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J Sans-Sabrafen

Publications and source records attributed to J Sans-Sabrafen.

At least 73 records · Page 4Linked to original sources
PubMed1981

Congenital dyserythropoietic anemia type I. Report of a case.

A new case of congenital dyserythropoietic anemia type I is reported in a 12-year-old Spanish boy. In regard to the morphological study at an optical and ultrastructural level, the most outstanding feature was the internuclear bridging connecting two erythroblastic nuclei within a single cytoplasm or stretched between two erythroblasts perfectly individualized.

Anemia, Dyserythropoietic, Congenital↗
PubMed1979

[Argyriasis. Report of a case (author's transl)].

A case of argyriasis in a 45-year-old woman is reported. When the patient was 34 she took for a period of 25 months a silver-containing pharmacological product (colloidal silicon with 0.5 percent of silver) in order to treat an intestinal dyspepsia with diarrheic episodes. A few months after discontinuing the treatment a cutaneous pigmentation of a greyish-blue color extending over the whole body, developed. There was also discoloration of the nails, hair, and of the oral and gingival mucosae. The differential diagnosis with other conditions that also develop anomalies of cutaneous pigmentation was established. It is thought that the appearance of the silver poisoning is due to different factors such as the quantity of silver intake, the individual sensitivity to the metal, and the greater or lesser period of exposure to sunlight. The importance of the skin biopsy in order to confirm the diagnosis is commented on. Typical pathologic findings include the presence of silver granules in the basal membrane of sudoriparous glands, around the pilosebaceous follicles, and in the connective tissue. Lastly, the authors insist on the necessity to avoid the prescription of silver-salt containing drugs as far as possible, since the cutaneous pigmentation is irreversible.

Argyria↗
PubMed1979

[Complete clinical remission over 50 months of a patient with acute monocytic leukaemia (author's transl)].

A case of Schilling type pure acute monocytic leukaemia (M5 variety of the FAB classification) in a 38-year-old man is presented. The initial predominant clinical manifestation was an increase hypertrophic gingivitis. The cytological type was established according to the morphologic and cytochemical criteria currently in use. The patient was treated with daunomycin (60 mg/m2 one day) and ARA-C (100 mg/m2/12 hours for 7 days) in a 2-week interval cycles. Total remission was achieved after the fifth series. The series have been repeated over 4 years with intervals of up to 6 or 8 weeks. Complete haematologic remission has been constant, with normalization of the serum muramidase levels. A gingival biopsy taken at the same time has shown no evidence of leukaemic infiltration. The high incidence of malignancy in this cytological variety of acute leukaemia is pointed out; it is normally less sensitive to chemotherapy than the myeloid types. Remission has been total over a long period of time, which is exceptional in this type of leukaemia. The mean survival rate as recorded in the literature is only a little over 3 months.

Adult↗
PubMed1979

[Immunoblastic sarcoma (author's transl)].

A case of immunoblastic sarcoma in a 56-year-old man is presented. He had no history of predisposing diseases. His clinical condition was typical of a highly aggressive disseminated malignant lymphoma and he presented important heterogenous hypergammaglobulinemia. The patient died 9 months after the onset of the disease, following brief and incomplete response to various chemotherapeutic associations. The importance of cytological and cytochemical studies of lymph node by touch prep is stressed, since this condition could have been misdiagnosed, in our case, with a malignant histiocytosis. The cell proliferation was shown cytochemically to be of B-lymphoid origin, not histiocytic. It was a monomorphic and nearly massive proliferation of large, intensely basophilic, nonphagocytolytic cells; reactions to naphthol-As-D-acetate esterase, acid phosphatase, beta-glucuronidase, and Perl's stain were negative. The relatively few phagocytolytic cells were shown cytochemically to be normal, true histiocytes, not identifiable with the atypical proliferating cells. This was an essential fact in establishing the diagnosis of immunoblastic sarcoma. In light of today's knowledge, the authors believe that immunoblastic sarcoma is a lymphomatous condition which should be distinguished from centroblastic lymphadenopathy. Lastly, they comment on a retropsective study of lymphomas previously catalogued as reticulo-sarcomas, which has shown that the majority of cases were centroblastic lymphomas and some were immunoblastic sarcomas.

Diagnosis, Differential↗
PubMed1979

[Malignant hypereosinophilic syndrome. Report of an exceptional case (author's transl)].

The case of a 43-year-old man with a highly malignant hypereosinophilic syndrome is reported. The condition is classified as such according to Hardy and Anderson's criteria, accepted by many other authors. Other diseases of known etiology which may present high levels of eosinophils in the peripheral blood, such as parasitosis, allergies, neoplasias, collagenosis, etc., were discounted beforehand. The difficulties in distinguishing between these diseases are discussed; they are often accompanied by clinical manifestations which also arise in very different conditions including eosinophilic leukemia, Engfeldt and Zetterström's eosinophilic collagenosis, Löffler's fibroplastic endocarditis, etc. A particularly striking feature of this condition is the formation of large tumor masses of mature eosinophils. They begin in various bones, which they destroy almost completely, and invade the surrounding tissues, destroying them as well. These tumors act similarly to malignant eosinophilic myelocytomas, a fact which has not been reported previously in the literature as far as we know. Although the eosinophils act as though they were neoplastic, they maintain the characteristics of mature cells, both cytomorphologically and ultrastructurally as well as cytochemically (consistently chloroacetate esterase negative). The tendency to diagnose eosinophilic leukemia solely on the basis of the malignancy of the condition and a tissue infiltration of eosinophils without determining the existence of cytologic and/or cytochemical anomalies of the cells showing them to be leukemic is discussed. The authors were unable to find any reports in the literature in which the eosinophils were presented with unmistakeably blastic cellular characteristics. Various nosologic considerations are offered.

Adult↗
PubMed1979

[Chronic T-cell lymphocytic leukemia. Report of two cases (author's transl)].

Two patients with chronic lymphocytic leukemia of T-cell immunological origin are studied. One case was of the prolymphocytic variety, and the other corresponded to the "classical" type of chronic T-cell lymphocytic leukemia. From a morphological point of view what stood out was the hyperchromatic aspect of the cytoplasm of the lymphocytic proliferation. The high increase of acid hydrolases localized preferentially in the centrosomic area was the main cytochemical characteristic. Isoenzymatic study of leukocytic acid phosphatase showed a noticeable increase of band 3 and the absence of supernumerary band 3b. Immunological analysis revealed a significant decrease of the surface immunoglobulins and a rise in absolute terms in the number of lymphocytes forming spontaneous rosettes. In the case of the prolymphocytic variety what was particularly noticeable was the great number of lymphocytes bearing complement receptors. The combination of cytomorphologic, isoenzymatic and immunological data make it possible to differentiate between lymphoproliferative diseases of T and B-cell origin at the present time.

Acid Phosphatase↗