Cármino A. Souza

Bone marrow morphology in patients with neutropenia due to chronic exposure to organic solvents (benzene): Early lesions

The authors present the histological and cytological features of bone marrow (BM) in 152 employees from the steel plant of Cubatão (S. Paulo-Brazil) who presented with neutropenia, due to chronic exposure to benzene and its homologues. All patients were male. Mean age was 35 years. At the time of this study, all patients were removed from risk areas because of the hematological abnormality. BM morphology was characterized by a hypocellular hemopoiesis (82%). Decrease of the granulocytic precursors (86%) was the most outstanding feature. Erythroid and megakaryocytic series were diminished in 40% and 57% of the cases, respectively. Eosinophilia in BM was observed in 71% of the cases. Cell atypias and stromal changes (necrosis, increase in reticulin fibres) were frequent.

Histological and cytological heterogeneity of bone marrow in Philadelphia-positive chronic myelogenous leukaemia at diagnosis

Summary. Cytological and histological features of the bone marrow in Philadelphia‐positive chronic myelogenous leukaemia (CML) at diagnosis were analysed in a prospective study. Formerly described (Frisch et al, 1984) subtypes GRAN and GRAN/MEG were found, and myelofibrosis and incipient blast crisis could also be detected. A more or less continuous spectrum of number and degree of atypias in megakaryocytes was found. Biopsy and imprint were complementary in making differential diagnosis of both subtypes. Cytologically, type GRAN/MEG was characterized by a mixed proliferation of neutrophil, eosinophil and basophil series, besides proliferation of megakaryocytes. Type GRAN showed a predominantly neutrophilic proliferation. The importance of performing bone marrow analysis in evaluation of CML patients is discussed.

Confirmation of the utility of the International Staging System and identification of a unique pattern of disease in Brazilian patients with multiple myeloma

Multiple myeloma (MM) is one of the most frequent hematologic malignancies, and its incidence varies worldwide. Except for occasional case series or correlative biological studies, little is known about the incidence and clinical features of MM in Latin America. In Brazil, national estimates for the

Imbalances in serum angiopoietin concentrations are early predictors of septic shock development in patients with post chemotherapy febrile neutropenia.

BackgroundFebrile neutropenia carries a high risk of sepsis complications, and the identification of biomarkers capable to identify high risk patients is a great challenge. Angiopoietins (Ang -) are cytokines involved in the control microvascular permeability. It is accepted that Ang-1 expression maintains endothelial barrier integrity, and that Ang-2 acts as an antagonizing cytokine with barrier-disrupting functions in inflammatory situations. Ang-2 levels have been recently correlated with sepsis mortality in intensive care units.MethodsWe prospectively evaluated concentrations of Ang-1 and Ang-2 at different time-points during febrile neutropenia, and explored the diagnostic accuracy of these mediators as potential predictors of poor outcome in this clinical setting before the development of sepsis complications.ResultsPatients that evolved with septic shock (n = 10) presented higher levels of Ang-2 measured 48 hours after fever onset, and of the Ang-2/Ang-1 ratio at the time of fever onset compared to patients with non-complicated sepsis (n = 31). These levels correlated with sepsis severity scores.ConclusionsOur data suggest that imbalances in the concentrations of Ang-1 and Ang-2 are independent and early markers of the risk of developing septic shock and of sepsis mortality in febrile neutropenia, and larger studies are warranted to validate their clinical usefulness. Therapeutic strategies that manipulate this Ang-2/Ang-1 imbalance can potentially offer new and promising treatments for sepsis in febrile neutropenia.

Six cases of leprosy associated with allogeneic hematopoietic SCT.

We report here the first six cases of leprosy associated with HLA-identical allogeneic SCT in different phases and with different findings and outcomes. Skin and peripheral nerves may be sites of leprosy associated with SCT, stressing the importance of differential diagnosis between leprosy and GVHD or drug reactions. Clinical manifestations of leprosy before or after transplantation did not influence the outcome of SCT in our cases.

Granulocytic Sarcoma of the Larynx Preceding Chronic Myeloid Leukemia

The authors report one case of granulocytic sarcoma infiltrating the larynx and cervical lymph nodes in a 50-year-old smoking patient. At the time of diagnosis there was no clinical and laboratory evidence of acute myeloid leukemia or chronic myeloproliferative disease. Four months after diagnosis, bone marrow morphology was consistent with chronic myeloid leukemia, accelerated phase. Cytogenetic abnormalities (Ph 1 chromosome, t(1; 12) (p36; p13), and trisomy of chromosome 20) were also found in hemopoetic cells. Granulocytic sarcoma preceding installation of chronic myeloid leukemia, as described here, seems to be a rare clinical event.

Estudo retrospectivo do tratamento de leucemia mielóide aguda com o transplante de medula óssea: a experiência brasileira

Data from the International Bone Marrow Transplant Registry (IBMTR) contribute for the improvement of Bone Marrow Transplant (BMT) worldwide. We studied the Brazilian experience in BMT for AML to compare this with international data. We performed a retrospective study by sending questionnaires to 16 BMT centers regarding clinical and treatment variables. Statistical analyses concerning autologous BMT (autoBMT) and allogeneic BMT (alloBMT) were performed using the Kaplan-Meier method and the log-rank test. All p-values were two-tailed. We collected data from 731 patients (205 autoBMT and 526 alloBMT). Median overall survival (OS) for autoBMT patients was longer than alloBMT patients (1035 vs. 466 days, p=0.0012). AlloBMT stem cell source (SCS): 73% bone marrow stem cell (BMSC), 23% peripheral blood stem cells (PBSC) and 4% umbilical cord blood. Among the autoBMT patients, the SCS was 63% PBSC, 22% BMSC and 15% both. The SCS did not impact on OS. There was no difference in OS between different FAB classifications in the alloBMT group, but in the autoBMT the M3 patients had longer survival. As expected, the main cause of mortality among autoBMT patients was related to disease relapse (60%), while in the alloBMT, to infection (38%). In both groups we found longer OS in first complete remission (1CR) compared to second (2CR) and other (p<0.0001), and longer OS in de novo AML than in secondary. In the alloBMT group we found more patients with advanced disease (60%), while in the autoBMT group, we found more M3 patients (24%), which could explain the difference in OS. Most of our results are in accordance with IBMTR data. One should consider the fact that this is a retrospective study and our findings should be analysed with caution.

Molecular identification of the HLA-DRB1-DQB1 for diagnosis and follow-up of acute leukemias.

We analyzed a group of 45 Brazilian individuals, 30 with acute myeloid leukemia (AML), 15 with acute lymphoid leukemia (ALL) and 100 healthy controls to assess genetic factor risk and HLA association contribution to the disease. Patient rates were compared with age and sex-matched control groups by directly typing the HLA-DRB1/3/4/5 and -DQB1 loci by PCR analysis. We observed significantly increased allelic distribution of HLA-DRB107 in AML patients and of HLA-DRB103 in ALL patients, which suggests that individuals in both groups are susceptible to the disease. We also found significantly decreased allelic distribution of HLA-DQB104 in AML patients and of HLA-DRB104 and -DQB103 in ALL patients, which suggests protection against the disease. We further found increased HLA-DRB107 and -DQB102 haplotypes in AML patients, which suggests susceptibility to the disease and decreased HLA-DRB104 and -DQB103 haplotypes in ALL patients, which also suggests protection against the disease. Future studies with larger and/or multicentric samples will be required for better comprehension of the HLA role in acute leukemia pathogenesis.

Terapêutica citoprotetora em pacientes tratados com quimio e/ou radioterapia anti neoplásica

In recent years, cytoprotective agents have been developed to protect normal cells from the toxic effects of chemotherapy and radiotherapy. The ideal cytoprotectant is that which is able to allow intensification of chemotherapy; protects a broad spectrum of normal tissues and organs when used with a variety of chemotherapeutic agents; confers specific protection for normal tissues; preserves anti tumour activity and has little or manageable toxicity of its own. A cytoprotectant is administered prior to cytotoxic therapy, in contrast to the colony stimulant factors and Leucovorin, which are administered after chemotherapy to rescue the bone marrow and stimulate haematological recovery. Currently there are three cytoprotectors: two chemotherapy-specific (Dexrazoxane and Mesna) and one broad-spectrum (Amifostine). The authors discuss the main properties and usefulness of these drugs in Oncohematology.

Herpetic geometric glossitis: acyclovir resistant case in a patient with acute myelogenous leukemia.

Herpes simplex virus (HSV) infections in an immunocompromised host may be atypical in location and morphology. Lesions are more extensive and aggressive, slow healing or nonhealing and extremely painful. Intraoral lesions are ulcerative and may involve any intraoral, oropharyngeal, or esophageal site. Herpetic geometric glossitis is a recently described form of lingual HSV infection in an immunocompromised patient. It was described as ulcer on the dorsum of the tongue sensitive for acyclovir therapy. A patient is presented with acute myelogenous leukemia that developed herpetic geometric glossitis which was acyclovir resistant.