RESUMO
Cerebro-retinal microangiopathy with calcifications and cysts (CRMCC) or Coats plus syndrome is a pleiotropic disorder affecting the eyes, brain, bone and gastrointestinal tract. Its primary pathogenesis involves small vessel obliterative microangiopathy. Recently, autosomal recessively inherited mutations in CTC1 have been reported in CRMCC patients. We herein report an adolescent referred to our hospital following new seizures in a context of an undefined multisystem disorder. Cerebral imaging disclosed asymmetrical leukopathy, intracranial calcifications and cysts. In addition, he presented other typical CRMCC features i.e. a history of intrauterine growth retardation, skeletal demineralization and osteopenia, bilateral exudative vitreo-retinopathy reminiscent of Coats disease, recurrent gastrointestinal hemorrhages secondary to watermelon stomach and variceal bleeding of the esophagus due to idiopathic portal hypertension and telangiectatic and angiodysplasic changes in the small intestine and colon, and anemia due to recurrent bleeding and bone marrow abnormalities. The patient was diagnosed with Coats plus syndrome. CTC1 gene screening confirmed the diagnosis with the identification of heterozygous deleterious mutations. CRMCC due to CTC1 mutations has a broad clinical expressivity. Our case report illustrates the main possible associated phenotypes and their complications, demonstrating the need for a careful etiological search in order to initiate appropriate therapeutic and preventive measures.
Assuntos
Ataxia/genética , Neoplasias Encefálicas/genética , Calcinose/genética , Cistos do Sistema Nervoso Central/genética , Leucoencefalopatias/genética , Espasticidade Muscular/genética , Doenças Retinianas/genética , Convulsões/genética , Proteínas de Ligação a Telômeros/genética , Adolescente , Ataxia/fisiopatologia , Neoplasias Encefálicas/fisiopatologia , Calcinose/fisiopatologia , Cistos do Sistema Nervoso Central/fisiopatologia , Retardo do Crescimento Fetal/genética , Hemorragia Gastrointestinal/etiologia , Genes Recessivos/genética , Humanos , Leucoencefalopatias/fisiopatologia , Imageamento por Ressonância Magnética , Masculino , Espasticidade Muscular/fisiopatologia , Mutação/genética , Doenças Retinianas/fisiopatologia , Convulsões/fisiopatologiaRESUMO
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease characterized by malignant proliferation of a contingent blastic plasmacytoid dendritic cell. This rare entity is recognized mostly by cutaneous spreading, or not having a leukaemic component. The prognosis is very poor. OBJECTIVES: To study a large cohort of 90 patients with BPDCN, to define additional symptoms to form a correct diagnosis earlier, and to manage such patients accordingly. METHODS: We retrospectively reviewed BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database between November 1995 and January 2012. Ninety patients were studied. Demographic data, clinical presentation, initial staging and outcome were recorded. RESULTS: The group contained 62 male and 28 female patients (sex ratio 2·2). Their ages ranged from 8 to 103 years at the time of diagnosis (mean 67·2 years). Three major different clinical presentations were identified. Sixty-six patients (73%) presented with nodular lesions only, 11 patients (12%) with 'bruise-like' patches and 13 (14%) with disseminated lesions (patches and nodules). Mucosal lesions were seen in five patients (6%). The median survival in patients with BPDCN was 12 months. CONCLUSIONS: We here distinguish three different clinical presentations of BPDCN. A nodular pattern is a more common feature than the originally reported 'bruise-like' pattern. Despite the fact that BPDCN may initially appear as a localized skin tumour, aggressive management including allogeneic bone marrow transplantation should be considered immediately, as it is currently the only option associated with long-term survival.
Assuntos
Células Dendríticas/patologia , Neoplasias Hematológicas/patologia , Neoplasias Bucais/patologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Diagnóstico Tardio , Feminino , Neoplasias Hematológicas/mortalidade , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Mucosa Bucal/patologia , Neoplasias Bucais/mortalidade , Estudos Retrospectivos , Neoplasias Cutâneas/mortalidade , Adulto JovemRESUMO
Symptomatic nervous system leukemic infiltration is rarely observed in CLL. Various clinical manifestations including headache, confusion, cranial nerve palsies, focal central deficits and peripheral neuropathies have been seldom reported, occurring in less than 1% of patients. We report herein 2 CLL patients with unusual clinical presentations of nervous system invasion. They presented multiple progressive peripheral deficits due to meningoradiculitis. In both, CSF immunophenotyping analysis identified a majority of T cells (>90%), and less than 10% of B-CLL cells expressing CD5, CD19 and CD20. Our analyses revealed the transformation of CLL into an aggressive B-cell lymphoma in one case (Richter's syndrome). A post mortem study showed massive infiltration of cranial nerves and spinal roots by large B lymphomatous cells. In the other case, CNS oriented chemotherapy led to remission and total neurological recovery. In practice, the etiological diagnosis of neurological deficits in CLL patients is difficult. CSF analysis may be useful, requiring viral PCR, repeated cytological studies and immunophenotyping analysis. Although rare, leptomeningeal leukemic localization has to be discussed, even in the absence of overt Richter syndrome, and may require an early therapeutic test.
Assuntos
Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/patologia , Meningite/etiologia , Meningite/patologia , Radiculopatia/etiologia , Radiculopatia/patologia , Idoso , Idoso de 80 Anos ou mais , Nervos Cranianos/patologia , Evolução Fatal , Humanos , Infiltração Leucêmica/patologia , Masculino , Raízes Nervosas Espinhais/patologiaRESUMO
OBJECTIVE: Maternal and fetal risk is often high during pregnancy in sickle cell disease. Our objective was to evaluate the benefits of a transfusion program adapted to each pregnant patient, either by red cell transfusion or by automated red cell exchange, in sickle cell patients with a history of serious obstetrical and/or sickling complications. STUDY DESIGN: We managed 18 pregnancies in 14 patients (12 SS, 1 SC, 1 S/b-thalassemia), seven of whom had a history of one or more pregnancies, with severe maternofetal complications in nine out of 10 cases. The other seven patients were pregnant for the first time and were in care because of a history of severe sickling complications. The aim was to achieve a proportion of abnormal hemoglobin (hemoglobin S or S+C) below 50% and a hemoglobin level between 9 and 11 g/dL. The choice between transfusion and red cell exchange was made in the light of the hemoglobin level. Red cell exchange was done using a Fresenius Com. Tec blood cell separator. Patients had red cell exchange in 10 cases, and transfusions in five cases. In three cases, patients had successive transfusions and red cell exchange. RESULTS: No serious maternal complication was observed. No fetal or perinatal death occurred. In one case, delivery was induced at 36 weeks of gestation because of fetal distress and hypotrophy. CONCLUSIONS: Our study suggests that women with severe sickle cell disease, even if they have a serious obstetrical history, can carry their pregnancy to term, without major obstetric complications, through a combination of early management by a multidisciplinary team and a suitable policy of prophylactic transfusion or automated red cell exchange.
Assuntos
Anemia Falciforme/terapia , Transfusão de Eritrócitos , Transfusão Total , Complicações Hematológicas na Gravidez/terapia , Adulto , Automação , Feminino , Hemoglobina Falciforme/análise , Humanos , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Resultado do TratamentoRESUMO
After failure of erythropoiesis-stimulating agents (ESAs), lenalidomide (LEN) yields red blood cell (RBC) transfusion independence (TI) in 20-30% of lower-risk non-del5q myelodysplastic syndrome (MDS). Several observations suggest an additive effect of ESA and LEN in this situation. We performed a randomized phase III study in 131 RBC transfusion-dependent (TD, median transfusion requirement six RBC units per 8 weeks) lower-risk ESA-refractory non-del5q MDS. Patients received LEN alone, 10 mg per day, 21 days per 4 weeks (L arm) or LEN (same schedule) + erythropoietin (EPO) beta, 60,000 U per week (LE arm). In an intent-to-treat (ITT) analysis, erythroid response (HI-E, IWG 2006 criteria) after four treatment cycles (primary end point) was 23.1% (95% CI 13.5-35.2) in the L arm and 39.4% (95% CI 27.6-52.2) in the LE arm (P=0.044), while RBC-TI was reached in 13.8 and 24.2% of the patients in the L and LE arms, respectively (P=0.13). Median response duration was 18.1 and 15.1 months in the L and LE arms, respectively (P=0.47). Side effects were moderate and similar in the two arms. Low baseline serum EPO level and a G polymorphism of CRBN gene predicted HI-E. Combining LEN and EPO significantly improves erythroid response over LEN alone in lower-risk non-del5q MDS patients with anemia resistant to ESA.
Assuntos
Transfusão de Sangue , Deleção Cromossômica , Cromossomos Humanos Par 5/genética , Eritropoetina/uso terapêutico , Síndromes Mielodisplásicas/tratamento farmacológico , Talidomida/análogos & derivados , Idoso , Anemia/prevenção & controle , Inibidores da Angiogênese/uso terapêutico , Quimioterapia Combinada , Feminino , Seguimentos , Humanos , Lenalidomida , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/patologia , Estadiamento de Neoplasias , Prognóstico , Estudos Prospectivos , Fatores de Risco , Talidomida/uso terapêuticoRESUMO
Thirty-nine patients with untreated acute promyelocytic leukemia (APL) were randomly allocated to receive rubidazone (zorubicin) 200 mg/m2/d, days 1 to 4 plus cytarabine (Ara C) 200 mg/m2/d, days 1 to 7 (arm A, 21 patients), or amsacrine (Amsa) 150 mg/m2/d, days 1 to 4 plus Ara C 200 mg/m2/d, days 1 to 7 (arm B, 18 patients). Prophylaxis of disseminated intravascular coagulation was made by platelet transfusions and heparin. In case of leukemic resistance, patients received a second course with 2 days of rubidazone (arm A) or Amsa (arm B) and 3 days of Ara C. Patients who achieved complete remission (CR) received three consolidation courses with the two drugs used for induction and maintenance therapy for 3 years. Two patients in arm A and one in arm B were allografted in first CR. Initial characteristics were similar in both arms. In arm A, 18 patients (86%) reached CR, two had hypoplastic death, and one had leukemic resistance after two courses. In arm B, 12 patients (66%) achieved CR, two had early death (CNS bleeding, one case; ventricular fibrillation, one case), and four had resistant leukemia after two courses. The difference in CR rate between the two arms was not significant. In arm A, disease-free survival (DFS) showed a plateau at 54.3% after 34 months (95% confidence interval [CI], 32.1% to 74.9%), with eight CRs longer than 34 months. In arm B, DFS was significantly shorter (P less than .03), showing a plateau at 16.7% after 38 months (95% confidence interval, 4.7% to 44.6%), and only two prolonged CRs were seen. The difference in DFS remained significant after censoring allografted patients and patients who died in CR (one in arm A, two in arm B). Our results suggest that Amsa-Ara C combinations may be inferior to anthracycline-Ara C combinations in the treatment of APL, because they seem to provide shorter DFS and, possibly, a higher incidence of initial leukemic resistance. However, studies with larger numbers of patients are required.
Assuntos
Amsacrina/administração & dosagem , Antibióticos Antineoplásicos/administração & dosagem , Daunorrubicina/análogos & derivados , Leucemia Promielocítica Aguda/tratamento farmacológico , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Citarabina/administração & dosagem , Daunorrubicina/administração & dosagem , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Indução de Remissão , Taxa de SobrevidaRESUMO
We conducted a randomized multicenter trial comparing low-dose cytarabine (LD ARA-C) (20 mg/m2 for 21 days) with an intensive chemotherapy (rubidazone [a daunorubicin-derived agent], 100 mg/m2 for 4 days, ARA-C 200 mg/m2 for 7 days) in 87 patients over 65 years of age with de novo acute nonlymphocytic leukemia (ANLL). Forty-one patients received LD ARA-C and 46 received intensive chemotherapy. The number of complete remissions (CRs) but also of early deaths was higher in the intensive chemotherapy group, while partial remissions (PRs) and failures were more frequent in the LD ARA-C group (P less than .001). Infectious complications during induction treatment were more numerous and more severe in the intensive chemotherapy group (P less than .01). Patients treated with LD ARA-C required fewer RBC transfusions (P less than .02), fewer platelet transfusions (P less than .01), and had a shorter hospital stay for induction treatment (P less than .01). Overall survival and CR duration were not significantly different in either group. In the LD ARA-C group, the survival of patients with PR and those of patients in CRs was identical. We conclude that in a selected group of elderly patients with de novo ANLL a higher number of CRs may be obtained with intensive chemotherapy, but that with LD ARA-C, the number of early deaths is lower, and long-lasting PRs are obtained, resulting in a similar overall survival.
Assuntos
Antineoplásicos/uso terapêutico , Citarabina/uso terapêutico , Daunorrubicina/análogos & derivados , Leucemia Mieloide Aguda/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/administração & dosagem , Antineoplásicos/toxicidade , Medula Óssea/efeitos dos fármacos , Medula Óssea/patologia , Citarabina/administração & dosagem , Citarabina/toxicidade , Daunorrubicina/administração & dosagem , Daunorrubicina/uso terapêutico , Daunorrubicina/toxicidade , Feminino , Seguimentos , Humanos , Leucemia Mieloide Aguda/patologia , Masculino , Prognóstico , Distribuição Aleatória , Indução de RemissãoRESUMO
PURPOSE: The aim of this phase II trial was to assess the efficacy of fludarabine monophosphate in untreated and pretreated mantle-cell lymphomas (MCL). PATIENTS AND METHODS: Fifteen patients with MCL were included in the study. In two cases, fludarabine was the first-line therapy, the second in four cases, the third in five cases, and the fourth in four cases. The diagnosis of MCL was based on the criteria of the European Lymphoma Task Force (ELTF), with morphologic, immunologic, and cytogenetic data. Patients were treated with intravenous fludarabine 25 mg/m2/d for 5 days every 4 weeks. RESULTS: Toxicity of fludarabine was mild: World Health Organization (WHO) grade 3 and 4 granulocytopenia occurred in 15 of 56 assessable cycles (cy) (27%), there was no grade 3 or 4 thrombocytopenia, one grade 3 bacterial lung infection, and no treatment-related death. There were five partial responses (33%) but no complete response. The duration of these responses was short and ranged from 4 to 8 months. CONCLUSION: These results suggest that fludarabine can be moderately effective in the treatment of MCL. Fludarabine appears to be far less effective than in chronic lymphocytic leukemia (CLL) and follicular non-Hodgkin's lymphoma (NHL). Therefore, fludarabine should be evaluated in association with other chemotherapeutic agents in MCL.
Assuntos
Antimetabólitos Antineoplásicos/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Fosfato de Vidarabina/análogos & derivados , Adulto , Idoso , Antimetabólitos Antineoplásicos/efeitos adversos , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fosfato de Vidarabina/efeitos adversos , Fosfato de Vidarabina/uso terapêuticoRESUMO
PURPOSE: To compare progression-free survival (PFS), overall survival (OS), and toxicity of a doxorubicin-containing regimen administered alone or in combination with interferon alfa-2b (IFNalpha) in patients with low-grade follicular lymphoma (FL) and poor prognostic factors. PATIENTS AND METHODS: Two hundred sixty-eight patients with advanced-stage FL received cyclophosphamide, doxorubicin, teniposide, and prednisone (CHVP) monthly for 6 months, then every 2 months for 12 months. After randomization, 242 patients were evaluated for efficacy: 119 received CHVP alone, and 123 also received IFNalpha at a dose of 5 million units three times weekly for 18 months. RESULTS: After a 6-year median follow-up, the patients treated with CHVP + IFNalpha showed significantly longer median PFS than those who received CHVP alone (2.9 years v 1.5 years, respectively; P = .0002) and significantly longer median OS (not reached v 5.6 years, respectively; P = .008). Although some side effects, which included neutropenia, asthenia, fever, elevated serum transaminase levels, flu-like symptoms, and thrombocytopenia, were more frequently observed in patients who received the combination regimen, these reactions were moderate. IFNalpha was withdrawn because of toxicity in 10% of the patients, and a dosage reduction or temporary suspension was required in 28%. CONCLUSION: With long-term follow-up of 6 years, these results confirm that the addition of IFNalpha to a doxorubicin-containing regimen for patients with advanced-stage and clinically aggressive FL not only increased PFS, as in most other similar trials, but also prolonged OS. Toxicity was moderate. The beneficial effects of this combined chemotherapy and IFNalpha regimen on OS probably reflect the selection of FL patients with poor prognostic factors.
Assuntos
Antineoplásicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Interferon-alfa/uso terapêutico , Linfoma Folicular/tratamento farmacológico , Antineoplásicos/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Feminino , Humanos , Incidência , Interferon alfa-2 , Interferon-alfa/efeitos adversos , Masculino , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Prognóstico , Proteínas Recombinantes , Análise de Sobrevida , Teniposídeo/administração & dosagemRESUMO
Our study concerns eight pregnancies, six of which were successful, in four patients with paroxysmal nocturnal hemoglobinuria (PNH). Several complications of PNH during pregnancy were prevented: chronic anemia, folate and iron deficiency, and deep-vein thrombosis. During puerperium, acute hemolytic crises, most probably triggered by delivery, were observed in two patients. Thrombotic complications could be prevented by early initiation of an anticoagulant therapy after delivery. The only neonatal complication, observed in two cases, was isoimmune hemolytic anemia related to the multiple blood transfusions received before and during pregnancy. These results show that successful pregnancies are possible in women with PNH provided that both the obstetricians and physicians in charge monitor the pregnancies closely.
Assuntos
Hemoglobinúria Paroxística/terapia , Complicações Hematológicas na Gravidez/terapia , Adulto , Transfusão de Sangue , Cumarínicos/uso terapêutico , Parto Obstétrico , Transfusão de Eritrócitos , Feminino , Heparina/uso terapêutico , Humanos , GravidezRESUMO
We have studied in detail the effects of cholera toxin (CT), its pentameric B-chain subunit (toxoid) and the A-promoter chain on the differentiation of hemopoietic progenitor cells. Murine marrow cells were treated either with CT or its subunits. After stimulation with either the multilineage growth factor (multi-CSF; also called interleukin 3 or HCGF) or other hemopoietic regulators (colony-stimulating factors, or CSF), the clones grown in semisolid collagen cultures were scored in situ. Pluripotent stem cells (CFU-S) and multilineage (mixed CFU) or lineage-restricted progenitors (CFU-c) were estimated. We found that CT sensitivity is gradually gained by cells through the stepwise differentiation processes (i.e., CFU-S less than multilineage CFC less than committed CFC less than maturing cells). CT also has a selective, dose-dependent inhibitory effect (1 microM to 1 pM) on hemopoietic lineages (basophil-mast cells less than megakaryocytes less than neutrophils less than monomacrophages). These phenomena were obvious when the clonal growth was supported by multi-CSF but, interestingly, were not observed when lineage-restricted CSF were used. They furnished additional evidence that multi-CSF activates cells in a specific manner. This growth factor involved in progenitor cell self-renewal control may contribute to maintaining, on maturing cells, characteristics that are normally the attributes of progenitors.
Assuntos
Toxina da Cólera/farmacologia , Células-Tronco Hematopoéticas/citologia , Animais , Basófilos/efeitos dos fármacos , Ciclo Celular/efeitos dos fármacos , Ensaio de Unidades Formadoras de Colônias , Fatores Estimuladores de Colônias/antagonistas & inibidores , Granulócitos/efeitos dos fármacos , Interleucina-3 , Linfocinas/antagonistas & inibidores , Mastócitos/efeitos dos fármacos , Megacariócitos/efeitos dos fármacos , Camundongos , Monócitos/efeitos dos fármacosRESUMO
PURPOSE: To assess whether abnormalities depicted by Thallium-201 scintigraphy can predict the occurrence of late cardiac complications in patients with Hodgkin's disease treated with mantle field radiation therapy. METHODS AND MATERIALS: Thallium scintigraphy was performed in 49 patients at a median of 75 months after initial treatment (range 28-208 months). Initial treatment consisted in chemotherapy, given to two-thirds of the patients and mantle field radiation, delivered to all patients, using a 25-MV linear accelerator. Myocardial perfusion defects were observed in 78% of patients on thallium scintigraphy. These patients had their cardiac status reassessed at a median follow-up of 13.5 years after treatment. RESULTS: Forty-two patients were assessable, as data on the cardiac status were missing in 7 patients. The majority of patients received at least 40 Gy, and 75% of them were treated with one field per day. The median follow-up of patients is 13.5 years (range 9-24.5). Eleven cardiac complications were observed in 9 patients (coronary artery disease [n = 2], conduction-system abnormalities [n = 3], valvular defects [n = 5], and congestive heart disease [n = 1]). The median 15-year actuarial incidence of cardiac complications was 21% (95% confidence interval of 9-40%). The positive and negative predictive value of thallium scintigraphy was 19% and 77%, respectively. The univariate analysis showed that the extent of left ventricle exposure to irradiation was an adverse prognostic factor, and chemotherapy administered before mantle field irradiation was of borderline significance. CONCLUSION: Thallium scintigraphy is not predictive of late cardiac complications. The extent of left ventricle exposure to radiation and possibly chemotherapy given before radiation treatment are adverse prognostic factors.
Assuntos
Circulação Coronária/efeitos da radiação , Cardiopatias/etiologia , Coração/diagnóstico por imagem , Coração/efeitos da radiação , Doença de Hodgkin/radioterapia , Radioisótopos de Tálio , Adolescente , Adulto , Intervalos de Confiança , Doença das Coronárias/etiologia , Feminino , Seguimentos , Doença de Hodgkin/tratamento farmacológico , Humanos , Masculino , Mediastino , Valor Preditivo dos Testes , Dosagem Radioterapêutica , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
Most cases of Ki-1 + anaplastic large cell lymphomas (ALCL) express the CD25 antigen, and we have recently shown that IL-2 producing cells can also be detected in these lymphomas. The latter cells may thus constitute targets for anti-CD25 Mab treatment. In one patient with a chemoresistant disseminated Ki-1 + ALCL such a therapeutic approach was undertaken. We report the dramatic antitumoral effect of anti-CD25 Mab obtained in this patient. This confirms the observation, previously reported in adult T cell leukemia, showing that this therapeutic regimen is also efficient in CD25 + solid tumors.
RESUMO
We recently demonstrated that IL-2 is produced by reactive T cells in CD25-positive malignant lymphomas (ML). Using in situ hybridization, we investigated IL-6 mRNA expression in these CD25-positive ML. The ML tested included 9 anaplastic large cell lymphomas and 3 B-diffuse large cell lymphomas. Five CD25-negative ML were studied as controls. We show that IL-6 producing cells are present in all these ML. The density of positive cells was heterogeneous from case to case. However 3 cases of CD25-positive ML showed a dramatically higher density of IL-6 producing cells (70, 50, 43 producing cells per 10,000 cells, respectively) as compared to the other 9 cases of CD25-positive ML (mean 6.03 +/- 2.1 per 10,000). Morphological and topographical data suggested that several types of cells including fibroblasts, lymphocytes, macrophages and endothelial cells may synthesize IL-6. A combination of immunohistochemistry and in situ hybridization showed that reactive T cells and endothelial cells express the IL-6 gene whereas CD30-positive ML cells do not express this gene. Previous studies showed that IL-6 was capable to induce IL-2 receptor expression as well as production of IL-2 and stimulation of lymphomatous cells growth. Our present results indicate that the paracrine production of this cytokine may play a role in the proliferation of malignant lymphomas.
Assuntos
Interleucina-6/biossíntese , Linfoma Difuso de Grandes Células B/imunologia , RNA Mensageiro/metabolismo , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Expressão Gênica , Humanos , Imuno-Histoquímica , Interleucina-2/biossíntese , Interleucina-6/genética , Linfoma Difuso de Grandes Células B/genética , Masculino , Pessoa de Meia-Idade , RNA Mensageiro/genética , Receptores de Interleucina-2RESUMO
The authors report the results of a chemotherapy regimen of methyl-GAG, etoposide and prednimustine in 21 elderly patients (median age = 78 years) with aggressive non-Hodgkin's lymphoma. Sixteen patients responded (11 complete remissions, 5 partial remissions) with minor toxic side-effects. This regimen could be proposed for patients whose age and/or cardiovascular status prohibit treatment with usual anthracycline-containing chemotherapy programs.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Relação Dose-Resposta a Droga , Etoposídeo/administração & dosagem , Feminino , Humanos , Linfoma não Hodgkin/mortalidade , Masculino , Mitoguazona/administração & dosagem , Prednimustina/administração & dosagem , Taxa de SobrevidaRESUMO
The authors report a complex case in which chronic lymphoid leukaemia was associated with leiomyoblastoma of the digestive tract and cutaneous thrombosing vasculitis with serum anti-cardiolipin antibodies. They discuss a new physiopathogenetic mechanism of vasculitis in blood diseases.
Assuntos
Anticorpos Anticardiolipina/análise , Leiomioma , Leucemia Linfocítica Crônica de Células B/complicações , Vasculite/etiologia , Neoplasias Duodenais , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas , Vasculite/imunologiaRESUMO
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by sensitive populations of erythrocytes, granulocytes and platelets. PNH is a disease of young adults with a slight female predominance. Several complications of PNH during pregnancy, could be prevented; chronic anemia, folate and iron deficiency, deep vein thrombosis. We report seven pregnancies, six of which were successful in four patients. One pregnancy was terminated after 25 weeks by a fetal death during an acute hemolytic crisis. Diagnosis of PNH was made in the four patients before the pregnancy by the acidified serum lysis assay and the sucrose lysis assay. During puerperium, acute hemolytic crisis, most probably triggered by delivery, were observed in two patients. Thrombotic complications could be prevented by early initiation of an anticoagulant therapy after delivery. The only neonatal complication, observed in two cases was iso immune hemolytic anemia related to the multiple blood transfusions received before and during pregnancy. These results show that successful pregnancies are possible in PNH women when monitoring is especially close. To allow optimal fetal development, patients were transfused with saline-washed or frozen-thawed packed red-cells to prevent the precipitation of hemolysis, so that the hemoglobin level remained higher than 10 g/dl. During the whole pregnancy, patients had to be given dietary supplementation with folic acid and iron therapy whenever deficiency was demonstrated, under close surveillance of hemolysis. To prevent thrombotic complications during pregnancy, anticoagulant therapy was used if the patients had to be bedridden, or within 8 hours following delivery.
Assuntos
Hemoglobinúria Paroxística/fisiopatologia , Complicações Hematológicas na Gravidez/fisiopatologia , Adulto , Androgênios/uso terapêutico , Peso ao Nascer , Transfusão de Sangue , Cesárea , Feminino , Hemoglobinúria Paroxística/terapia , Humanos , Recém-Nascido , Gravidez , Complicações Hematológicas na Gravidez/terapiaRESUMO
The authors report 3 pregnancies in 3 patients with essential thrombocythemia. In the 3 cases, the course of the pregnancy was successful, with normal delivery in 2 cases, and premature delivery of a live fetus because of an abruptio placentae in the third case. From these 3 cases and a review of the literature, the authors analyse the fetal and maternal risks of pregnancy in these patients and the potential usefulness of preventive treatments (platelet antiaggregating drugs, anticoagulants). Besides, in these 3 cases a steadily decrease in blood platelet counts was observed during pregnancy with a nadir just before delivery followed by a sharp increase in the days following delivery. These findings may give some useful information on the regulation of thrombopoiesis.
Assuntos
Aspirina/uso terapêutico , Heparina de Baixo Peso Molecular/uso terapêutico , Complicações Hematológicas na Gravidez/tratamento farmacológico , Trombocitemia Essencial/tratamento farmacológico , Adulto , Feminino , Humanos , Contagem de Plaquetas , Período Pós-Parto , Gravidez , Complicações Hematológicas na Gravidez/sangue , Trombocitemia Essencial/sangue , Trombocitemia Essencial/complicações , Tromboembolia/etiologia , Tromboembolia/prevenção & controle , Fatores de TempoRESUMO
The association between thrombocytopenic thrombotic purpura (TTP) and infectious or multisystem disease, pregnancy, transplantation, drugs and toxins is well known. The onset of TTP in the course of neoplasm is rarely described. The authors report one case of TTP associated with prostatic tumor. TTP preceded the discovery of neoplasm by 12 months. Moreover, TTP showed an original course with four recurrences despite antiplatelet and hormonal therapies; the successive regressions were obtained with less and less effort, as TTP was discovered at an early stage.
Assuntos
Neoplasias da Próstata/complicações , Púrpura Trombocitopênica Trombótica/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias da Próstata/patologia , Neoplasias da Próstata/terapia , Púrpura Trombocitopênica Trombótica/terapia , RecidivaRESUMO
INTRODUCTION: The reactivation of varicella-zoster virus occurs in immunocompromised patients, especially in cases of hematological malignancy. Disseminated reactivation could involve digestive tract with life-threatening condition. CASE REPORT: A 76-year-old woman, with a history of chronic lymphocytic leukemia, presented with left hypochondrium pain, and a vesicular rash with hemorrhagic shock that revealed an hemorrhagic gastritis due to varicella-zoster virus. The literature review identified 28 additional cases of gastrointestinal mucosal damage during reactivation of varicella-zoster virus. Mortality is 40%. We report here the first case in the course of low-grade lymphoid malignancy. CONCLUSION: Acute gastrointestinal symptoms in immunocompromised patients should evoke a varicella-zoster virus reactivation with gastrointestinal involvement. This clinical manifestation, although rare, should not be ignored because of its severity.