RESUMO
INTRODUCTION: Due to lack of patient/health care provider awareness causing delayed diagnosis, the bleeding phenotype and provider interventions in adolescents with heavy menstrual bleeding (HMB) and bleeding disorders (BD) may be different when compared to adults. AIM: The aim of this study was to compare/characterize bleeding phenotype and provider interventions in postmenarchal adolescents < 18 years and premenopausal adults ≥ 18 years with HMB and BD. METHODS: Patient demographics, BD, and provider interventions/therapy details for HMB were compared between both age groups enrolled in the Centers for Disease Control and Prevention (CDC) Female Universal Data Collection (UDC) surveillance project in United States hemophilia treatment centres. Cross-sectional descriptive analyses including frequency distributions, summary statistics, bivariate and logistic regression analyses were performed. RESULTS: Of 269 females (79 adolescents; median age 16 years, interquartile range (IQR) = 2; 190 adults; median age 27 years, IQR = 13) evaluated, BD distribution was similar in both groups. Compared to adolescents, adults more often had family history of bleeding (Adjusted odds ratios [AOR] = 2.6, 1.3-5.6), delay in diagnosis (AOR = 2.5, 1.2-4.9), bleeding with dental procedures (AOR = 2.0, 1.0-4.0), gastrointestinal bleeding (AOR = 4.6, 1.0-21.9), anaemia (AOR = 2.7, 1.4-5.2), utilized desmopressin less often (AOR = 0.4, 0.2-0.8) and underwent gynaecologic procedure/surgery more frequently (AOR = 5.9, 1.3-27.3). CONCLUSION: Bleeding phenotypes of adolescents and adults with HMB and BD were different with more frequent bleeding complications, anaemia, gynaecologic procedures/surgeries, less desmopressin use and more delay in diagnosing BD in adults. Longitudinal studies are needed to determine whether improved patient/provider awareness and education will translate to early diagnosis and timely management of BD/HMB in adolescents that may prevent/reduce future haematologic/gynaecologic complications.
Assuntos
Transtornos da Coagulação Sanguínea/diagnóstico , Menorragia/diagnóstico , Adolescente , Adulto , Anemia/etiologia , Antifibrinolíticos/uso terapêutico , Transtornos da Coagulação Sanguínea/complicações , Transtornos da Coagulação Sanguínea/tratamento farmacológico , Estudos Transversais , Desamino Arginina Vasopressina/uso terapêutico , Diagnóstico Tardio , Feminino , Hemorragia Gastrointestinal/etiologia , Hemostáticos/uso terapêutico , Humanos , Modelos Logísticos , Menopausa , Menorragia/complicações , Menorragia/tratamento farmacológico , Menorragia/etnologia , Razão de Chances , Fenótipo , Adulto JovemRESUMO
Factor X is a vitamin K-dependent, liver-produced serine protease that serves a pivotal role in coagulation as the first enzyme in the common pathway to fibrin formation. Inherited factor X deficiency is a rare autosomal recessive bleeding disorder that is estimated to occur in 1:1,000,000 individuals up to 1:500 carriers. Several international registries of FX-deficient patients have greatly expanded the knowledge of clinical phenotype. A proposed classification of severity is based on FX:C activity measurements: an FX:C measurement <1% is severe, an FX:C measurement of 1-5% is moderate and an FX:C measurement of 6-10% is mild. Levels above 20% are infrequently associated with bleeding and heterozygotes are usually asymptomatic. Among patients with FX:C levels <10%, unlike moderate or severe haemophilia A and B, mucocutaneous bleeding symptoms such as epistaxis and menorrhagia occur in the majority. In addition, patients with moderate-severe deficiency may have symptoms similar to that of haemophilia A and B, including haemarthrosis, intracranial haemorrhage, and gastrointestinal bleeding. Genotype characterization may offer important clues about clinical prognosis. More than 80 mutations of the F10 gene have been identified, most of which are missense mutations. There is no specific FX replacement product yet readily available, but fresh frozen plasma and prothrombin complex concentrates can be used for treatment of bleeding symptoms and preparation for surgery.
Assuntos
Coagulantes/uso terapêutico , Deficiência do Fator X/genética , Fator X/fisiologia , Hemorragia/genética , Sistema de Registros/estatística & dados numéricos , Adulto , Fatores de Coagulação Sanguínea/uso terapêutico , Testes de Coagulação Sanguínea , Criança , Fator X/uso terapêutico , Deficiência do Fator X/classificação , Deficiência do Fator X/diagnóstico , Deficiência do Fator X/tratamento farmacológico , Feminino , Genótipo , Hemorragia/tratamento farmacológico , Heterozigoto , Homozigoto , Humanos , Lactente , Recém-Nascido , Irã (Geográfico)/epidemiologia , Itália/epidemiologia , Masculino , Mutação de Sentido Incorreto , Plasma , Gravidez , Prevalência , Doenças Raras , Índice de Gravidade de Doença , Reino Unido/epidemiologia , Adulto JovemRESUMO
Considerable progress has been made in the past decade in describing the obstetrical and gynaecological aspects of von Willebrand's disease (VWD). In addition, epidemiological studies have established an approximately 11-16% prevalence of the laboratory diagnosis of VWD in women presenting with menorrhagia. However, it is not established presently whether an upfront VWD screening should be a part of the standard evaluation of menorrhagia. This is because it is presently not known whether therapy in the VWD patient tailored specifically for VWD will appreciably alter the natural history of menorrhagia compared with the non-VWD menorrhagia patient. There are also subtleties involved in securing the diagnosis of VWD in women presenting with menorrhagia in terms of fluctuation of von Willebrand factor (VWF) levels vis-à-vis the menstrual cycle and the potential impact of oral contraceptive on VWF levels. Regarding management of VWD-related menorrhagia, pending ongoing comparative trials of intranasal desmopressin (DDAVP), tranexamic acid, oral contraceptive and the levonorgestrel intrauterine device, specific recommendations cannot be made presently regarding the superiority of one intervention compared with the other. The management of VWD-related postpartum haemorrhage is also an area of active debate in terms of 'best practice' in type 1 (? prophylactic DDAVP), type 2 [? expectant management if factor VIII:C (FVIII:C) level normalizes] and type 3 patients (? intensity and duration of infusional therapy with a VWF-containing plasma-derived FVIII concentrate). This review summarizes the present state of knowledge and highlights numerous questions for future study based on our present understanding of VWD in women.
Assuntos
Doenças de von Willebrand/complicações , Desamino Arginina Vasopressina/uso terapêutico , Feminino , Hemostáticos/uso terapêutico , Humanos , Programas de Rastreamento/métodos , Menorragia/etiologia , Menorragia/terapia , Hemorragia Pós-Parto/etiologia , Hemorragia Pós-Parto/prevenção & controle , Doenças de von Willebrand/diagnósticoRESUMO
BACKGROUND: Endometrial ablation has recently gained popularity as a treatment of menorrhagia in the general population. In the von Willebrand disease (VWD) patient, intuitively, it would appear that the failure rate would be higher because of the underlying hypocoaguability increasing the likelihood for re-bleeding. In a consecutive series of seven patients, we assessed the efficacy and safety of endometrial ablation in VWD-related menorrhagia. PATIENTS AND METHODS: We performed a retrospective analysis using chart review and a 21-item questionnaire administered to seven (six type 1, one type 2A) women who underwent endometrial ablation between the years 1997 and 2001. Parameters assessed included operative complications, the development of abdominal pains, recurrence of menstrual bleeding post-ablation and the change in the pre-/post-ablation quality of life (QOL). Three patients underwent endomyometrial resection and one each underwent rollerball, thermal, electrocautery and balloon ablation. All patients were pre-treated with i.v. desmopressin (DDAVP) except the 2A patient who received Humate P. Mean age of the patient was 41 +/- 6 years and follow-up was for 45 months (range 31-73) post-ablation. RESULTS: No significant perioperative bleeding complications were observed in any of the patients. All patients initially responded (two amenorrhoea, four hypomenorrhoea, one moderate improvement). In all patients, QOL assessed by 10 parameters improved significantly following the ablation procedure, regardless of the specific technique used. However, at the end of follow-up, only one patient remained amenorrheic, one was hypomenorrheic, one had moderate improvement and four patients experienced recurrence of menorrhagia, three eventually requiring a hysterectomy at a median of 11 months post-ablation. CONCLUSION: Endometrial ablation appears to be a safe procedure that improves the QOL in patients with VWD-related menorrhagia. However, its long-term efficacy appears to be lower in VWD patients when compared with women with menorrhagia without VWD.
Assuntos
Ablação por Cateter/métodos , Endométrio/cirurgia , Menorragia/cirurgia , Doenças de von Willebrand/complicações , Adulto , Feminino , Humanos , Menstruação , Pessoa de Meia-Idade , Período Pós-Operatório , Qualidade de Vida , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Central nervous system (CNS) involvement with malignant cells is a well recognized complication of hematologic neoplasms. A number of disorders such as acute lymphoblastic leukemia and high grade lymphoma frequently involve the CNS and prophylactic therapy is advised. Disorders such as acute myeloid leukemia (AML) and multiple myeloma are less likely to be associated with CNS involvement. This series describes three cases of CNS involvement by malignant hematologic disease: myelomatous meningitis, CNS chloromas complicating AML, and primary lymphomatous meningitis.
Assuntos
Neoplasias Encefálicas/diagnóstico , Leucemia Mieloide Aguda/diagnóstico , Linfoma/diagnóstico , Neoplasias Meníngeas/diagnóstico , Meninges/patologia , Mieloma Múltiplo/diagnóstico , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Córtex Cerebral/patologia , Evolução Fatal , Granulócitos/patologia , Humanos , Leucemia Mieloide Aguda/patologia , Leucemia Mieloide Aguda/terapia , Linfoma/patologia , Linfoma/terapia , Masculino , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/terapia , Mieloma Múltiplo/patologia , Mieloma Múltiplo/terapia , RadioterapiaRESUMO
The study of the proliferation and differentiation of the MDS clone at the molecular level, including the details of apoptosis, may hopefully lead to more effective differentiation-induction/antiapoptotic agents. The study of the cytokines at the cellular/molecular level may lead to more effective trails of combination therapy with differentiation-induction agents, chemotherapy, and/or early-acting cytokines. Further phenotypic characterization of the MDS clone may lead to negative selection of these cells or positive selection of normal stem cells as part of an autotransplant strategy, as is presently being done in chronic-phase chronic myeologenous leukemia. The use of agents such as the topoisomerase I inhibitors (e.g., topotecan), which have mechanisms of action disparate from agents already used in MDS, may increase the efficacy of chemotherapy for MDS. The further clinical refinements in reducing treatment-related mortality and the study of T cells at the molecular level may hopefully lead to improvement in the prevention and therapy of graft-versus-host disease, in turn increasing the upper age limit of allogeneic BMT for MDS and increasing the feasibility of matched unrelated allogeneic BMT. At present, we can tailor the approach to a MDS patient based on his or her IPSS risk stratification, degree of cytopenia, and age, as outlined in Figure 2. At present, we can tailor the approach to a MDS patient based on his or her IPSS risk stratification, degree of cytopenia, and age, as outlined in Figure 2.
Assuntos
Síndromes Mielodisplásicas/terapia , Apoptose , Transplante de Medula Óssea , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Humanos , Prognóstico , Transplante HomólogoRESUMO
One hundred and thirty-six patients autografted for relapsed or refractory non-Hodgkin's lymphoma (NHL) were evaluated to assess long-term event-free survival and to identify important prognostic factors. High-dose therapy consisted primarily of carmustine (BCNU), etoposide, cytarabine, and cyclophosphamide (BEAC) followed by unpurged autologous stem cell rescue. The 5-year Kaplan-Meier event-free survival (EFS) for the entire cohort was 34% (95% confidence interval: 24-44%) with a median follow-up of approximately 3 years (range 0-7.5 years). For patients entering with minimal disease (defined as all areas < or = 2 cm), the 5-year EFS was 40 vs 26% for those entering with bulky disease (P = 0.0004). In the multivariate analysis, minimal disease on entry and administration of involved-field XRT post-transplant were significantly associated with improved EFS; the latter association was observed mainly in the cohort of patients with bulky disease. The overall 100-day treatment-related mortality rate was 4.4% (3% for the last 71 patients). New strategies are needed to reduce the high rate of relapse (50-60%) following auto-transplantation for relapsed or refractory NHL.
Assuntos
Transplante de Medula Óssea , Linfoma não Hodgkin/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Linfoma não Hodgkin/patologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Neoplasia Residual , Prognóstico , Recidiva , Transplante AutólogoRESUMO
Flutamide, an anti-androgen used in prostate cancer therapy, is also a derivative of aniline. Mild, usually asymptomatic, methaemoglobinaemia has been reported. We report a patient receiving flutamide therapy who developed cyanosis, dyspnoea and anaemia, initially attributed to marked methaemoglobinaemia by the CO-Oximeter method. An unsuccessful trial of methylene blue therapy led to the finding of marked sulphaemoglobinaemia. Sulphaemoglobinaemia has not previously been reported with flutamide use. Recognition of this association is important, given the refractoriness of sulphaemoglobinaemia to methylene blue therapy.
Assuntos
Antineoplásicos Hormonais/efeitos adversos , Cianose/tratamento farmacológico , Flutamida/efeitos adversos , Azul de Metileno/uso terapêutico , Sulfemoglobinemia/induzido quimicamente , Idoso , Cianose/induzido quimicamente , Humanos , Masculino , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/tratamento farmacológico , Sulfemoglobinemia/tratamento farmacológico , Falha de TratamentoRESUMO
A 44-year-old women was treated for hyperparathyroidism resulting from parathyroid hyperplasia. Several months later, following a flu-like episode, she developed fever, confusion, abdominal pain, and diffuse petechiae, with severe thrombocytopenia and hemolytic anemia. She died on the 11th day of hospitalization. At autopsy she had multiple endocrine neoplasia type I, with two islet cell tumors, adrenal adenoma, pituitary adenoma, and bronchial carcinoid with liver metastasis. Florid visceral microthrombi involved arterioles and capillaries of the heart, including the conduction system. Brain, kidney, pancreas, adrenal, and portal areas of the liver were also heavily involved, but thrombi were rare in the liver sinusoids and the lungs. PAS-positive subendothelial deposits were demonstrated. In spite of the disseminated malignancy, the morphologic and laboratory findings were inconsistent with disseminated intravascular coagulation (DIC), and supported the clinical diagnosis of TTP. To the best of our knowledge this is the first report association of TTP with MEN and raises the question of a genetic linkage and/or hormonal interaction.
Assuntos
Neoplasia Endócrina Múltipla Tipo 1/complicações , Púrpura Trombocitopênica/complicações , Adulto , Evolução Fatal , Feminino , Humanos , Neoplasia Endócrina Múltipla Tipo 1/patologia , Metástase Neoplásica , Púrpura Trombocitopênica/patologiaRESUMO
BACKGROUND: Cyclophosphamide (CP) is one of the relatively few drugs implicated in systemic allergic reactions for which the metabolites are well known. Formation of CP metabolites is a multistep, time-dependent process (hours) with significant interindividual differences. Although allergic reactions to CP have been recorded in 17 previous reports, skin testing with CP or its metabolites has been included in only five. We now describe five patients receiving monthly cycles of intravenous CP whose allergic reactions included clinical features of type I hypersensitivity but were atypical in their markedly delayed onset (i.e., 8 to 16 hours in patients 1 to 4 and 10 days in patient 5). OBJECTIVE: The objective was to investigate these late-developing clinical reactions by skin testing with CP and two of its major metabolites. METHODS: The five patients and a control group receiving intravenous CP uneventfully were studied by the same skin test protocol. RESULTS: The four individuals in the control group were unreactive to CP or its metabolites. All five patients with late-onset allergic reactions had positive immediate skin test results to CP metabolites but not to CP itself. We propose that the allergic reactions in patients 1 to 4 were mediated, wholly or in major part, by IgE antibodies reactive with allergens derived from time-dependent drug metabolites. The 10-day lag time in patient 5 is unexplained. Immunomodulation by the underlying malignancies or by the immunosuppressive drugs could have contributed. CONCLUSION: IgE-mediated allergic drug reactions may have a delayed onset if the allergen is a time-dependent drug metabolite, illustrated in this study by CP.
Assuntos
Antineoplásicos Alquilantes/efeitos adversos , Ciclofosfamida/efeitos adversos , Hipersensibilidade a Drogas/etiologia , Hipersensibilidade Tardia/etiologia , Adulto , Anticorpos/sangue , Antineoplásicos Alquilantes/metabolismo , Ciclofosfamida/metabolismo , Hipersensibilidade a Drogas/imunologia , Feminino , Humanos , Hipersensibilidade Tardia/imunologia , Imunoglobulina E/sangue , Pessoa de Meia-Idade , Testes Cutâneos , Fatores de TempoRESUMO
Fourteen consecutive patients with chronic myelogenous leukemia (CML) blast crisis were treated with a more dose-intensive regimen of daunorubicin (70 mg/m2 per day on days 1-3) and cytosine arabinoside (200 mg/m2 per day as continuous infusion on days 1-9) than usually used in de novo acute myelogenous leukemia. The median age of the patients was 50 years (27-78 years). Eleven of 13 evaluable patients were aplastic at day 14 after a single course of therapy (11/13, 85%). Over-all response rate (complete + partial response) was 9/13 (69%). Restoration to chronic phase was achieved in 7/13 patients (54%) lasting a median of 78 days (49-235 days). However, four of these seven patients proceeded to bone marrow transplant (BMT) and so the true remission duration for this therapy cannot be determined. Treatment-related mortality was 4/14 (29%). Presently, four of nine patients evaluable are surviving post-induction, three S/P allogeneic BMT (0.6, 3.8, 5 years) and one patient S/P autologous BMT (3.3 years post-induction). These results of an intensive induction regimen achieving marrow aplasia in all except one patient to date warrants further study as the first step possibly towards BMT after CML blast crisis.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Crise Blástica/tratamento farmacológico , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologia , Adulto , Idoso , Transplante de Medula Óssea , Citarabina/administração & dosagem , Citarabina/efeitos adversos , Daunorrubicina/administração & dosagem , Daunorrubicina/efeitos adversos , Feminino , Seguimentos , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Masculino , Pessoa de Meia-IdadeRESUMO
Large granular lymphocyte (LGL) leukemia is typically associated with neutropenia and occasionally pure red cell aplasia. We now report a new association of LGL leukemia with amegakaryocytic aplasia that was also accompanied by pure red cell aplasia. An 82-year-old female presented with a platelet count of 16,000/microliters at the time of a gastrointestinal bleed. The white blood cell count was within normal limits but there was a relative lymphocytosis of large granulated cells that by flow cytometric and gene rearrangement studies was consistent with a monoclonal process. Platelet and red cell transfusion dependence persisted over 17 months with numerous immunosuppressive treatments given including prednisone, cyclophosphamide, vincristine, cyclosporine, gammaglobulin, and azathioprine. Presently, 36 months since the time of presentation, she is in a clinical remission with follow-up flow cytometric and gene rearrangement studies of the peripheral blood without definite evidence of the LGL clone. This case report illustrates the various therapeutic approaches to be considered in LGL leukemia and is a reminder that severe and life-threatening thrombocytopenia can be a feature of LGL leukemia unlike the usual course of LGL leukemia with neutropenia as the major clinical feature.
Assuntos
Imunossupressores/uso terapêutico , Leucemia Linfoide/diagnóstico , Púrpura Trombocitopênica/complicações , Aplasia Pura de Série Vermelha/complicações , Idoso , Idoso de 80 Anos ou mais , Feminino , Citometria de Fluxo , Rearranjo Gênico da Cadeia beta dos Receptores de Antígenos dos Linfócitos T , Humanos , Imunofenotipagem , Leucemia Linfoide/complicações , Leucemia Linfoide/tratamento farmacológico , Indução de RemissãoRESUMO
If chronic myelomonocytic leukemia (CMML) transforms into an acute leukemic phase, the blast crisis is invariably myeloid. Occasionally, the other subtypes of myelodysplastic syndrome (MDS) (refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation) have been noted to transform into acute lymphoblastic leukemia (ALL). We now report a case of CMML that transformed into ALL and we review the literature of 13 other cases of MDS with ALL transformation. Such cases provide suggestive clinical evidence that MDS can involve a pluripotent stem cell.
Assuntos
Transformação Celular Neoplásica/patologia , Leucemia Mielomonocítica Crônica/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/patologiaRESUMO
To evaluate the safety and efficacy of marrow transplantation for older adults, the regimen-related mortality and event-free survival for patients > or = 40 years were compared with those for patients < 40 years. Of 148 consecutive patients receiving autotransplants for lymphoma or Hodgkin's disease, 70 were < 40 years and 78 were > or = 40 years at the time of transplant, including 40 who were > or = 50 years and 12 who were > or = 60 years. Eleven patients (16%) in the younger age group died from transplant-related complications compared with 4 (5%) in the older age group. The 4-year actuarial event-free survivals (EFS) for the younger and older age groups were 43% and 48%, respectively. After adjustment for covariates with prognostic significance, older age was marginally associated with improved event-free survival (P = 0.08). Of 92 consecutive patients undergoing allogeneic BMT during the same period, 62 patients were < 40 years and 30 patients were > or = 40 years, including 8 patients > or = 50 years, and 1 patient > 60 years. Non-relapse mortality (including deaths from GVHD) occurred in 28 of the younger patients (45%) and 9 of the older patients (30%). The 3-year actuarial EFS for the younger patients was 26% vs. 56% for the patients > or = 40 years (P = 0.057). However, this difference was mainly due to the higher proportion of patients with CML and early-stage leukemia in the older age group.(ABSTRACT TRUNCATED AT 250 WORDS)
Assuntos
Transplante de Medula Óssea , Doença de Hodgkin/terapia , Linfoma não Hodgkin/terapia , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Intervalo Livre de Doença , Feminino , Doença de Hodgkin/mortalidade , Humanos , Linfoma não Hodgkin/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida , Transplante Autólogo , Transplante HomólogoRESUMO
A case of hepatitis B reactivation following allogeneic bone marrow transplantation for acute myelogenous leukemia is reported. The presence of hepatitis B surface antibody prior to transplantation indicated previous infection with hepatitis B. The presence of hepatitis B surface antigen was first detected 21 months after transplantation. Testing for hepatitis B reactivation was prompted by the development of acute hepatitis B in the patient's spouse. Screening for hepatitis B infection may be helpful when abnormal liver function tests develop after bone marrow transplantation.
Assuntos
Transplante de Medula Óssea , Hepatite B/diagnóstico , Doença Aguda , Adulto , Hepatite B/transmissão , Antígenos de Superfície da Hepatite B/sangue , Antígenos E da Hepatite B/sangue , Humanos , Leucemia Mieloide/terapia , Masculino , RecidivaRESUMO
We report a case of adult de novo acute lymphoblastic leukemia (ALL) with unique cytogenetic abnormalities and discrepant morphologic and immunologic features. Morphology was L1 by the French-American-British classification, but flow cytometry was consistent with a mature B-cell phenotype. Cytogenetic analysis showed numerous chromosome abnormalities nonspecific for lymphoid neoplasm except for t(1;19) and t(14;18). The former is characteristic of pre-B-ALL and the latter is characteristic of follicular lymphoma. This is the first report of these two translocations occurring concurrently in ALL.
Assuntos
Linfoma de Burkitt/genética , Cromossomos Humanos Par 14 , Cromossomos Humanos Par 18 , Cromossomos Humanos Par 19 , Cromossomos Humanos Par 1 , Translocação Genética , Medula Óssea/patologia , Linfoma de Burkitt/patologia , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
PURPOSE: One hundred autotransplants for Hodgkin's disease (HD) or non-Hodgkin's lymphoma (NHL) were examined prospectively to identify variables with prognostic significance. PATIENTS AND METHODS: Ninety-six patients with relapsed or refractory HD or NHL underwent 100 autotransplants. Patients received high-dose carmustine (BCNU), etoposide, cytarabine, and cyclophosphamide (BEAC) followed by unpurged autologous stem-cell rescue. RESULTS: The 3-year actuarial event-free survival (EFS) rate for the 47 HD patients is 49%, with a median followup duration of 2 years. For the 53 NHL patients, the 3-year actuarial EFS rate is 40%, with a median follow-up duration of 19 months. By multivariate analysis, minimal disease on admission (all areas < or = 2 cm) is associated with improved EFS (HD, P = .003, NHL, P = .03). The projected EFS rate for HD patients entering with minimal disease is 70% versus 15% for patients with bulky disease (P = .0001). The projected EFS rate for NHL patients with minimal disease is 48% versus 25% for patients with bulky disease (P = .04). Posttransplant involved-field radiotherapy, administered to 26 of the last 61 patients, was associated with an improved EFS rate for NHL patients (P = .015). The BEAC regimen was well tolerated by patients who entered the study with minimal disease (mortality rate, < 5%), but caused significant toxicity in patients with bulky disease (mortality rate, 25%). CONCLUSION: Disease burden before autotransplantation is an important predictor of regimen-related toxicity and EFS. Posttransplant involved-field radiotherapy may improve outcomes in select patients with NHL. The BEAC regimen is safe and effective, particularly for patients with minimal disease.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/terapia , Linfoma/terapia , Transplante de Células-Tronco , Análise Atuarial , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Terapia Combinada , Feminino , Doença de Hodgkin/tratamento farmacológico , Humanos , Linfoma/tratamento farmacológico , Masculino , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Estudos Prospectivos , Recidiva , Índice de Gravidade de Doença , Análise de Sobrevida , Resultado do TratamentoRESUMO
The FAB classification has provided a common language for clinicians and pathologists in the investigation of myelodysplasia. It has also provided prognostic information, given the excellent correlation between FAB subgroup and survival (see Table 2). Refinements in immunologic and cytogenetic typing should provide further prognostic power and correlation with the underlying biologic processes.