Biomedical subjects
Publications and source records attributed to M Recio.
An account is given of a case of hairy-cell leukaemia associated with a 'lupus-type' anticoagulant and a positive direct Coombs' test, both of which were clinically symptom free. This is yet another example of the coexistence of hairy-cell leukaemia and an auto-immune disorder, but the disorder in question has not been described previously.
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A new case of acquired von Willebrand's syndrome (vWS) is described in a 31-year-old woman with a hydatid splenomegaly and with a history of repeated abortions at an advanced stage of pregnancy, a positive serology for syphilis and a mildly elevated titre of antinuclear antibodies, with no family history of bleeding. There is an inhibitory effect on factor VIII: C (antihaemophilic factor) as well as on factor VIIIR: Ag (related antigen) and on factor VIIIR: RCo (ristocetin cofactor), and it is precipitated by rabbit anti-IgG antiserum. This inhibitory effect was demonstrated using the patient's plasma heated to 56 degrees C for one hour so as to dissociate circulating immunocomplexes. All the abnormalities of haemostasis, as well as the positive serology for syphilis, disappeared after splenectomy, and the ANA titre reverted to normal. The clinical and biological peculiarities of the case are discussed, and are interpreted in the light of the findings recorded in patients showing "lupus" anticoagulant.
An ultrastructural study of the aspirated bone marrow of a patient with congenital dyserythropoietic anemia type I is presented. Both in transmission electron microscopy (TEM) and in scanning electron microscopy (SEM) ultrastructural abnormalities of the bone marrow erythroid precursors were seen. These abnormalities in TEM included uneven condensation of chromatin with spongy appearance and alterations of cellular division. The most striking among these alterations were: firstly, the intercellular bridges which were typified by the presence of chromatin and the absence of the midbody and contractile ring and, secondly, the anomalies of the nuclear membrane. Occasionally, autolytic areas within the cytoplasma and iron-laden mitochondria were to be seen. In the SEM the following features were notable: the length of the intercellular bridges, the absence of the central ridge in the midbody area, as well as the scanty number of cytoplasmic blebs on the surface of the bridges.
A morphologic study was made of the cell population which had infiltrated the bone marrow of a five-year-old boy. These cells showed a tendency to form rosette-like structures. These structures as well as the presence (at ultrastructural level) of neurosecretory granules, cell processes, and microtubules in the neoplastic cells led to a diagnosis of neuroblastoma. Certain characteristics, not previously reported in neuroblastoma, were identified, such as gap junction type intercellular contacts, paracrystalline arrays in mitochondrial atpyical cristae and nucleolus-like bodies (nematosomes). Gap junctions are involved in the intercellular transfer of ions and low molecular weight metabolites and may explain the tendency to form cellular cluster in "rosettes" which are characteristic of this neoplasm. The presence of nematosomes in the tumor cell cytoplasm is one more piece of evidence which substantiates the nervous origin of these cells.
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Mediastinal involvement was found in 11 (group A) of 43 patients affected with acute lymphatic leukemia when early thoracic roentgenograms of these patients were reviewed. Several clinicobiological characteristics of these patients were compared with those with a normal mediastinum as shown in their roentgenograms (group B). Statistically significant differences were observed between group A and group B not only as far as age was concerned (13 years group A, 7.4 years group B) but also in the ratio males/females (10:1 group A, 10:20 group B) and with regard to the leukocyte counts (266 x 10(9)/liter group A, 20 x 10(9)/liter group B). In addition, patients from group A showed a greater 'tumoral mass' and a more prominent extrahematological involvement (45% group A, 15% group B). In these cases 'convoluted' cells were frequently discovered and the blastic cells exhibited significantly lower scores of PAS-positivity and more marked acid phosphatase activity than those in group B. Although the rate of complete remissions (CR) obtained in both groups was similar (80% group A, 93% group B), marked differences were observed not only in the duration of CR but in the period of survival after CR as well, both factors being more prolonged in group B patients. 50% of patients with mediastinal involvement (group A) relapsed in the first 6 months of the evolution of the disease.
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