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PubMedDate not supplied

Conjunctival involvement with T-cell prolymphocytic leukemia: report of a case and review of the literature.

T-cell prolymphocytic leukemia is a rare and highly aggressive hematological neoplasm. A patient with T-cell prolymphocytic leukemia presented with bilateral perilimbal conjunctival infiltrates. Conjunctival biopsy showed aggregates of large atypical lymphocytes in the substantia propria with a concentration of atypical cells in the perivascular areas. Immunophenotyping of the malignant cells identified an abnormal clonal T-cell population consistent with T-cell prolymphocytic leukemia. A literature review of all reports of conjunctival involvement with leukemia was performed. The three cases of ocular prolymphocytic leukemia, including the one case of ocular T-cell prolymphocytic leukemia, are discussed in detail as well as 14 reported clinical cases of biopsy-proven conjunctival leukemia. The majority of cases occurred in the setting of acute leukemia, and conjunctival involvement was frequently a presenting sign of the disease or signified disease relapse. Conjunctival involvement with leukemia was consistent with good visual acuity; however, it portended a poor prognosis.

Alemtuzumab↗
PubMed2005

[Neutrophilic eccrine hidradenitis secondary to thioguanine in a neutropenic patient].

Neutrophilic eccrine hidradenitis (NEH) is an infrequent, self-limited inflammatory dermatosis characterized by a neutrophilic infiltrate around the eccrine glands. Clinically, it presents with different types of lesions. NEH occurs most frequently in patients who have undergone chemotherapy for hematologic neoplasms. We present a case of NEH in a 70-year-old neutropenic male who received thioguanine for acute myeloid leukemia. The erythematous plaques disappeared in 3-4 weeks. The histological findings were compatible with NEH. Skin cultures ruled out infectious causes.

Aged↗
PubMed2003

Interferon gamma levels in pleural fluid for the diagnosis of tuberculosis.

PURPOSE: To assess the utility of interferon gamma levels, including identification of the best cutoff for the diagnosis of tuberculosis. METHODS: We prospectively studied consecutive patients in a tertiary care, university-affiliated hospital who had pleural effusions. Interferon gamma levels were measured blindly by radioimmunoassay. The diagnosis of tuberculosis was established using prespecified standard criteria. RESULTS: Of the 595 patients with pleural effusions, 82 patients (14%) had tuberculosis. The area under the receiver operating characteristic (ROC) curve for elevated interferon gamma levels in the diagnosis of tuberculosis was 0.99 (95% confidence interval [CI]: 0.97 to 1.00). A cutoff of 3.7 IU/mL yielded a sensitivity of 0.98 (95% CI: 0.91 to 1.00) and a specificity of 0.98 (95% CI: 0.96 to 0.99). The areas under the ROC curves, and the test's sensitivity and specificity, were similar among patients of different ages and by percentage of lymphocytes in the pleural fluid. In 5 of the 28 patients with hematologic malignancies, interferon gamma levels were slightly above the cutoff; no patient with vasculitis or granulomatous diseases had levels higher than 3.7 IU/mL. The 14 immunocompromised patients and the 3 transplantation patients with tuberculosis had interferon gamma levels greater than the cutoff. CONCLUSION: Elevated pleural interferon gamma levels (>3.7 IU/mL) are very valuable in diagnosing pleural tuberculosis. Patients with pleural effusion due to hematologic neoplasms occasionally have levels slightly above the cutoff.

Aged↗
PubMed2002

Cardiac involvement in a case of acute lymphoblastic leukemia.

We present an extremely rare case of an immunocompromised patient with a T-cell acute lymphocytic leukemia relapse presenting as a right atrial tumor. Problems in diagnosis, vulnerability due to previous immunosuppression and bone marrow transplant, and successful surgical excision are highlighted. Cardiac involvement with hematologic neoplasms should be taken with more than academic interest, as it may be amenable to treatment.

Adult↗
PubMed2003

Neural network in hematopoietic malignancies.

BACKGROUND: In the last 30 years, the automatization of the hematology laboratory has led to a high increase in the number of full blood counts (FBC) performed daily as well as a very high degree in the quality assessment in terms of accuracy and precision. Moreover, the produced data are so many that their full interpretation needs an expert; often these new parameters are not exploited. The new challenge of the hematology laboratory consists in the translation of numerical data and new parameters into clinical meaningful information also offering, when possible, a pre-diagnostic guideline, maintaining similar high quality level also in these new aspects. METHODS: Since the first development of automated cytochemistry for leukocyte differential count, a new efficient pre-microscopic approach to leukemia diagnosis and classification, was made possible at the time of the automated blood cell count. This original method, used by the Bayer hematological H* series and ADVIA120 analyzers, is based on a light assessment of basic cell properties: volume and peroxidase activity (P) in the Perox channel, which is used to differentiate the main leukocyte types according to the different enzyme content and Nuclear density (ND) in the basophil channel, which is used to count basophils and blast cells according the chromatin pattern and to flag the presence of abnormal or immature cells. The simple observation of P and ND two-dimensional cytograms can be used to include any single case in a separate and distinct diagnostic category correlated to FAB and WHO classifications of hematological malignancies. Moreover, on the RBC map, it is possible to obtain some information about the pathological cluster. The simultaneous observation of these three cytograms and the introduction of a simple and quick score provide objective informations on blast lineage, level of myeloid differentiation, chronic versus acute leukemias. Using a simple visual score system, we have reached in a previous study a pre-diagnostic efficiency in 91% of the analyzed samples. ADVIA120 provides cytogram cell distribution at the end of the analytical processes that include the evaluation of 492 signals, the so-called raw data. The working hypothesis is to create a knowledge-based system, an Artificial Neural Network able to directly handle these raw-data for producing classes of diagnostic probability with a high level of efficiency. The Bayer R&D team in Tarrytown has created an ANN software for connecting a set of input data to output through weighted "hidden layers" (i) assessing the 84 ADVIA120 parameter sets to be used, (ii) defining the interim analysis tool for fitting to standard "normal archetypes", (iii) finding a discriminant function normal vs. pathological. We have collected from 22 Italian hematological centers data from peripheral blood of 1000 patients having mainly hematopoietic disorders at diagnosis. RESULTS AND CONCLUSIONS: The ANN has been trained with labeled samples. Same analysis has been performed for constructing the "pathological archetypes". Finally, we have tested the ANN model with a small selected set of the collected pathological samples. The preliminary encouraging results show the high capability of this ANN of clustering signals according to the pre-defined normal as well as pathological archetypes. The next big step is to create an application for discriminating different types of anemia, simply using data from patient's peripheral blood.

Hematologic Neoplasms↗
PubMed1991

Management of patients with myelodysplastic syndromes.

The myelodysplastic syndromes are composed of a group of clonal hematologic neoplasms, the course of which is complicated by ineffective hematopoiesis or leukemic transformation (or both). Therapeutic results may have been misleading in the past. Most patients have been managed with supportive measures, such as transfusions of erythrocytes, administration of antibiotics, and transfusions of platelets during active bleeding episodes. These supportive measures have prolonged and improved the quality of life of patients with myelodysplastic syndromes. Vitamin supplementation (folate, vitamin B12, and pyridoxine) has seldom been rewarding. Differentiation agents such as cis-retinoic acid and 1,25-dihydroxyvitamin D3 have been of benefit in only a limited number of patients. Androgens have not been useful, although danazol, which is an attenuated androgen, has been effective in a subset of patients with the presence of cell-bound platelet antibodies. Low-dose cytarabine, which has been studied extensively because of its differentiating activity in vitro, is associated with a generally low rate of complete remission and substantial toxicity. Antileukemic therapy is generally useful in young patients with rapidly progressive disease. Several hematopoietic growth factors are currently being evaluated in clinical trials; their use in combination or in conjunction with chemotherapy may be opening new horizons for these patients. With improvements in the prevention and treatment of graft-versus-host disease, allogeneic transplantation is a viable option for patients younger than 55 years of age who have severe cytopenias.

Humans↗
PubMed2001

Classes of c-KIT activating mutations: proposed mechanisms of action and implications for disease classification and therapy.

Mutations causing constitutive activation of KIT have been shown to be causative in some forms of mastocytosis, and several types of mutations have been associated with myeloproliferative disorders (MPDs), acute myelogenous leukemia (AML), sinonasal lymphomas, and gastrointestinal stromal tumors (GIST). We divide these activating mutation into two types - 'regulatory type' mutations, which affect regulation of the kinase molecule, and 'enzymatic pocket type' mutations, which alter the amino acid sequence directly forming the enzymatic site. KIT inhibitors have been suggested as therapeutic drugs for these conditions, but different types of activating mutations respond differentially to KIT inhibitors, so classification of individuals on the basis of specific mutations is necessary to guide therapy.

Acute Disease↗
PubMedDate not supplied

Reactivity of anti-AraC-resistant cell monoclonal antibody, YU-311, in formalin-fixed paraffin-embedded specimens of various hematopoietic disorders.

YU-311 is a monoclonal antibody reacting with cytosine arabinoside (AraC)-resistant human leukemic cell line and identifies a 92 kDa membrane protein. We have examined YU-311 reactivity with various hematopoietic disorders by an immunohistochemical method and evaluated a correlation between YU-311 expression and refractoriness to chemotherapy, retrospectively. YU-311 reacted with AraC-resistant human leukemia cell lines, in which a 92 kDa membrane protein was identified by Western blotting, whereas drug-resistant cell lines to other than AraC failed to express YU-311 antigen. The frequency of YU-311 positivity was significantly increased in relapsed cases. Only five cases were positive for YU-311 at diagnosis and 24 cases at relapse. Unexpectedly, only eight cases of relapsed leukemia/lymphoma expressed YU-311 and P-glycoprotein simultaneously. Most of the YU-311-positive relapsed cases showed clinical refractoriness for chemotherapy and then failed to induct complete remission or relapsed at short periods with short disease-free duration. These findings indicate that YU-311 expression is closely associated with some aspects of drug resistance, especially with AraC resistance.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2001

Amplification of the AML1(CBFA2) gene on ring chromosomes in a patient with acute myeloid leukemia and a constitutional ring chromosome 21.

In the genesis of hematologic neoplasms gene amplification is a mechanism for illegitimate activation of proto-oncogenes. We report a phenotypically normal patient with a constitutional ring chromosome 21 who developed acute myeloid leukemia (AML). The leukemic cells revealed size-variable ring chromosomes 21 with amplification of the proto-oncogene AML1, located in the chromosomal band 21q22, within the rings. Hitherto, amplification of the proto-oncogene AML1-also in form of a ring chromosome-has been described recently only in one patient with myelodysplastic syndrome (MDS). In AML, gene amplification by ring formation has been demonstrated only in another three patients (amplification of the MLL gene in two cases and of the ETV6 gene in one case). Here we present the new evidence that the internal rearrangement of a constitutional ring chromosome 21 resulted in multiplication of a proto-oncogene in bone marrow cells and provided obviously a selective growth advantage. Moreover the amplification of ribosomal DNA was observed in the ring chromosomes of the tumor cells.

Acute Disease↗
PubMed2001

FISH characterization of t(8;12)(q12;p13) observed as the sole karyotypic anomaly in a myelodysplastic syndrome patient.

We report a t(8;12)(q12; p13) as the sole cytogenetic anomaly in a patient with a myelodysplastic syndrome (MDS). By means of FISH, we mapped the genomic region involved in the breakpoint (bkp) on both chromosomes. The 12p13 bkp mapped between markers WI-664 and WI-9218, immediately distal to the breakpoint cluster region frequently involved in hematological neoplasms targeted by y964C10. The 8q12 bkp (not yet investigated by FISH) was characterized and found to occur between markers WI-3263 and D8S524 within the region recognized by y874E10.

Aged↗
PubMed1998

Two new cases of near-tetraploidy in adult acute myeloid leukemia.

Tetraploid or near-tetraploid karyotype has been described rarely in hematologic neoplasms. Herein we report two new cases of adult acute myeloblastic leukemia, M0 and M1 FAB subtypes that showed near-tetraploid clones that were studied with conventional cytogenetics and in situ hybridization (ISH). We compare our new cases with those previously reported.

Aged↗
PubMed1997

Expression and identification of aberrant c-kit transcripts in human cancer cells.

We have previously cloned and sequenced a novel 3.5 kb c-kit mRNA expressed in a colon carcinoma cell line Colo201. Here we examined the expression of this truncated form of c-kit in 14 gastrointestinal cancer cells and 16 hematopoieic cancer cells by RT-PCR. Expression of the aberrant c-kit transcript was observed in various cancer cell lines. Furthermore, a new transcript which is 78 bp shorter than the transcript previously described was identified and characterized. These results indicate that two kinds of aberrant c-kit transcript produced by alternative promoter in intron 15 are expressed in human cancer cells.

Adenocarcinoma↗
PubMed2004

[Results of a survey on practices in onco-hematology in France in 2001].

Further to the organisation of out-patient management, it appeared necessary to understand the type of organisation of the hospital teams working in oncology and haematology. Questions were asked on the medical workforce in the hospital, the rules for the organisation of the management fo these patients, the type of organisation in the patients homes and the choice of treatment. THE PERCENTAGE OF RESPONSE: Thiry-one centres (46%) replied to the survey: 20 hospital centres with 5 departments of paediatrics and 11 oncology departments. Around two thirds of the centres in France confronted with short lasting neutropenia with fever opted for their treatment at home. WHAT IS IMPORTANT: Although the responsibility of those surrounding the patient and that of the treating physician are clearly implied, the general practitioner frequently has no access to training or to the information necessary to be able to manage the initial assessment and the potentially unfavourable evolution. And this is the pivotal point of the external hospital treatment system.

Ambulatory Care↗
PubMed2000

Meta-analysis in hematology and oncology.

In 1992, a review article about meta-analysis identified only 15 meta-analyses of randomized, controlled trials of cancer therapy. Since then, the total number of meta-analyses in this field has increased almost sixfold. More importantly, the number of randomized, controlled trials in this discipline has also grown tremendously. The expansion in the literature will provide a fertile ground for future meta-analyses. The quality of the recent publications has also improved. An ongoing world-wide effort, the Cochrane Collaboration, is systematically assembling and synthesizing several hundred thousand randomized, controlled trials to improve the delivery of health care. Meta-analysis has many important advantages. It allows the viewing of the complete picture of the evidence. The advent of meta-analysis has sensitized researchers to issues of quality and has improved methodology in clinical research. Detection and explanation of bias and heterogeneity are prime objectives of meta-analysis in clinical research. An array of methods has been developed that allows a better understanding of bias and heterogeneity, beyond simple averaging of results from diverse studies. Meta-analyses of individual patient data, in particular, may promote the development of international collaborations. Several examples of their application are already available in oncology. Meta-analysis may point out deficiencies in the study design of past and current studies, suggest the need for new studies, and inform researchers about the size and design of these studies. In the end, meta-analysis helps to integrate evidence and make recommendations for medical care and medical practice.

Clinical Trials as Topic↗