Primary myelofibrosis: risk stratification by IPSS identifies patients with poor clinical outcome

OBJECTIVES: To evaluate whether risk scores used to classify patients with primary myelofibrosis and JAK-2 V617F mutation status can predict clinical outcome. METHODS: A review of clinical and laboratory data from 74 patients with primary myelofibrosis diagnosed between 1992 and 2011. The IPSS and Lille scores were calculated for risk stratification and correlated with overall survival. RESULTS: A V617F JAK2 mutation was detected in 32 cases (47%), with no significant correlation with overall survival. The patients were classified according to the scores: Lille - low, 53 (73.%); intermediate, 13 (18%); and high, 5 (7%); and IPSS- low, 15 (26%); intermediate-1, 23 (32%); intermediate-2, 19 (26%); and high, 15 (31%). Those patients presenting a higher risk according to the IPSS (high and intermediate-2) had a significantly shorter overall survival relative to the low risk groups (intermediate-1 and low) (p = 0.02). CONCLUSIONS: These results emphasize the importance of the IPSS prognostic score for risk assessment in predicting the clinical outcome of primary myelofibrosis patients.

Associação entre HLA e leucemia em uma população brasileira de etnia mista / Association between HLA and leukemia in a mixed Brazilian population

OBJETIVOS: O objetivo deste estudo foi investigar a freqüência de antígenos HLA Classe I e de alelos HLA Classe II em 164 pacientes com vários tipos de leucemias: 35 pacientes com LLA (leucemia linfóide aguda), 50 com LMA (leucemia mielóide aguda) e 78 com LMC (leucemia mielóide crônica). MÉTODOS: A tipagem HLA Classe I foi realizada por microlinfocitotoxicidade e a de Classe II por PCR-SSP (polymerase chain reaction - sequence specific of primers), ambas da One Lambda (Canoga Park, CA, US). RESULTADOS: Em pacientes com LLA, as freqüências das variantes HLA-B45 e HLA-B56 foram maiores (P = 0,02; OR = 3,13; 95 por centoIC = 0,94-10,44; P = 0,03; OR = 3,61; 95 por centoIC = 0,47-27,64, respectivamente), quando comparadas com controles. Nos pacientes com LMA, a freqüência de HLA-B7 (P = 0,01; OR = 2,41; 95 por centoIC = 1,25-4,67) foi maior que em controles. A presença de HLA-B45 (P= 0,01; OR = 3,29; 95 por centoIC = 1,46-7,40) e de HLA-DRB1*04 (P = 0,002; OR = 2,17; 95 por centoIC = 1,36-3,46) e HLA-DRB1*08 (P = 0,004; OR = 2,36; 95 por centoIC = 1,34-4,16) foi associada ao maior risco de desenvolver LMC. CONCLUSÃO: Nossos resultados sugerem que variantes HLA conferem susceptibilidade a algumas formas de leucemia e podem prover novas ferramentas para a investigação da genética e etiologia desta doença.

OBJECTIVE: The main purpose of this study was to investigate the class I HLA antigens and class II HLA allele frequencies in 164 patients with leukemia: 35 patients with ALL (acute lymphoid leukemia), 50 with AML (acute myeloid leukemia) and 78 with CML (chronic myeloid leukemia). METHODS: The genotyping of class I HLA was performed by microlymphocytotoxicity and of class II by PCR-SSP (polymerase chain reaction - sequence specific of primers) (One Lambda, Canoga Park, CA, USA). RESULTS: In patients with LLA, frequencies of HLA-B45 and HLA-B56 were higher (P = 0.02; OR = 3.13; 95 percentIC = 0.94-10.44; P = 0.03; OR = 3.61; 95 percentIC = 0.47-27.64, respectively), than in controls. In patients with AML, the frequency of HLA-B7 (P = 0.01; OR = 2.41; 95 percentIC = 1.25-4.67) was higher than in controls. The presence of HLA-B45 (P= 0.01; OR = 3.29; 95 percentIC = 1.46-7.40), HLA-DRB1*04 (P = 0.002; OR = 2.17; 95 percentIC = 1.36-3.46) and HLA-DRB1*08 (P = 0.004; OR = 2.36; 95 percentIC = 1.34-4.16) was associated to increased risk of CML developing. CONCLUSION: Our results suggest that variants of HLA confer susceptibility to the same forms of leukemia, and could provide new tools for the investigation of genetics and etiology of this disease.

Conventional chemotherapy for acute myeloid leukemia: a Brazilian experience

CONTEXT: Young patients affected by acute myeloid leukemia (AML) achieve complete remission (CR) using conventional chemotherapy in about 55-85 percent. However, 30 percent of patients fail to achieve CR and the remission duration is often only about 12 months. More intensive treatment after CR seems to be necessary in order to maintain CR and obtain a definitive cure. In Brazil, few reports have been published on this important subject. OBJECTIVE: The aim of this study was to describe a Brazilian experience in the treatment of "de novo" acute myeloid leukemia (AML) in younger adult patients (age < 60 years). DESIGN: Retrospective analysis. SETTING: University Hospital, Hematology and Hemotherapy Center, State University of Campinas, Brazil...

Vanishing bile duct syndrome in Hodgkin's disease: case report

CONTEXT: Liver damage is relatively common in patients affected by Hodgkin's disease. A smaller proportion of cases develops jaundice. Recently, the vanishing bile duct syndrome was described in Hodgkin's disease. The mechanisms of this severe complication have been poorly understood until now. OBJECTIVE: To describe a rare case of intra-hepatic cholestasis due to vanishing bile duct syndrome. DESIGN: Case report. CASE REPORT: A 38-year-old male patient affected by Hodgkin's disease. Liver biopsy showed no detectable Hodgkin's disease. Intra-hepatic cholestasis was found and none of the six portal tracts analyzed contained normal bile ducts. The treatment was based on conventional and high-dose escalation chemotherapy. The patient died from an irreversible liver failure while in complete remission from Hodgkin's disease

As características da Megacariopoese nas Síndromes Mielodisplásicas / The characteristics of a megakaryocyte in myelodysplasia

As síndromes mielodisplásicas säo um grupo heterogêneo de distúrbios do precursor hemopoiético pluripotencial. Encontra-se frequentemente aumento de número e atipias nos precursores megacariocíticos. Examinamos a megacariopoese em 25 pacientes com SMD ao diagnóstico e a comparamos com a de oito doadores de medula óssea usados como controles. Os megacariócitos estiveram aumentados em 16/25 casos usando-se o anticorpo CD61 e em 12/25 casos quando se usou o CD42b. O número de megacariócitos contados em cortes de medula óssea correlacionou com o de células CD42b positivas, mas näo com o de células CD61 positivas. Houve, porém, uma correlaçäo entre os resultados das duas técnicas imunocitoquímicas. Assim, as três técnicas foram úteis para avaliar a megacariopoese nas SMD. O CD61, porém, evidenciou os precursores mais imaturos.

Comprometimento cardíaco secundário às doenças hematológicas / Heart onset caused by hematological disease

Os autores apresentam uma revisäo da literatura e procuram demonstrar, de forma organizada e didática, as anormalidades adquiridas do tecido cardíaco e do sistema vascular coronário, causadas diretamente e/ou desencadeadas por mecanismos dependentes da presença de doenças hematológicas benignas ou malignas, ou, ainda, secundárias aos tratamentos utilizados. O texto discute as alteraçöes cardíacas de causa anóxica, principalmente causadas pelas anemias e pela síndrome de hiperviscosidade. Dentre as anemias, destacam-se as hemoglobinopatias estruturais e quantitativas e, na síndrome de hiperviscosidade, as causadas pela elevaçäo do número de células sanguíneas e pelo aumento das imunoglobulinas séricas. Säo apresentados, ainda, os comprometimentos cardíacos devido a infiltraçäo endocárdica, miocárdica e pericárdica por células neoplásicas; a hemocromatose, depósito anormal de ferro no coraçäo; e as cardiopatias associadas às drogas antineoplásicas e por açäo de radiaçäo de uso terapêutico.