Myelodisplastic

Acute myeloid leukaemia (AML) is mainly affecting elderly patients. Elderly patients are increasingly affected by impairment of functional status (FS). FS is of prognostic relevance for survival in different tumours. Data for patients with AML are rare. Within a prospective trial we recruited patients with newly diagnosed AML and measured FS by two different methods: Karnofsky performance status (KPS) and instrumental activities of daily living (IADL). Sixty-three patients aged 19-85 years (median 61.1) were included...

Myelodisplastic syndromes (MDS) are clonal hematopoietic disorders, characterized by ineffective hemopoiesis resulting in single or multiple lineages and a high risk of conversion to acute leukemia. Currently, the only established therapy with curative potential for MDS is a hemopoietic stem cell transplant (HSCT). Their results are determined by the type of MDS, age at the BMT and the score according to the international index. In the main studies the disease-free survival (DFS) were 35-43%, relapse 20 to 39% and transplantation-related mortality (TRM) 36-45%...

Several studies have demonstrated the existence of an autonomous intranuclear phospho-inositide cycle that involves the activation of nuclear PI-PLC and the generation of diacylglycerol (DG) within the nucleus. Although several distinct isozymes of PI-PLC have been detected in the nucleus, the isoform that has been most consistently highlighted as being nuclear is PI-PLC-beta1. Nuclear PI-PLC-beta1 has been linked with either cell proliferation or differentiation. Remarkably, the activation mechanism of nuclear PI-PLC-beta1 has been shown to be different from its plasma membrane counterpart, being dependent on phosphorylation effected by p44/42 mitogen activated protein (MAP) kinase...

A distinct polyphosphoinositide cycle is present in the nucleus, and growing evidence suggests its importance in DNA replication, gene transcription, and apoptosis. Even though it was initially thought that nuclear inositol lipids would function as a source for second messengers, recent findings strongly indicate that lipids present in the nucleus also fulfil other roles. The scope of this review is to highlight the most intriguing advances made in the field over the last few years, such as the possibility that nuclear phosphatidylinositol (4,5) bisphosphate is involved in maintaining chromatin in a transcriptionally active conformation, the new emerging roles for intranuclear phosphatidylinositol (3,4,5) trisphosphate and phosphoinositide 3-kinase, and the evidence which suggests a tight relationship between a decreased level of nuclear phosphoinositide specific phospholipase C-beta1 and the evolution of myelodisplastic syndrome into acute myeloid leukemia...

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Benzene causes many kinds of blood disorders in workers employed in many different environments. These diseases include myelodisplastic syndrome and acute and chronic myelocytic leukemia. In the present study, five occupational work places, including six industrial process types, namely, printing, shoe-making, methylene di-aniline (MDA), nitrobenzene, carbomer, and benzene production were selected, and the levels of breath benzene, and trans,trans-muconic acids (t,t-MA) and phenol in urine were evaluated, as well as hematological changes and lymphocyte DNA damage...

Excessive apoptosis has a central role in ineffective hematopoiesis in myelodysplastic syndrome (MDS). The aim of the study was to quantify apoptosis and Bcl-2 expression in patients with MDS and to use these parameters in the evaluation of treatment efficacy with compounds modulating proapoptotic cytokines. Bone marrow (BM) samples from eight MDS patients were studied: four with refractory anemia and four with refractory anemia with ringed sideroblasts. Two patients with Hodgkin disease without BM determination were studied for control...

The immunophenotyping of leukaemia and non-Hodgkin's lymphoma cells is based on staining the cells with monoclonal antibodies against surface and cytoplasmic determinants followed with flow cytometry analysis. The problems of immuno-phenotyping are associated with technical difficulties, changes in expression of determinants and the rare types of leukaemia and haematological disorders typical for newborns and infants. The lack of blast cells within cell suspension obtained for test may be the result of bone marrow disorder (aplastic anaemia, preleukaemic cytopenia) or technical pitfall...

No abstract text is available yet for this article.

Topotecan, a semi-synthetic derivative of the alkaloid camptothecin is an antitumor drug that like other camptothecin derivatives, targets DNA topoisomerase I, an enzyme that is present in cells in concentration relatively independent of the stage in the cell cycle. Topotecan stabilizes the complex formed between topoisomerase I and DNA, leading to DNA strand breakage and cell death. In accordance with preclinical studies, clinical efficacy of topotecan was documented in ovarian carcinoma, in small cell lung cancer and in childhood solid tumors...

Growth factors (GF) are reported to play an important role in the therapy of myelodisplastic syndromes (MDS). After in vitro administration a consistent group of MDS may respond to GF but the possibility of differentiation, regulation or expansion of myelodisplastic clones following GF therapy is still a question to be answered as their optimum dose and combinations. To validate if in vivo treatment with GF, may promote the regulation or the recovery of myelopoiesis and/or modify the clonality of the responses, we gave G-CSF after intensive chemotherapy in high risk MDS and acute leukemia evolving from MDS patients...

A study of paediatric enterocystoplasty in 22 children of 1 to 14 years of age (M = 8) during 5 years is presented. The diagnoses were: neurogenic bladder 16 (73%), extrophic bladder 3, urethral valves 1, and complex uropathies 2. The intestinal segments used were: ileum 12 cases, sigmoid 8, and ileocaecal region 2 (non myelodisplastic). During the last years sigmoid have been preferred due to its bigger size. The middle follow up is 3.5 years (R 1-6). The results have been good in: renal function 86%, and upper urinary tract 95%...

A multicentric study about the first year of life of myelodisplastic children, was done. Family, pregnancy, delivery, newborn somatometry, and anatomical-pathological and terapeuthical aspects were review in 393 patients born before july 1992. Annual prevalence vary from 6 (1973) to 28 (1984). The families clinical history was not significant. There were 9 (2,3%) gemelar pregnancies, but both children were affected only in one case. Prenatal ultrasound was not diagnostic in 61%-84% of patients are meningomyeloceles, the anatomical level of myelodisplasia was lumbar and or sacral in 98%...

Myelodisplastic syndromes (MDS) in childhood deserve a negative prognosis even though disease-free survival has been obtained in 20% of cases by using aggressive chemotherapy. We describe three children with refractory anemia with excess of blasts in transformation (RAEB-T) who underwent bone marrow transplantation (BMT). We also reviewed 21 additional cases (median age was 8 years) with primary MDS recently reported in the literature with the aim of clarifying the role of BMT in treating these patients. Twelve of the 24 children were long-term survivors and free from disease at a median time of 1,320 days (range 302-2,340)...

Since the beginning of cytogenetics, there has been a constant improvement of chromosomal culture and banding techniques. In 1976, Yunis described a high chromosomal resolution technique (HRC), that permits the detection of subtle chromosomal abnormalities. The present work, reports the results obtained when HRC was applied to the study of chromosomal abnormalities in patients with high risk of such. The study comprised 434 specimens of venous blood and 182 bone marrow aspirates. The samples were classified according to the presuntive diagnoses...

The aim of the present study was to evaluate the significance of immunomorphometric assessment of megakaryocyte size and number in normal and pathologic human bone marrow. Thus, we compared morphometric characteristics of megakaryocytes in 56 bone marrow trephine biopsies stained by immunohistochemical and conventional techniques. Morphometric results showed that precise megakaryocyte size in normal and pathologic samples can be calculated even by using conventional staining technique, but only employing specific stereological corrections...

The authors while describing their patients suffering from osteopetrosis disease, discuss its morphological aspects and possible patho-mechanism. The disease with osteosclerosis can be inherited recessively or dominantly. The recessively inherited type is less frequent and leads to early death due to secondary developing myelofibrosis. The dominantly inherited form is more benevolent, the patients are free of symptoms in half of cases. The patients described by the authors belong to the dominantly inherited type of the Albers-Schönberg disease...

CD86 (B70/B7.2) is an antigen of the immunoglobulin superfamily expressed on monocytes, dendritic cells and activated B, T, and natural killer cells. CD86 was recently identified as a second ligand for the T cell antigens CD28 and CTLA-4, and plays an important role in the co-stimulation of T cells in a primary immune response. We report here the assignment of the CD86 gene to human chromosome 3 using Southern blot analysis on a panel of hamster x human somatic cell hybrid genomic DNA. Fluorescence hybridization in situ on metaphase chromosomes coupled with GTG banding (G-bands by trypsin using Giemsa staining) confirmed this assignment and localized the CD86 gene to 3q13-q23 region...

The properties of human bone marrow fibroblastoid colonies (CFU-F) were studied in normal subjects and in patients with myeloproliferative disorders. Colony incidence was within normal values in all groups of patients analyzed except for myelodisplastic syndromes, with higher mean value. The growth rate of CFU-F is inversely related to the initial colony-forming efficiency both in normal subjects and in patients. Direct correlation between CFU-F and granulocyte-macrophage colony-forming unit (GM-CFU) was detected only in normal subjects, but lacked in patients...

Seven patients were treated with low dose Ara-C (LDAC). Five patients had acute nonlymphoid leukemia (ANLL), two patients had myelodisplastic syndrome (MDS): refractory anemia (RA) and refractory anemia with excess of blasts in transformation (RAEB-t). Ara-C treatment was given by s.c. injections at a dose of 10-11 mg/m2 every 12 h and only on two occasions by continuous infusion. No improvement, or limited improvement, was observed in five patients and they died of leukemia or of disease-related complications...