with itraconazole, P< .001). The same trend was observed when only proven/probable mold infections were considered (posaconazole, 2.7% vs itraconazole, 10.7%, P= .02). There were no significant differences in the IFD-associated mortality rate, while posaconazole prophylaxis had a significant impact on overall survival at day 90 (P= .002). CONCLUSIONS: During the last years, the use of posaconazole prophylaxis in high-risk patients has significantly increased. Although our study was not randomized, it demonstrates in a real-life setting that posaconazole prophylaxis confers an advantage in terms of both breakthrough IFDs and overall survival compared to itraconazole prophylaxis. CLINICAL TRIALS REGISTRATION: NCT01315925.
Assuntos
Antifúngicos/uso terapêutico , Itraconazol/uso terapêutico , Leucemia Mieloide Aguda/complicações , Micoses/prevenção & controle , Triazóis/uso terapêutico , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micoses/complicaçõesRESUMO
AIM: The main objective is to prospectively evaluate the therapeutic efficacy of minilaparocholecystectomy combined with videolaparoscopic view in cases of complicated gallstones where VLC was risky. MATERIAL OF STUDY: We carried out minilaparotomic video-aided cholecystectomy on 110 patients (32 males and 78 females) with preoperative diagnosis of intraabdominal adhesions or biliary severe inflammation. RESULT: No significant intra or postoperative complications was reported and in all cases pain was never greater than that reported after VLC. In all these cases the anesthetists reported an easier intra-operative management of the vital parameters than with VLC procedures. DISCUSSION: Minilaparocholecystectomy appears a type of alternative procedure able to combine mini-invasiveness with as low a number as possible of intra- and post-operative complications, in cases where VLC have risk. No significant postoperative pain was reported, and in any case pain was never greater than that reported after VLC. Recovery times were similar to those after VLC; patients were able to return to their normal social and working life within a mean 3 days. The procedure carried out by us is a low-cost one: it does not require disposable instruments From the esthetic viewpoint, video-aided minicholecystectomy minimal scars in our cases, wound ranged from 4 to 6 cm. in length. CONCLUSIONS: In patients in whom VLC have risks, video-aided minilaparocholecystectomy represents an easy-to-perform and low-cost alternative. VMC can also be proposed as a procedure of choice in cases of complicated gallstones instead of the traditional open cholecystectomy technique.
Assuntos
Colecistectomia Laparoscópica/métodos , Cirurgia Vídeoassistida , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
BACKGROUND: The aim of this study was to evaluate prognostic factors, treatments and outcome of invasive aspergillosis in patients with acute myeloid leukemia based on data collected in a registry. DESIGN AND METHODS: The registry, which was activated in 2004 and closed in 2007, collected data on patients with acute myeloid leukemia, admitted to 21 hematologic divisions in tertiary care centers or university hospitals in Italy, who developed proven or probable invasive aspergillosis. RESULTS: One hundred and forty cases of invasive aspergillosis were collected, with most cases occurring during the period of post-induction aplasia, the highest risk phase in acute myeloid leukemia. The mortality rate attributable to invasive aspergillosis was 27%, confirming previous reports of a downward trend in this rate. Univariate and multivariate analyses revealed that the stage of acute myeloid leukemia and the duration of, and recovery from, neutropenia were independent prognostic factors. We analyzed outcomes after treatment with the three most frequently used drugs (liposomal amphotericin B, caspofungin, voriconazole). No differences emerged in survival at day 120 or in the overall response rate which was 71%, ranging from 61% with caspofungin to 84% with voriconazole. CONCLUSIONS: Our series confirms the downward trend in mortality rates reported in previous series, with all new drugs providing similar survival and response rates. Recovery from neutropenia and disease stage are crucial prognostic factors. Efficacious antifungal drugs bridge the period of maximum risk due to poor hematologic and immunological reconstitution.
Assuntos
Antifúngicos/uso terapêutico , Aspergilose/etiologia , Leucemia Mieloide Aguda/complicações , Adolescente , Adulto , Idoso , Anfotericina B/uso terapêutico , Aspergilose/tratamento farmacológico , Aspergilose/mortalidade , Aspergillus/fisiologia , Caspofungina , Equinocandinas/uso terapêutico , Feminino , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/microbiologia , Lipopeptídeos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Pirimidinas/uso terapêutico , Sistema de Registros , Taxa de Sobrevida , Resultado do Tratamento , Triazóis/uso terapêutico , Voriconazol , Adulto JovemRESUMO
The aim of the study is to evaluate clinical features, treatments and outcome of patients with systemic mast cell disease (MCD) who arrived to the attention of hematologists. A retrospective study was conducted over 1995-2006 in patients admitted in 18 Italian hematological divisions. Twenty-four cases of advanced MCD were collected: 12 aggressive SM (50%), 8 mast cell leukemia (33%), 4 SM with associated clonal non-mast cell-lineage hematologic disease (17%). Spleen and liver were the principal extramedullary organ involved. The c-kit point mutation D816V was found in 13/18 patients in which molecular biology studies were performed (72%). Treatments were very heterogeneous: on the whole Imatinib was administered in 17 patients, alpha-Interferon in 8, 2-CdA in 3; 2 patients underwent allogeneic hematopoietic stem cell transplantation. The overall response rate to Imatinib, the most frequently employed drugs, was of 29%, registering one complete remission and four partial remission; all responsive patients did not present D816V c-kit mutation. Overall three patients (12%) died for progression of disease. We conclude that MCD is characterized by severe mediator-related symptoms but with a moderate mortality rate. D816V c-kit mutation is frequent and associated with resistance against Imatinib. Because of the rarity of these forms, an effective standard of care is lacking. More data are needed to find new and successful therapeutic strategies.
Assuntos
Mastocitose/genética , Mastocitose/mortalidade , Mutação Puntual , Proteínas Proto-Oncogênicas c-kit/genética , Adulto , Idoso , Substituição de Aminoácidos , Antineoplásicos/administração & dosagem , Antivirais/administração & dosagem , Benzamidas , Intervalo Livre de Doença , Feminino , Transplante de Células-Tronco Hematopoéticas , Humanos , Mesilato de Imatinib , Interferon-alfa/administração & dosagem , Itália , Fígado/metabolismo , Masculino , Mastocitose/metabolismo , Mastocitose/terapia , Pessoa de Meia-Idade , Piperazinas/administração & dosagem , Proteínas Proto-Oncogênicas c-kit/metabolismo , Pirimidinas/administração & dosagem , Indução de Remissão , Estudos Retrospectivos , Baço/metabolismo , Taxa de Sobrevida , Transplante HomólogoRESUMO
This randomized phase III clinical trial explored the efficacy of DaunoXome (DNX) versus Daunorubicin (DNR) in acute myeloid leukaemia (AML) patients aged >60 years. Three hundred and one AML patients were randomized to receive DNR (45 mg/m(2) days 1-3) or DNX (80 mg/m(2) days 1-3) plus cytarabine (AraC; 100 mg/m(2) days 1-7). Patients in complete remission (CR) received a course of the same drugs as consolidation and then were randomized for maintenance with AraC+ all trans retinoic acid or no further treatment. Among 153 patients in the DNR arm, 78 (51.0%) achieved CR, 55 (35.9%) were resistant and 20 (13.1%) died during induction. Among 148 patients in the DNX arm, 73 (49.3%) achieved CR, 47 (31.8%) were resistant and 28 (18.9%) died during induction. Univariate analysis showed no difference as to induction results. After CR, DNX showed a higher incidence of early deaths (12.5% vs. 2.6% at 6 months, P = 0.053) but a lower incidence of relapse beyond 6 months (59% vs. 78% at 24 months, P = 0.064), with a cross in overall survival (OS) and disease-free survival (DFS) curves and a later advantage for DNX arm after 12 months from diagnosis. DNX seems to improve OS and DFS in the long-term follow-up, because of a reduction in late relapses.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Daunorrubicina/administração & dosagem , Leucemia Mieloide Aguda/tratamento farmacológico , Idoso , Daunorrubicina/uso terapêutico , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Leucemia Mieloide Aguda/mortalidade , Lipossomos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Recidiva , Indução de Remissão/métodos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Most of the information about the genetic composition of parathyroid tumors has been obtained by comparative genomic hybridization (CGH) and loss of heterozygosity (LOH) studies, whereas only few conventional cytogenetic investigation results are available. We have performed cytogenetic analysis of short-term cultures from 3 parathyroid adenoma tissue samples. Two cases showed a normal karyotype in all the metaphases obtained from independent primary cultures. In one case 5 metaphases (in a total of 25) from 2 independent cultures showed a nonrandom translocation t(4;13)(q21;q14), which was therefore accepted as clonal. To our knowledge this is the second clonal translocation described in this tumor type. Further conventional cytogenetic analysis of more parathyroid tumor specimens would be necessary to identify other specific abnormalities and the involved genes with a potential important role in the diagnosis, prognosis and pathogenesis of parathyroid tumors.
Assuntos
Adenoma/genética , Aberrações Cromossômicas , Cromossomos Humanos Par 13/genética , Cromossomos Humanos Par 4/genética , Neoplasias das Paratireoides/genética , Translocação Genética , Análise Citogenética , Humanos , CariotipagemRESUMO
BACKGROUND AND OBJECTIVES: The aim of this study was to evaluate the incidence and outcome of invasive fungal infections (IFI) in patients with hematologic malignancies. DESIGN AND METHODS: This was a retrospective cohort study of patients admitted between 1999 and 2003 to 18 hematology wards in Italy. Each participating center provided information on all patients with newly diagnosed hematologic malignancies admitted during the survery period and on all episodes of IFI experienced by these patients. RESULTS: The cohort was formed of 11,802 patients with hematologic malignacies: acute leukemia (myeloid 3012, lymphoid 1173), chronic leukemia (myeloid 596, lymphoid 1104), lymphoma (Hodgkin's 844, non-Hodgkin's 3457), or multiple myeloma (1616). There were 538 proven or probable IFI (4.6%); 373 (69%) occurred in patients with acute myeloid leukemia. Over half (346/538) were caused by molds (2.9%), in most cases Aspergillus spp. (310/346). The 192 yeast infections (1.6%) included 175 cases of candidemia. Overall and IFI-attributable mortality rates were 2% (209/11802) and 39% (209/538), respectively. The highest IFI-attributable mortality rates were associated with zygomycosis (64%) followed by fusariosis (53%), aspergillosis (42%), and candidemia (33%). INTERPRETATION AND CONCLUSIONS: Patients with hematologic malignancies are currently at higher risk of IFI caused by molds than by yeasts, and the incidence of IFI is highest among patients with acute myeloid leukemia. Aspergillus spp are still the most common pathogens, followed by Candida spp. Other agents are rare. The attributable mortality rate for aspergillosis has dropped from 60-70% to approximately 40%. Candidemia-related mortality remains within the 30-40% range reported in literature although the incidence has decreased.
Assuntos
Neoplasias Hematológicas/imunologia , Micoses/epidemiologia , Candidíase/epidemiologia , Estudos de Coortes , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/mortalidade , Humanos , Incidência , Micoses/classificação , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND AND OBJECTIVES: Cryptococcosis is an important cause of morbidity and death in immunocompromised patients. The aim of this study was to evaluate clinical and laboratory characteristics, and outcome of patients with cryptococcosis complicating hematologic diseases. DESIGN AND METHODS: This was a retrospective study, conducted over a ten-year period (1993-2002) in 21 hematology divisions, in tertiary care or university hospitals. RESULTS: This study evaluated 17 patients with hematologic diseases who developed cryptococcosis. Possible risk factors recognized before the onset of the infection were: administration of steroids for the underlying malignancy (6 patients), diabetes mellitus (4 patients), cutaneous lesions (2 patients) and autoimmune disease, hepatic cirrhosis, chronic renal failure and exposure to pigeons (1 patient each). Five patients received prophylaxis, consisting of fluconazole in 2 cases. Fever, neurological and respiratory signs developed according to the primary sites of infection (5 blood, 5 central nervous system, 4 lung, and 1 each in gut, skin and mouth). Diagnosis was made by positive microbiological culture, antigen detection in serum or cerebrospinal fluid, or polymerase chain reaction. All patients started specific treatment (fluconazole, 7 patients; amphotericin-B deoxycolate or liposomal amphotericin-B, 10 patients). Two patients died from cryptococcosis within 30 days after diagnosis. INTERPRETATION AND CONCLUSIONS: Cryptococcosis in patients with hematologic malignancies is a rare complication. In neutropenic patients, it is less fatal than other fungal infections (i.e. aspergillosis or candidemia). Specific treatment, started promptly, positively influences the outcome.
Assuntos
Criptococose/complicações , Neoplasias Hematológicas/microbiologia , Adolescente , Adulto , Idoso , Antifúngicos/uso terapêutico , Criptococose/diagnóstico , Criptococose/tratamento farmacológico , Feminino , Fluconazol/uso terapêutico , Neoplasias Hematológicas/imunologia , Humanos , Hospedeiro Imunocomprometido , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Esteroides/efeitos adversosRESUMO
Toxoplasmosis is one of the most common parasitic infections in humans, but in most cases it does not cause serious illness; this protozoan can nevertheless cause devastating disease in immunocompromised hosts such as HIV-positive individuals. Only rarely is toxoplasmosis documented in hematological patients, and among them, those who undergo a transplant procedure are more frequently affected. In a retrospective multicenter survey, we collected data on six cases of toxoplasmosis in hematological patients. In the majority of cases, patients had undergone transplant procedures (five had undergone autologous or allogeneic transplantation). This complication needed special care in diagnosis, usually based on serology, neuroradiology, and PCR examination. However, in our experience the appropriate therapeutic approach was successful in the majority of cases.
Assuntos
Leucemia/complicações , Leucemia/parasitologia , Linfoma/complicações , Linfoma/parasitologia , Toxoplasma/fisiologia , Toxoplasmose/complicações , Toxoplasmose/parasitologia , Adolescente , Adulto , Idoso , Animais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Toxoplasmose/fisiopatologia , Toxoplasmose/prevenção & controleRESUMO
In this study, we describe an extremely rare case of co-existence of a Philadelphia chromosome positive acute megakaryoblastic and B-lymphoblastic mixed blast crisis of chronic myeloid leukemia with chronic lymphocytic leukemia. A morphological, immunophenotypical and cytogenetic study has been performed to characterize the case and in order to identify the origin of two disorders. After the failure of the conventional therapy, the patient was treated with Imatinib with a complete hematological and cytogenetic response and a marked reduction of bone marrow fibrosis.
Assuntos
Crise Blástica , Linfoma de Burkitt , Leucemia Linfocítica Crônica de Células B , Leucemia Megacarioblástica Aguda , Leucemia Mielogênica Crônica BCR-ABL Positiva , Neoplasias Primárias Múltiplas , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Benzamidas , Crise Blástica/genética , Crise Blástica/patologia , Linfoma de Burkitt/genética , Linfoma de Burkitt/patologia , Citarabina/administração & dosagem , Daunorrubicina/administração & dosagem , Feminino , Humanos , Mesilato de Imatinib , Imunofenotipagem , Cariotipagem , Leucemia Linfocítica Crônica de Células B/genética , Leucemia Linfocítica Crônica de Células B/patologia , Leucemia Megacarioblástica Aguda/genética , Leucemia Megacarioblástica Aguda/patologia , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologia , Neoplasias Primárias Múltiplas/genética , Neoplasias Primárias Múltiplas/patologia , Piperazinas/uso terapêutico , Pirimidinas/uso terapêutico , Indução de RemissãoAssuntos
Mieloma Múltiplo/patologia , Neoplasias Pleurais/secundário , Neoplasias Testiculares/secundário , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Evolução Fatal , Humanos , Imunoglobulina A/análise , Imunoglobulina G/análise , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/tratamento farmacológico , Mieloma Múltiplo/imunologiaRESUMO
Patients with multiple myeloma (MM) have increased bone marrow (BM) angiogenesis; however, the proangiogenic properties of myeloma cells and the mechanisms of MM-induced angiogenesis are not completely clarified. The angiopoietin system has been identified as critical in the regulation of vessel formation. In this study we have demonstrated that myeloma cells express several proangiogenic factors, and, in particular, we found that angiopoietin-1 (Ang-1), but not its antagonist Ang-2, was expressed by several human myeloma cell lines (HMCLs) at the mRNA and the protein levels. In a transwell coculture system, we observed that myeloma cells up-regulated the Ang-1 receptor Tie2 in human BM endothelial cells. Moreover, in an experimental model of angiogenesis, the conditioned medium of HMCLs significantly stimulated vessel formation compared with control or vascular endothelial growth factor (VEGF) treatment. The presence of anti-Tie2 blocking antibody completely blunted the proangiogenic effect of XG-6. Finally, our in vitro results were supported by the in vivo finding of Ang-1, but not Ang-2, mRNA and protein expression in purified MM cells obtained from approximately 47% of patients and by high BM angiogenesis in patients with MM positive for Ang-1, suggesting that the angiopoietin system could be involved, at least in part, in MM-induced angiogenesis.
Assuntos
Indutores da Angiogênese/fisiologia , Glicoproteínas de Membrana/fisiologia , Mieloma Múltiplo/metabolismo , Proteínas de Neoplasias/fisiologia , Neovascularização Patológica/metabolismo , Proteínas Proto-Oncogênicas , Adulto , Indutores da Angiogênese/análise , Indutores da Angiogênese/biossíntese , Indutores da Angiogênese/genética , Angiopoietina-1 , Angiopoietina-2 , Anticorpos Monoclonais/imunologia , Anticorpos Monoclonais/farmacologia , Células da Medula Óssea/metabolismo , Técnicas de Cocultura , Meios de Cultivo Condicionados/farmacologia , Fatores de Crescimento Endotelial/farmacologia , Endotélio/metabolismo , Regulação Neoplásica da Expressão Gênica , Humanos , Peptídeos e Proteínas de Sinalização Intercelular/farmacologia , Leucemia Plasmocitária/metabolismo , Leucemia Plasmocitária/patologia , Linfocinas/farmacologia , Glicoproteínas de Membrana/biossíntese , Glicoproteínas de Membrana/genética , Pessoa de Meia-Idade , Mieloma Múltiplo/irrigação sanguínea , Mieloma Múltiplo/genética , Mieloma Múltiplo/patologia , Proteínas de Neoplasias/antagonistas & inibidores , Proteínas de Neoplasias/biossíntese , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/imunologia , Neovascularização Patológica/genética , RNA Mensageiro/biossíntese , RNA Neoplásico/biossíntese , Receptor TIE-2 , Fator A de Crescimento do Endotélio Vascular , Fatores de Crescimento do Endotélio VascularRESUMO
A retrospective survey was conducted over a 10-year period (1990-99) among 52 haematology divisions in order to evaluate the clinical and laboratory characteristics and outcome of patients with proven Pneumocystis carinii pneumonia (PCP) complicating haematological diseases. The study included 55 patients (18 with non-Hodgkin's lymphoma, 10 with acute lymphoblastic leukaemia, eight with acute myeloid leukaemia, five with chronic myeloid leukaemia, four with chronic lymphocytic leukaemia, four with multiple myeloma, three with myelodysplastic syndrome, two with myelofibrosis and one with thalassemia) who developed PCP. Among these, 18 (33%) underwent stem cell transplantation; only two received an oral prophylaxis with trimethroprim/sulphamethoxazole. Twelve patients (22%) developed PCP despite protective isolation in a laminar airflow room. The most frequent symptoms were: fever (86%), dyspnoea (78%), non-productive cough (71%), thoracic pain (14%) and chills (5%); a severe hypoxaemia was present in 39 patients (71%). Chest radiography or computerized tomography showed interstitial infiltrates in 34 patients (62%), alveolar infiltrates in 12 patients (22%), and alveolar-interstitial infiltrates in nine patients (16%). Bronchoalveolar lavage was diagnostic in 47/48 patients, induced sputum in 9/18 patients and lung biopsy in 3/8 patients. The diagnosis was made in two patients at autopsy. All patients except one started a specific treatment (52 patients trimethroprim/sulphamethoxazole, one pentamidine and one dapsone). Sixteen patients (29%) died of PCP within 30 d of diagnosis. Multivariate analysis showed that prolonged steroid treatment (P < 0.006) and a radiological picture of diffuse lung involvement (P < 0.003) were negative diagnostic factors.