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Biomedical subjects

L Florensa

Publications and source records attributed to L Florensa.

At least 73 records · Page 4Linked to original sources
PubMed1994

Cytogenetic studies in five patients with Sézary syndrome.

A cytogenetic study was performed in five patients with Sézary syndrome. Metaphases were obtained from a phytohemagglutinin-stimulated lymphocyte culture. The five patients showed abnormal karyotypes. The chromosomes preferentially involved in numerical aberrations were chromosomes 10 (monosomy) and 13 (monosomy); involved in structural changes were chromosomes 1, 2, 4, 6, and 14. In our series, all patients showed progression of the disease.

Aged↗
PubMed1994

Granular lymphocyte proliferative disorders: a multicenter study of 20 cases.

A series of 20 patients with granular lymphocyte proliferative disorders (GLPD) is reported. The criterion of inclusion was presence of persistent (> or = 6 months) granular lymphocytosis in the absence of any causative illness. Diagnoses made upon analytical control in half the patients of splenomegaly (25%) and hepatomegaly (25%) were infrequent. Clinical course was nonprogressive in 17/20 patients, but two developed high-grade NHL several years later and one showed progressive disease. Actuarial probability of survival at 5 years was 85%. Granular lymphocyte morphology was relatively homogeneous, and peripheral blood counts were preserved in the most patients. Bone marrow lymphocytic infiltration was low, as assessed by bone marrow aspiration and/or biopsy. Eosinophilia was an outstanding feature in eight cases. Ultrastructurally, all cases showed parallel tubular arrays; cytoplasmic granules and numerous short microvilli were noticed. The lymphoid phenotype was heterogeneous, the most common being CD2+CD3+CD4-CD8+, but six patients (30%) were CD4+ with variable expression of natural killer-associated antigens. Chromosomal analysis was abnormal in 4/10 patients [trisomy 19, t(5;6); inv(14) and inv(10)]. The study of beta-chain of the T-cell receptor revealed clonal rearrangements in 14 (78%), restricted to CD3+ patients (92%). In vitro culture of myeloid precursors showed decreased CFU-GM in 5/6 patients. Virological studies for HTLV-I and II were negative. In conclusion, the presence of a clonal proliferation was not correlated with the clinical course or an associated disease.

Aged↗
PubMed1994

Congenital dyserythropoietic anemia type II: morphological characterization of the erythroid colonies (BFU-E) from the bone marrow and peripheral blood of two patients.

The numerical and morphological findings of erythroid burst colonies from the peripheral blood and bone marrow of two patients with congenital dyserythropoietic anemia type II (CDA-II) are described. In both patients there was an increase of medullary and peripheral BFU-E that was explained by a compensating mechanism against the destruction of erythrocytes. In most of the colonies normal and abnormal erythroblasts co-existed. The ultrastructural analysis of erythroblasts showed, as in vivo, bi- and multinuclearity, autophagic vacuoles, and aberrant membranes that sometimes gave rise to the double-membrane appearance. These abnormalities were found in both patients simultaneously in the blood and bone marrow. These findings point to defective erythroid stem cells. The clinical expression of the disease may depend partly on the ratio of normal to abnormal erythroid colonies.

Adult↗
PubMed1994

Burkitt's type translocation in multiple myeloma.

We report a case of multiple myeloma with a t(8;22)(q24;q11) found during the progression of the disease. The relation between the association of a Burkitt's type translocation with cytological characteristic features is presented. To our knowledge, there is no report of a multiple myeloma with t(8;22)(q24;11).

Aged↗
PubMed1994

Chromosomal and in vitro culture studies in a case of primary plasma cell leukemia.

We present a cytogenetic study of a case of primary plasma cell leukemia (PCL) whose plasma cells were cultured in vitro with different mitogens. Cytogenetic studies demonstrated a reduction of the genome, monosomy of chromosomes 8, 13, and 22 being the most frequent. Neither structural changes nor marker chromosomes were observed. The hypodiploid karyotype was confirmed and confined to the neoplastic clone (lambda positive cells) by the MAC (Morphology, Antibody, Chromosome) method, which allows a simultaneous study of chromosomes, cell morphology, and immunologic phenotype. Stimulating the culture of plasmocytes in vitro with IL-6 + PHA, only normal metaphases could be obtained; on the other hand, a large number of abnormal metaphases were observed with the use of LPS as a mitogen. A surprisingly high yield of metaphases was obtained in this case, contrary to the rule in the in vitro growth of plasmocytic proliferations. Possible explanations of this fact are considered.

Adult↗
PubMed1994

Refractory anaemia with hypereosinophilia and clonal abnormal metaphases detected only in the neutrophilic granulopoietic series.

We report a case with refractory anaemia terminating in an acute leukaemia, which showed from the very beginning an intense eosinophilia that lasted for the whole disease, and in which the eosinophilic metaphases, as documented by the 'Morphology, Antibody, Chromosome' technique, were normal. An unusual karyotypic anomaly in the setting of a myelodysplastic syndrome could only be detected in the neutrophilic series. A general approach to detect structural aberrations of specific human chromosomes in metaphase cells by chromosomal in situ suppression hybridization of DNA libraries from sorted human chromosomes has been applied for chromosomes 11, 3 and 2, in order to identify an extra copy of chromosome 2.

Acute Disease↗
PubMed1994

Expression of lymphocyte function-associated antigen (LFA)-1 in B-cell chronic lymphocytic leukemia.

We investigated the expression of the lymphocyte function associated (LFA)-1 adhesion molecule, recognized by the CD11a and CD18 monoclonal antibodies, in 53 patients with B-cell chronic lymphocytic leukemia (B-CLL) and in 37 controls. The mean percentage of control lymphocytes expressing CD11a and CD18 positivity was 92 +/- 5.5 and 90 +/- 8.7, respectively and, in patients with B-CLL, 14.2 +/- 10.3 and 14.8 +/- 10.3 respectively (p < 0.001). No statistical difference was found between CD11a and CD18 expression and the Rai clinical stages. In our experience a decrease of LFA-1 is a constant finding in B-CLL, in the early stages of the disease. It may, therefore, be useful to differentiate between slight increases of neoplastic cells and reactive blood lymphocytosis.

Antigens, CD↗
PubMed1994

[Marrow hypoplasia associated with congenital dyskeratosis. Case report].

Congenital dyskeratosis is a rare disease involving ectodermal derived tissues and presenting bone-marrow hypoplasia as a complication in one half of the cases. A 25 year-old male is presented who at age 12 showed retarded development with shortness of the 4th finger of his left hand, anomalous implantation of teeth, hyperpigmented skin and hyperkeratosis on his knees, hands and feet. He had anaemia (Hb 92 g/L) and leucopenia (2.7 x 10(9)/L) with neutropenia (0.34 x 10(9)/L) and his bone-marrow showed hypoplasia, especially affecting granulopoiesis. The cytogenetic studies were normal. No treatment was given, and a haematological re-evaluation performed 13 years later showed no significant quantitative changes. Decreased number of myeloid and megakaryocytic colonies were present in the bone-marrow cultures. The clinical and laboratory characteristics of the bone-marrow aplasia associated to congenital dyskeratosis are commented, stress being laid on its differentiation from other constitutional forms of aplasia, especially Fanconi's anaemia.

Adult↗
PubMed1993

Isochromosome 17q as a sole anomaly: a distinct myelodysplastic syndrome entity?

We report four patients with myelodysplastic syndrome (MDS) with isochromosome i(17q) as the sole chromosomal anomaly. One patient was classified as refractory anemia (RA) and three as refractory anemia with excess of blasts (RAEB). All four patients shared several features such as male sex, advanced age, severe anemia, as well as a bone marrow with myeloproliferative characteristics: hypercellularity, prominent baso- and eosinophilia, and marked increase of micromegakaryocytes. We suggest that patients with i(17q) as the sole chromosomal anomaly may identify a distinct MDS with characteristics between MDS and chronic myeloproliferative disorders (CMPD).

Aged↗
PubMed1993

[Selective chromosome painting using in situ hybridization].

A method of in situ hybridization with entire chromosome-specific DNA libraries for visualizing individual human chromosomes has been developed and applied to the detection of structural aberrations in both metaphase and interphase cells. Unlabeled human genomic DNA is used to inhibit the cross-hybridization of repetitive sequences in the library that bind to multiple chromosomes. The remaining single-stranded DNA is hybridized to specimens of interest and detected with fluorescent or enzyme labeled biotin conjugates following post-hybridization washes. This general approach is called "chromosome painting" or "chromosomal in situ suppression (CISS)" hybridization. In the present report, DNA inserts from recombinant libraries from chromosomes 1, 4, and 9 has been applied on controls and patients in order to decorate specifically their complementary chromosomes. Numerical changes, deletions and chromosomal translocations involving these chromosomes can be strikingly visualized.

Cells, Cultured↗
PubMed1992

Myelodysplastic syndromes and malignant solid tumors: analysis of 21 cases.

We studied the association between myelodysplastic syndromes (MDS) and malignancies in a cohort of 155 patients with MDS, 21 of whom presented malignant solid tumors. Myelodysplasia was present after the diagnosis of cancer in eight patients (interval between the diagnosis of both conditions 18 months, median survival 49.5 months), simultaneously with diagnosis in 11 (median survival 8 months), and before malignancy in two patients (interval between the diagnosis of both conditions 47 and 7 months). One patient was given chemotherapy for lung cancer, and three patients received radiotherapy for adenocarcinoma of the kidney and cancer of the prostate. At the time of diagnosis of MDS, nine patients already presented metastatic spread. Fourteen patients died, ten as a result of tumor-related complications and four because of transformation to acute nonlymphocytic leukemia. The analysis of the incidence of malignancy in patients with MDS was statistically significant for males, and the relative risk was significant in both sexes. The results of this study show that MDS patients present a higher incidence of malignant tumors than the general population, that MDS may be of real paraneoplastic significance, and that the occurrence of MDS in cancer patients may be considered to be related to the malignancy rather than an independent phenomenon.

Adenocarcinoma↗