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1.
Presse Med ; 48(7-8 Pt 1): 842-849, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31447330

RESUMO

Hairy cell leukemia (HCL) is a well-defined entity. Proliferation with hair cells, morphological aspects of hairy cells are easy to identify. Hairy cells express markers CD11c, CD25, CD103 and CD123. In 80% of cases, a BRAFV600E mutation is highlighted. In the absence of a BRAFV600E mutation, the differential diagnosis with other hair cell proliferations can be difficult, especially with the variant form of hairy leukemia, diffuse lymphoma of the red pulp of the spleen or splenic lymphoma of the marginal zone. Purine analogues (PNA) with or without anti-CD20 antibodies remain the first-line reference treatment. In case of relapse or resistance to PNA, BRAF inhibitors, with or without MEK inhibitors, are proposed in patients with the mutation. In the absence of BRAFV600E mutation, moxetumomab-pasudotox represents an interesting alternative. A multidisciplinary discussion is always necessary. In complex cases, expert advice is desirable.


Assuntos
Leucemia de Células Pilosas , Linfócitos B/patologia , Proliferação de Células , Diagnóstico Diferencial , Humanos , Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/patologia , Leucemia de Células Pilosas/terapia , Linfoma de Zona Marginal Tipo Células B/patologia , Fatores de Risco , Baço/patologia , Neoplasias Esplênicas/patologia
2.
Int J Hematol ; 108(4): 416-422, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30043333

RESUMO

Hairy cell leukemia (HCL) is a rare B-cell lymphoid malignancy that is difficult to distinguish from other morphological variants. The frequency of HCL has not been determined accurately in Japan. Recent studies revealed that the BRAF V600E mutation is the causal genetic event in HCL. We assessed the BRAF mutation in Japanese patients with HCL and related diseases using the quenching probe (QP) method, a single-nucleotide polymorphism detection system, and evaluated the incidence rate of HCL among Japanese patients with chronic lymphocytic leukemia, and related diseases. We identified 18 cases (33.3%) harboring the BRAF mutation among 54 patients diagnosed with, or suspected of having HCL. Of BRAF V600E-positive patients, 7 were only detected using the QP method, not by direct sequencing, whereas 11 were positive using both tests. In a larger cohort of Japanese patients diagnosed with chronic lymphoid leukemia or related diseases, the frequency of HCL was 4%. Patients with the BRAF V600E mutation had a significantly higher frequency of neutropenia, thrombocytopenia, and elevated soluble interleukin-2 receptor and common B-cell surface markers than patients without the mutation. Our results confirm that BRAF V600E-positive HCL is a relatively rare disorder in the Japanese leukemia patient population.


Assuntos
Leucemia de Células Pilosas/genética , Mutação de Sentido Incorreto , Sondas de Ácido Nucleico , Polimorfismo de Nucleotídeo Único , Proteínas Proto-Oncogênicas B-raf/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Substituição de Aminoácidos , Grupo com Ancestrais do Continente Asiático , Feminino , Humanos , Japão/epidemiologia , Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/metabolismo , Masculino , Pessoa de Meia-Idade , Proteínas Proto-Oncogênicas B-raf/metabolismo
3.
Br J Haematol ; 175(3): 402-409, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27351754

RESUMO

Repeated therapy of hairy cell leukaemia (HCL) with treatments that have potential long-term toxicities has raised concerns regarding increased risk for younger patients. We compared clinical outcomes and disease complications in 63 patients with HCL aged ≤40 years at diagnosis with 268 patients >40 years treated at Memorial Sloan Kettering Cancer Center. The rate of complete remission following initial therapy was 87% and 83% (P = 0·71) and estimated 10-year overall survival was 100% and 82% (P = 0·25) in younger and older patients, respectively. Younger patients required therapy earlier and had a significantly shorter time between first and second therapy (median: 63 months vs. 145 months) (P = 0·008). Younger patients required significantly more lines of therapy during follow-up. The 10-year cumulative incidence of secondary malignancies in young and old patients was 0·205 and 0·287, respectively (P = 0·22). The incidence of secondary cancers in patients aged >40 years at diagnosis increased with the number of treatments for HCL (P = 0·018). These results highlight that young patients with HCL have shorter responses to treatment and require more lines of therapy to maintain disease control, while attaining similar long-term survival. This has implications in the design of future clinical trials given our findings that secondary malignancies increase with more chemotherapy exposure.


Assuntos
Leucemia de Células Pilosas/epidemiologia , Segunda Neoplasia Primária/epidemiologia , Segunda Neoplasia Primária/etiologia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Seguimentos , Humanos , Incidência , Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/terapia , Masculino , Pessoa de Meia-Idade , Segunda Neoplasia Primária/diagnóstico , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
4.
Best Pract Res Clin Haematol ; 28(4): 175-9, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26614895

RESUMO

Hairy cell leukaemia (HCL) is an orphan subtype of leukaemia which constitutes less than 2% of all leukaemia's, with an incidence of less than 1 per 100,000 persons per annum. Median age at presentation is 55 years and it is 3-4 times more frequent in males. It is also more frequently encountered in whites and less in Asians, Africans and Arabs. The epidemiologic data are multi-factorial and influenced by ethnicity and geographical factors. Other reported associations relate to some environmental exposures and possible occupational factors. Smoking appears to have an inverse correlation with the development of hairy cell leukaemia, while farming and exposure to pesticides, petroleum products, diesel and ionizing radiation have also been reported to be associated with an increased risk. National and international collaborative efforts are needed in order to undertake more extensive studies involving larger patient cohorts, aiming to determine the role of occupational and environmental risk factors in the development of this rare form of chronic leukaemia.


Assuntos
Criação de Animais Domésticos , Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/etiologia , Exposição Ocupacional/efeitos adversos , Adulto , Animais , Feminino , Interação Gene-Ambiente , Humanos , Leucemia de Células Pilosas/genética , Leucemia de Células Pilosas/patologia , Masculino , Praguicidas/efeitos adversos , Radiação Ionizante , Fatores Sexuais , Suécia/epidemiologia , Estados Unidos/epidemiologia
5.
Br J Haematol ; 171(1): 84-90, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26115047

RESUMO

Few studies have examined melanoma and non-melanoma skin cancer (NMSC) incidence rates after a diagnosis of hairy cell leukaemia (HCL). We assessed 267 HCL patients treated at Memorial Sloan Kettering Cancer Center (MSKCC) and Surveillance, Epidemiology and End Results (SEER) data for melanoma and NMSC incidence rates after HCL. Incidence data from MSKCC patients demonstrated a 10-year combined melanoma and NMSC skin cancer rate of 11·3%, melanoma 4·4% and NMSC 6·9%. Molecular analysis of skin cancers from MSKCC patients revealed activating RAS mutations in 3/9 patients, including one patient with melanoma. Of 4750 SEER patients with HCL, 55 (1·2%) had a subsequent diagnosis of melanoma. Standardized incidence ratios (SIRs) did not show that melanoma was more common in HCL patients versus the general population (SIR 1·3, 95% CI 0·78-2·03). Analysis of SEER HCL patients diagnosed before and after 1990 (approximately before and after purine analogue therapy was introduced) showed no evidence of an increased incidence after 1990. A better understanding of any potential association between HCL and skin cancer is highly relevant given ongoing trials using BRAF inhibitors, such as vemurafenib, for relapsed HCL, as RAS-mutant skin cancers could be paradoxically activated in these patients.


Assuntos
Leucemia de Células Pilosas/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adulto , Idoso , Feminino , Humanos , Incidência , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/enzimologia , Leucemia de Células Pilosas/genética , Masculino , Melanoma/tratamento farmacológico , Melanoma/enzimologia , Melanoma/epidemiologia , Melanoma/genética , Pessoa de Meia-Idade , Mutação , Inibidores de Proteínas Quinases/administração & dosagem , Proteínas Proto-Oncogênicas B-raf/genética , Proteínas Proto-Oncogênicas B-raf/metabolismo , Estudos Retrospectivos , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/enzimologia , Neoplasias Cutâneas/genética , Proteínas ras/genética , Proteínas ras/metabolismo
6.
Clin Lymphoma Myeloma Leuk ; 15(8): 484-8, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25882766

RESUMO

BACKGROUND: Several studies have reported excellent long-term overall survival (OS) of patients with hairy cell leukemia (HCL) without racial disparity. Studies in other cancers have demonstrated worse mortality among African American (AA) individuals. PATIENTS AND METHODS: We used the Surveillance, Epidemiology, and End Results 18 database to identify HCL patients diagnosed between 1978 and 2011. Kaplan-Meier curves were plotted to estimate OS. Univariate analysis using the life table method and multivariate Cox regression model were used to determine the independent effect of race on OS. RESULTS: The study population included 78% men and had a median age of 56 years. Race included 93% white, 3.5% Asian/Pacific Islander, and 3.5% AA. The 10-year OS was significantly less for AA as compared with white and Asian/Pacific Islander individuals (54% vs. 72% vs. 75%; P < .001). A Kaplan-Meier survival curve showed a significantly worse OS for AA versus other races (P < .001). In a multivariate analysis, AA race remained an independent predictor for a worse OS (hazard ratio 1.77; 95% confidence interval, 1.30-2.40; P < .001) after adjusting for age, sex, year of diagnosis, and marital status. CONCLUSION: In this population-based study, only half of AA patients but more than two-thirds of HCL patients from other racial groups were alive at 10 years. Such drastic racial differences in OS of HCL patients at the population level mandates further evaluation of the contributory biological, socioeconomic, health system, and other factors. Understanding and overcoming such racial disparities might close the racial differences in OS of this potentially curable disease.


Assuntos
Leucemia de Células Pilosas/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Grupos de Populações Continentais , Feminino , Humanos , Leucemia de Células Pilosas/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Estados Unidos , Adulto Jovem
7.
J Natl Cancer Inst Monogr ; 2014(48): 115-24, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25174032

RESUMO

BACKGROUND: Little is known about the etiology of hairy cell leukemia (HCL), a rare B-cell lymphoproliferative disorder with marked male predominance. Our aim was to identify key risk factors for HCL. METHODS: A pooled analysis of individual-level data for 154 histologically confirmed HCL cases and 8834 controls from five case-control studies, conducted in Europe and Australia, was undertaken. Age-, race and/or ethnicity-, sex-, and study-adjusted odds ratios (OR) and 95% confidence intervals (CI) were estimated using unconditional logistic regression. RESULTS: The usual patterns for age and sex in HCL were observed, with a median age of 55 years and sex ratio of 3.7 males to females. Cigarette smoking was inversely associated with HCL (OR = 0.51, 95% CI = 0.37 to 0.71) with dose-response relationships observed for duration, frequency, and lifetime cigarette smoking (P(trend) < .001). In contrast, occupation as a farmer was positively associated with HCL (OR = 2.34, 95% CI = 1.36 to 4.01), with a dose-response relationship observed for duration (OR = 1.82, 95% CI = 0.85 to 3.88 for ≤ 10 years vs never; and OR = 2.98, 95% CI = 1.50 to 5.93 for >10 years vs never; P(trend) = .025). Adult height was also positively associated with HCL (OR = 2.69, 95% CI = 1.39 to 5.29 for upper vs lower quartile of height). The observed associations remained consistent in multivariate analysis. CONCLUSIONS: Our observations of an increased risk of HCL from farming exposures and decreased risk from smoking exposures, independent of one another, support a multifactorial origin and an etiological specificity of HCL compared with other non-Hodgkin lymphoma subtypes. The positive association with height is a novel finding that needs replication.


Assuntos
Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/etiologia , Estilo de Vida , Exposição Ocupacional , Adulto , Idoso , Idoso de 80 Anos ou mais , Austrália/epidemiologia , Austrália/etnologia , Estudos de Casos e Controles , Comorbidade , Europa (Continente)/epidemiologia , Europa (Continente)/etnologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , América do Norte/epidemiologia , América do Norte/etnologia , Razão de Chances , Fatores de Risco , Fatores Sexuais , Adulto Jovem
8.
Ann Hematol ; 93(9): 1565-9, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24752417

RESUMO

Hairy cell leukemia (HCL) patients could have an excellent prognosis with adequate treatment. Treatments are not generally curative but are extremely effective in inducing long-lasting clinical remissions. An observational retrospective study was conducted on a single-center registry of 144 patients with a median follow-up of 11.5 years, focusing on long-lasting continuous first complete remissions (CR) wondering if patients can be cured only with front-line approach. CR for more than 5 years after first-line therapy were found in 22.2 % cases. The median duration of response was 9.8 years, while for relapsed patients, the first response had a median duration of 2.4 years. Three different subsets of long-lasting first CR were identified: 15 patients are between 5 and 10 years with a median duration of CR of 6.5 years; 7 patients are between 10 and 15 years with a median duration of CR of 12.3 years; and 10 patients present a follow-up superior to 15 years with a median duration of CR of 20.0 years. There is a need for continuous study in this field to better define the optimal therapeutic regimen and, in particular, the biological issues since at least 20-25 % of HCL patients can be cured with only one treatment.


Assuntos
Leucemia de Células Pilosas/terapia , Terapia Neoadjuvante , Adulto , Idoso , Cladribina/uso terapêutico , Feminino , Seguimentos , Humanos , Interferon-alfa/uso terapêutico , Leucemia de Células Pilosas/epidemiologia , Masculino , Pessoa de Meia-Idade , Pentostatina/uso terapêutico , Indução de Remissão , Estudos Retrospectivos , Esplenectomia/estatística & dados numéricos , Resultado do Tratamento
9.
Br J Haematol ; 166(3): 390-400, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24749839

RESUMO

A large, multicentre, retrospective survey of patients with hairy cell leukaemia (HCL) was conducted in France to determine the frequency of second malignancies and to analyse the long-term effects of the established purine nucleoside analogues (PNAs), cladribine and pentostatin. The survey retrospectively reviewed the medical history of patients and their immediate family, clinical and biological presentation at the time of HCL diagnosis, treatment choice, response to treatment, time to relapse and cause of death. Data were collected for 487 patients with HCL. Of the patients included in the survey, 18% (88/487) had a familial history of cancers, 8% (41/487) presented with malignancies before HCL diagnosis and 10% (48/487) developed second malignancies after HCL was diagnosed. An excess incidence of second malignancies was observed, with a standardized incidence ratio (SIR) of 1·86 (95% confidence interval (CI): 1·34-2·51), with no significant difference between PNAs. For second haematological malignancies alone, the SIR was markedly increased at 5·32 (95% CI: 2·90-8·92). This study highlights the high frequency of cancers in HCL patients and their family members. The frequency of second malignancies is notably increased, particularly for haematological malignancies. The respective role of pentostatin and cladribine in the development of second malignancies is debatable.


Assuntos
Leucemia de Células Pilosas/epidemiologia , Segunda Neoplasia Primária/epidemiologia , Segunda Neoplasia Primária/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Substituição de Medicamentos , Seguimentos , França/epidemiologia , Humanos , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/mortalidade , Pessoa de Meia-Idade , Segunda Neoplasia Primária/mortalidade , Segunda Neoplasia Primária/terapia , Estudos Retrospectivos , Esplenectomia , Resultado do Tratamento
10.
Leuk Lymphoma ; 53(6): 1169-73, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22035415

RESUMO

Treatment of hairy cell leukemia (HCL) with cladribine induces durable remissions. Common toxicities are myelosuppression and immunosuppression with low counts of CD4 + T cells. Skin rash (SR) is seldom described. We collected clinical and laboratory data of 35 patients with HCL treated in Hadassah between January 1999 and February 2010, in order to evaluate the frequency and characteristics of SR after treatment with cladribine. We found a high frequency of SR in our group of patients (18/35 patients, 51%), mostly related to febrile neutropenia and concomitant treatment with penicillins/trimethoprim-sulfamethoxazole (TMP-SMZ). The lymphocyte count was low in all patients with SR. We conclude that patients with HCL treated with cladribine have an increased rate of drug hypersensitivity, possibly due to T-cell imbalance induced by cladribine. Since TMP-SMZ and penicillins are related to SR in most cases and are important in the management of patients with HCL, a desensitization protocol should be considered. Rechallenge may be safe after immune reconstitution.


Assuntos
Cladribina/efeitos adversos , Cladribina/uso terapêutico , Exantema/induzido quimicamente , Exantema/epidemiologia , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Cladribina/administração & dosagem , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Combinação Trimetoprima e Sulfametoxazol/administração & dosagem , Combinação Trimetoprima e Sulfametoxazol/efeitos adversos , Adulto Jovem
11.
Acta Haematol ; 126(3): 186-93, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21846972

RESUMO

BACKGROUND: Classical hairy cell leukemia (HCL-C) and its variant (HCL-V) are rare chronic B-cell lymphoproliferative disorders. Only a few reports in Chinese patients are available. METHODS: We retrospectively reviewed 16 patients with HCL-C and HCL-V in Taiwan over a 17-year period. RESULTS: Eight were HCL-C and 8 were HCL-V. All HCL accounted for 0.7% of all adult leukemias. Compared to HCL-V, HCL-C was characterized by profound leukopenia, monocytopenia, thrombocytopenia and fewer circulating hairy cells. One HCL-C and 2 HCL-V patients had second malignancies. Seven HCL-C patients achieved hematological remission after splenectomy (n = 1) or 2-chlorodeoxyadenosine (n = 6). Of the 8 HCL-V patients, 6 received splenic irradiation. Only one achieved complete remission and another had partial remission; relapse or disease progression was noted 13.4 or 25.7 months later, respectively. Two of three HCL-V patients who underwent splenectomy had stable disease. All patients with HCL-C were alive while 3 with HCL-V expired. Compared to HCL-C, HCL-V had a significantly shorter leukemia-free survival. CONCLUSION: A relatively higher proportion of HCL-V in all HCL comparing to Westerners is observed. Second malignancies are common. With an inferior outcome and dismal response to most treatment, enrollment in a clinical trial should be considered for HCL-V.


Assuntos
Leucemia de Células Pilosas/patologia , Adulto , Idoso , Feminino , Humanos , Leucemia de Células Pilosas/classificação , Leucemia de Células Pilosas/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taiwan/epidemiologia
12.
Leuk Lymphoma ; 52 Suppl 2: 46-9, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21599605

RESUMO

Hairy cell leukemia is often reported as a disease of young males. The male predominance is strong, 4:1, but the median age in the Swedish national compulsory cancer registry is similar to that of follicular lymphoma, i.e. 62 years. The overall 6-year survival in the Swedish registry of patients diagnosed since 2000 is 80%, 93% of patients <60 years, and 68% of those >60 years. The yearly risk of secondary cancers is 1.75%. Cladribine is a prodrug which is selectively activated intracellularly. The intracellular initial half-life is 13 h and the terminal half-life is 30 h. Subcutaneous injection once daily is simple and effective due to 100% bioavailability and no local side effects from injection, and self-administration is easy. Long-term follow-up of Scandinavian patients treated with cladribine (mostly as subcutaneous injections) in the early 1990s shows a >80% 15-year survival from cladribine treatment in <60 years of age, but  <50% in older patients. Survival from diagnosis of these patients was similar for those previously treated and untreated.


Assuntos
Cladribina/farmacocinética , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/epidemiologia , Cladribina/uso terapêutico , Feminino , Seguimentos , Humanos , Masculino , Sistema de Registros , Análise de Sobrevida , Suécia
13.
Leuk Lymphoma ; 52 Suppl 2: 50-2, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21504285

RESUMO

The natural history of hairy cell leukemia (HCL) includes frequent and potentially life-threatening infections. Prior to the development of effective therapy, the incidence in patients followed for several years was as high as 60%, with infection as a prime cause of death in patients. Studies of the immune system of patients with HCL identified several potential reasons, including profound neutropenia and monocytopenia. In addition, treatment including chemotherapy and splenectomy further compromised the immune system. The success of new therapies has changed the frequency and severity of infections in patients with HCL. During the initial phase of treatment, however, infection risk remains high, with incidence ranging from 30 to 50%. Attempts to ameliorate the risk with growth factors in conjunction with treatment have not been successful, but lower doses of drugs and/or combination therapy have been tried with reported success. In the majority of patients, successful therapy results in normalization of the neutrophil count and marked reduction in the severity and frequency of infections. Interestingly, after purine nucleoside treatment, there is profound depression of CD4+ cells without development of the opportunistic infections seen with patients with human immunodeficiency virus (HIV). Studies to reduce morbidity and mortality should focus on initial induction regimens, as well as confirming the long-term benefit of treatment on risk of infection.


Assuntos
Leucemia de Células Pilosas/complicações , Infecções Oportunistas/etiologia , Humanos , Sistema Imunitário/efeitos dos fármacos , Imunossupressão/efeitos adversos , Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/terapia , Infecções Oportunistas/induzido quimicamente , Infecções Oportunistas/epidemiologia
14.
Dan Med Bull ; 57(12): A4216, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21122460

RESUMO

INTRODUCTION: Few population-based studies exist on incidence, risk of infection and mortality in hairy cell leukaemia (HCL). MATERIAL AND METHODS: We used population-based medical databases to identify 209 patients who were diagnosed with HCL in the period from January 1997 to August 2007 in Denmark. An age- and sex-matched comparison cohort of 2,090 persons was selected from the general population. We computed the incidence of HCL using demographic data. Hospitalizations with pneumonia and bacteraemia were determined from the Danish National Patient Registry. Cox regression analysis was used to estimate the relative risk (RR) of infection and mortality ratios (MRR) adjusting for age, sex and comorbidity. RESULTS: The HCL incidence rates were 1.97 (95% confidence interval 1.51-2.53) and 5.37 (4.57-6.28) per million person-years for women and men, respectively. During a median follow-up of 4.5 years, 48 HCL patients were hospitalized with pneumonia or bacteraemia. The adjusted RR of infection was 8.04 (4.99-12.95) the first year after diagnosis and 1.17 (0.71-1.94) for the remaining follow-up period. The adjusted MRRs were 4.26 (2.61-6.96) and 1.12 (0.75-1.65) the first year after diagnosis and the remaining follow-up period, respectively. CONCLUSION: In the second and subsequent years after HCL diagnosis, the risk of infection and mortality was similar to that of the general population.


Assuntos
Bacteriemia/epidemiologia , Leucemia de Células Pilosas/complicações , Leucemia de Células Pilosas/epidemiologia , Pneumonia/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos de Coortes , Dinamarca/epidemiologia , Feminino , Humanos , Incidência , Lactente , Leucemia de Células Pilosas/microbiologia , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Taxa de Sobrevida , Adulto Jovem
15.
Leuk Lymphoma ; 50 Suppl 1: 23-6, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19814694

RESUMO

Enormous progress has been made in the management of patients with hairy cell leukemia (HCL) over the past 50 years since this disease was initially described in 1958. The introduction of the two commonly used purine nucleoside analogs (pentostatin and cladribine, respectively) has independently changed the natural history of this rare malignancy. Both agents are equivalent in terms of response and long-term results. Advances in therapy are being further pursued with inclusion of monoclonal antibodies (e.g. rituximab) and other immunotherapeutic approaches. Patients with this disease now can live a near normal life expectancy, but the disease has not yet been cured. Clinical trials must continue to address the remaining unanswered questions.


Assuntos
Leucemia de Células Pilosas/tratamento farmacológico , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Seguimentos , Humanos , Interferon-alfa/uso terapêutico , Leucemia de Células Pilosas/epidemiologia , Pentostatina/uso terapêutico , Purinas/uso terapêutico , Indução de Remissão , Fatores de Tempo
16.
Med Monatsschr Pharm ; 32(7): 249-51, 2009 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-19731752

RESUMO

The hairy cell leukemia is a rare, low grade mature B-cell disease, a subtype of a Non Hodgkin-Lymphoma. The following article gives a short survey about the disease and the therapeutic options.


Assuntos
Antineoplásicos/uso terapêutico , Leucemia de Células Pilosas/patologia , Leucemia de Células Pilosas/terapia , Humanos , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/epidemiologia , Prognóstico
17.
Med Monatsschr Pharm ; 32(7): 252-60, 2009 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-19731753

RESUMO

The purine analogue cladribine is a highly active cytotoxic drug for the treatment of hairy cell leukemia. The substance is easy to use and patient friendly. After preparation of a special, sophisticated formulation by a pharmaceutical cytotoxic lab, the patient might even apply the drug himself. This review points out the potential of this non-patented pharmaceutical, besides practical advices.


Assuntos
Antineoplásicos/uso terapêutico , Cladribina/uso terapêutico , Leucemia de Células Pilosas/patologia , Leucemia de Células Pilosas/terapia , Animais , Cladribina/farmacologia , Humanos , Imunossupressores/farmacologia , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/epidemiologia , Prognóstico
18.
Eur J Haematol ; 82(3): 194-200, 2009 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-19077050

RESUMO

OBJECTIVE: Interferon-alpha (IFNalpha) was the first effective pharmacologic treatment of hairy cell leukemia (HCL). Since 1990 purine analogs replaced IFNalpha because of higher rates of complete remission and an invariable disease recurrence after cessation of IFNalpha. However, there are only limited data about long-term maintenance treatment with IFNalpha and none about dose finding in this phase. PATIENTS AND METHODS: Fifty-two consecutive patients treated at our institution for HCL are included in this retrospective analysis. Forty (77%) patients received IFNalpha and 35 patients continue on long-term IFNalpha maintenance therapy. The initial dose of IFNalpha was 3 Mio IU three times per week and was tapered 6 months after initiation to doses as low as 3 Mio IU/12 wk. Dose adaptation was performed by repeated measurement of soluble Interleukin 2 receptor (sIL2R) together with peripheral blood values. RESULTS: The median follow-up of patients in the long-term IFNalpha group was 13.6 +/- 7.5 yr. Long-term IFNalpha was in general well tolerated and only in six (17%) patients the treatment had to be changed to purine analogs in the long-term IFNalpha group because of side effects. There are no deaths directly related to HCL. CONCLUSIONS: IFNalpha is still an effective and well tolerated therapeutic option. By repeated measurements of sIL2R together with the peripheral blood values, IFNalpha doses can be tapered to the minimal effective dose. The advantages and disadvantage of IFNalpha in regards to the standard treatment in HCL patients are discussed.


Assuntos
Antineoplásicos/uso terapêutico , Interferon-alfa/uso terapêutico , Leucemia de Células Pilosas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/epidemiologia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Falha de Tratamento
19.
Occup Environ Med ; 66(5): 291-8, 2009 May.
Artigo em Inglês | MEDLINE | ID: mdl-19017688

RESUMO

OBJECTIVES: Investigating the relationship between occupational exposure to pesticides and the risk of lymphoid neoplasms (LNs) in men. METHODS: A hospital-based case-control study was conducted in six centres in France between 2000 and 2004. The cases were incident cases with a diagnosis of LN aged 18-75 years. During the same period, controls of the same age and sex as the cases were recruited in the same hospital, mainly in the orthopaedic and rheumatological departments. Exposures to pesticides were evaluated through specific interviews and case-by-case expert reviews. Four hundred and ninety-one cases (244 cases of non-Hodgkin's lymphoma (NHL), 87 of Hodgkin's lymphoma (HL), 104 of lymphoproliferative syndromes (LPSs) and 56 of multiple myeloma (MM) cases) and 456 controls were included in the analyses. The odds ratios (ORs) and 95% CI were estimated using unconditional logistic regressions. RESULTS: Positive associations between HL and occupational exposure to triazole fungicides and urea herbicides were observed (OR = 8.4 (2.2 to 32.4), 10.8 (2.4 to 48.1), respectively). Exposure to insecticides, fungicides and herbicides were linked to a threefold increase in MM risk (OR = 2.8 (1.2 to 6.5), 3.2 (1.4 to 7.2), 2.9 (1.3 to 6.5)). For LPS subtypes, associations restricted to hairy-cell leukaemia (HCL) were evidenced for exposure to organochlorine insecticides, phenoxy herbicides and triazine herbicides (OR = 4.9 (1.1 to 21.2), 4.1 (1.1 to 15.5), 5.1 (1.4 to 19.3)), although based on small numbers. Lastly, despite the increased ORs for organochlorine and organophosphate insecticides, carbamate fungicides and triazine herbicides, no significant associations were evidenced for NHL. CONCLUSIONS: The results, based on case-by-case expert review of occupation-specific questionnaires, support the hypothesis that occupational pesticide exposures may be involved in HL, MM and HCL and do not rule out a role in NHL. The analyses identified specific pesticides that deserve further investigation and the findings were consistent with those of previous studies.


Assuntos
Leucemia de Células Pilosas/epidemiologia , Linfoma/epidemiologia , Doenças Profissionais/epidemiologia , Exposição Ocupacional/estatística & dados numéricos , Praguicidas/toxicidade , Adolescente , Adulto , Idoso , Estudos de Casos e Controles , Emprego/estatística & dados numéricos , França/epidemiologia , Fungicidas Industriais/toxicidade , Herbicidas/toxicidade , Doença de Hodgkin/induzido quimicamente , Doença de Hodgkin/epidemiologia , Humanos , Inseticidas/toxicidade , Leucemia de Células Pilosas/induzido quimicamente , Linfoma/induzido quimicamente , Linfoma não Hodgkin/induzido quimicamente , Linfoma não Hodgkin/epidemiologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/induzido quimicamente , Mieloma Múltiplo/epidemiologia , Doenças Profissionais/induzido quimicamente , Exposição Ocupacional/efeitos adversos , Adulto Jovem
20.
Cancer Invest ; 26(8): 860-5, 2008 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-18798068

RESUMO

Hairy cell Leukemia (HCL) is a chronic lymphoproliferative disorder that was characterized in the late 1950s. HCL is defined, according to the WHO classification, as a mature (peripheral) B-cell neoplasm (1). HCL accounts for between 2-3% of all leukemia cases, with about 600 new cases diagnosed in the U.S. each year (1). HCL occurs more commonly in males, with an overall male to female ratio of approximately 4:1. The median age of onset is 52 years. This disease is seen more commonly in Caucasians and appears to be especially frequent in Ashkenazi Jewish males, with rare occurrence in persons of Asian and African descents (1). Hairy cells are distinct, clonal B cells arrested at a late stage of maturation. They are small B lymphoid cells that possess oval nuclei and abundant cytoplasm with characteristic micro-filamentous ("hairy") projections. They strongly express CD103, CD22, and CD11c (2). These cells typically infiltrate the bone marrow, the spleen, and to a lesser extent the liver, lymph nodes, and skin. Many patients present with splenomegaly and pancytopenia. Other clinical manifestations include recurrent opportunistic infections and vasculitis. Historically, HCL was considered uniformly fatal (2). However, recent treatment advances, using purine analogues such as Cladribine and Pentostatin, led to a significant improvement in prognosis with achievement of high response rates and durable remissions (2).


Assuntos
Leucemia de Células Pilosas , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Murinos , Antineoplásicos/uso terapêutico , Biomarcadores Tumorais/análise , Exame de Medula Óssea , Cladribina/uso terapêutico , Terapia Combinada , Diagnóstico Diferencial , Feminino , Previsões , Humanos , Interferon-alfa/uso terapêutico , Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/etiologia , Leucemia de Células Pilosas/cirurgia , Masculino , Pessoa de Meia-Idade , Pentostatina/uso terapêutico , Rituximab , Terapia de Salvação , Esplenomegalia/etiologia , Esplenomegalia/cirurgia , Resultado do Tratamento
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