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Biomedical subjects

L Florensa

Publications and source records attributed to L Florensa.

At least 19 recordsLinked to original sources

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

Immunocytochemical investigation of normal and chronic lymphocytic leukaemia lymphocytes reveals unexpectedly frequent reactivity with some myelomonocytic associated antibodies.

Information about the expression of some myelomonocytic markers in lymphocytes of patients with B-CLL is scarce. We studied the CD13, CD14, CD11c and CD68 surface antigens in 42 controls and in 38 patients with B-CLL to detect their possible reactivity. Eighty-nine percent of B-CLL expressed very strongly the CD14 antigen; on the contrary, the other myelomonocytic antigens tested were very weakly expressed. Forty-one of 42 controls showed a few CD14-positive lymphocytes with a statistical difference between normal and CLL lymphocytes. No statistical difference was recorded either between CD14 expression and Rai's staging system or Binet's stages, nor between CD14 and bone marrow involvement and doubling time or between CD14 and heavy or light chain expression. A minor B lymphocytic subset in humans coexpresses the CD14 and CD5 antigens, it being increasingly speculated that B chronic lymphocyte leukaemias originate precisely from this B CD5- and CD14-positive cells. Just as the CD5 antigen is regarded as an excellent B-CLL marker, it seems to us that a strong expression of the CD14 antigen might have the same diagnostic relevance.

Antigens, CD

Cytogenetic studies in 112 cases of untreated myelodysplastic syndromes.

Cytogenetic studies were performed in 112 untreated cases of myelodysplastic syndrome (MDS) between 1985 and 1990. Among 112 patients who were examined at the time of diagnosis, 54 had an abnormal karyotype (48%). The highest frequency of chromosome abnormalities was observed in refractory anemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-t) and the lowest in refractory anemia with ring sideroblasts (RARS) and chronic myelomonocytic leukemia (CMMoL). Numerical changes were observed in 19 cases and structural in 17; chromosome 8 was most frequently gained (11 cases), whereas chromosome 7 was most frequently lost (6 cases), 5q- in 14 (4 as a sole anomaly); involvement of 7q22 was seen in 3 cases, 11p in 2 patients, 11q in 3 (one patient as a sole anomaly), 12p in 4 (2 patients as a sole anomaly), i(17q) in 4 (3 patients as a sole anomaly), and complex chromosomal defects in 10 patients. If one takes into account the prognosis value, a complex karyotype and the presence of ring chromosomes were correlated with the worst prognosis, followed by -7/7q-; an intermediate prognosis corresponds to i(17q), 12p as a sole anomaly, +8 (as a sole anomaly or plus other anomalies), and involvement of 12p. Patients with a 5q- as a sole anomaly or with a normal karyotype, had the best prognosis.

Adolescent

[Description of 2 patients with cytogenetically abnormal clones].

We present two patients with two cytogenetically unrelated clones. A patient was diagnosed of refractory anaemia and showed an abnormal clone with trisomy 8 and other clone with 5q-; the other patient, diagnosed as chronic lymphocytic leukaemia showed a clone with an inversion of chromosome 2, inv(2) (p23q12) and the other clone with a 47,XX,+5,t(16;17)(p13;q11),+2ac karyotype. The discussion is focused on the presence of unrelated clones in relation to the monoclonal origin of cancer.

Aged

Acquired amegakaryocytic thrombocytopenic purpura associated with immunoglobulin deficiency.

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a haematological disorder characterized by severe thrombocytopenia due to an immunologically induced absence of megakaryocytes in an otherwise normal-appearing bone marrow. A 57-year-old male with a 6-month history of rectal and cutaneous bleeding is reported. Platelet count was 10 X 10(9)/l, while other haematological values were within the normal range, except for the presence of hypogammaglobulinaemia with decreased IgA and IgG. Both platelet median volume and half-life span were normal, and antiplatelet IgG determinations were negative. Bone marrow aspiration and biopsy showed no megakaryocytes, with a normal appearance of erythroblastic and granulopoietic series. An in vitro culture for megakaryocytic progenitor cells did not show any growth of megakaryocyte colonies. No inhibitory effect on the growth of normal marrow megakaryocytic colonies was observed when serum and lymphocytes of the patient were added. Following 4 weeks of prednisone therapy, the platelet count rose to 127 X 10(9)/l and the bone marrow aspirate showed some megakaryocytes. The possible pathogenetic mechanisms of this entity are discussed.

Humans

[Isochromosome 17q as the sole alteration in 2 patients with a myelodysplastic syndrome and its relation to myeloperoxidase activity].

Two patients diagnosed of myelodysplastic syndrome with an isochromosome i (17q) as the only chromosomal alteration are presented. The MAC method (i.e., morphology, antibodies, chromosomes) was applied in the study of one of the patients, it being found that all the myeloperoxidase-positive cells carried the i (17q) anomaly. Such finding might suggest that those patients with i (17q) as the only chromosomal alteration could have specific clinical and cytological features.

Aged

[Cytochemical detection of lymphocyte 5'-nucleotidase in chronic lymphatic leukemia].

5'-Nucleotidase is a degrading purine ectoenzyme acting at alkaline pH. It is located in both B and T lymphocytes and its study is of interest in chronic lymphoproliferative diseases. The present work compiles the cytochemical study of lymphocyte 5'-nucleotidase in a control group consisting of 277 haematologically normal subjects and a series of 77 chronic lymphocytic leukaemia (CLL) patients; phenotypic studies had been carried out in 40 of these last. The results were expressed as percentage of 5'-nucleotidase positive lymphocytes, and the value (means +/- SD) for the control group was 25 +/- 7, that of the CLL group being 10.7 +/- 18.12. Increased lymphocyte 5'-nucleotidase was present in a minority of the cases (13%), but the significance of this finding is unknown and unrelated to any clinical or cytomorphological data. Although lacking any statistical value, those B-CLL lymphocytes expressing surface IgM and IgD (thus being more mature cells) showed higher 5'-nucleotidase values than those cells expressing only IgM. This finding suggests that a given lymphocytic population would be more immature the lower 5'-nucleotidase value it may express. The incorporation of 5'-nucleotidase determination into the cytochemical study of CLL is encouraged as it is frequently decreased in this disease; at the same time, the enzyme may provide some information on the maturity of the leukaemic population involved.

5'-Nucleotidase

[Erythroid colonies derived from BFU-E from the bone marrow in a patient with type I congenital dyserythropoietic anemia].

The findings of in vitro culture of bone-marrow BFU-E from a patient with type I dyserythropoietic anaemia are reported, scarce data being seemingly available in the literature. The number of BFU-E in the culture was increased four-fold with respect to the normal values. The morphologic study of the colonies showed in all cases varying number of erythroblasts with internuclear bridges (5-20%). Upon ultrastructural examination of the colonies, a great number of erythroblasts exhibited morphologic alterations, spongy chromatin and internuclear bridges being commonest. These findings suggest that an alteration of the progenitor erythroid cells exists in type I dyserythropoietic anaemia, whereas the morphological defects appreciated show great variation in the progeny of each BFU-E.

Adult

[Pseudohemopathy caused by rhabdomyosarcoma].

A 26-year-old male with a primary rhabdomyosarcoma of maxillary sinus is reported. The tumor was initially mistaken for an anaplastic Ki-1 positive anaplastic lymphoma in the histological preparation, owing to its high indifferentiation degree. After a smear study it was categorized as IEA, and chemotherapy of CHOP type was started; after two courses, local telecobalt therapy was given. After this was completed, the disease showed a progression, involving the bone marrow and resulting in clinical and cytological features consistent with acute leukemia. At that time we saw the patient for the first time. He is presently in complete remission after having started polychemotherapy of CVADIC type. After a commentary on the major study steps that led to the diagnosis, the crucial role of electron microscopy and, particularly, immunocytology for the correct identification of anaplastic tumors is emphasized.

Adult

Circulating erythroid and megakaryocytic progenitors in polycythaemia vera and essential thrombocythaemia.

We studied the behaviour in culture of erythroid and megakaryocyte progenitor cells (BFU-E, CFU-MK) obtained from peripheral blood (PB) in 38 patients: 15 with essential thrombocythaemia, 3 with reactive thrombocytosis, 16 with polycythaemia vera and 4 with secondary polyglobulia. Clonal erythroid growth without added erythropoietin was observed in all patients with polycythaemia vera and in 5 out of 15 with essential thrombocythaemia, but in none of the patients with reactive thrombocytosis or secondary polyglobulia or in controls. When the CFU-MK were cultured without phytohaemagglutinin-stimulated medium (PHA-LCM), all patients with essential thrombocythaemia and 7 out of 16 with polycythaemia vera showed circulating CFU-MK but none of those with reactive thrombocytosis or secondary polyglobulia or controls did so. This study indicates that the growth in vitro of megakaryocytic and erythroid progenitors from such a readily available source as peripheral blood can be valuable in the diagnosis of certain borderline cases of thrombocytosis or erythrocytosis.

Cells, Cultured

[Erythroblastopenia associated with chronic myelomonocytic leukemia].

A patient is presented in whom the diagnoses of chronic myelomonocytic leukaemia (CMML) and erythroblastopenia were simultaneously established. Besides the conventional criteria for both haemopathies, the culture of bone-marrow precursor cells showed lack of growth of the erythroid stem cells. 6-Mercaptopurine given as therapy for CMML failed to induce any favourable changes in erythroblastopenia, which in turn improved with prednisone. Nevertheless, the patients died five months after diagnosis due to acute transformation of the CMML.

Aged