[Congenital dyserythropoietic anaemia, type I: a new case (author's transl)].
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Biomedical subjects
Publications and source records attributed to S Woessner.
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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.
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.
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.
Isoenzymatic study of leucocytic acid phosphatase under normal conditions identifies 3 isoenzymatic bands, which exhibit a noticeable cell specificity. Band 2 is granulocytic, band 3 lymphocytic and band 4 monocytic in origin. Pathologic deviations in the isoenzymatic pattern are both qualitative and quantitative. For some diseases such as chronic lymphocytic leukaemia there is a well-defined, differential pattern according to immunological B- or T-cell origin. The more significant qualitative aspects are related to the appearance of abnormal bands, especially band 3b, indicating blastic cellularity, and 5, corresponding to hairy cells. The isoenzymatic analysis of acid phosphatase activity is a simple haematologic complementary test, particularly useful in the differential diagnosis of lymphoproliferative disorders with peripheral blood manifestations.
The normal isoenzymatic pattern of leucocytic acid-phosphatase based on the study of 150 haematologically normal individuals is reported. The different pathologic patterns of the leucocytic acid-phosphatase isoenzymes occurring in non-lymphoblastic acute leukaemias are presented and correlated with the subdivisions of acute leukaemias established by the French-American-British (FAB) Co-operative Group. This study is considered to be especially useful in identifying pure acute monocytic leukaemias corresponding to subtype M5 of the FAB as well as acute erythraemias with unusual cytological and cytochemical features.
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A case of prolymphocytic leukaemia with immunological characteristics of T-cell type is reported. Three noteworthy findings can be emphasized: the presence of C3 receptors on the T-prolymphocytes, the study of the acid-phosphatase isoenzymatic pattern, which showed an increased band 3 with absence of band 3b, and the morphometric ultrastructural investigation. Cytochemistry and ultrastructural morphometry may be useful for a more precise characterization of prolymphocytic leukaemia and help to distinguish it from other lymphoproliferative disorders.
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A case of systemic mastocytosis with unusual clinical manifestations, appearing as an isolated splenohepatomegaly, is described. The proliferative character is evident from the cytological characteristics of immaturity and the presence of a moderate percentage of mast cells in the peripheral blood. These cells make up 40% of the total cells in the bone marrow. Special attention is given to the optical, morphological, cytochemical, and ultrastructural studies of the disease. Some anomalies were found at the subcellular level which apparetnly have not been recorded until present. Various dyshematopoietic features of this case are reported, which may be considered as manifestations of a paraneoplastic syndrome.
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