Expression of platelet glycoproteins by erythroid blasts in four cases of trisomy 21.

In four patients with trisomy 21 (three constitutional, one acquired) with a morphological undifferentiated leukemia, diagnosis of erythroid leukemia was established by both immunophenotyping and ultrastructural studies. Indeed, a majority of blasts from three patients expressed several erythroid markers such as carbonic anhydrase 1, spectrin beta chain, and glycophorin A. In addition, band 3 and hemoglobin were immunologically detected in a fraction of the blast cells from two cases. At ultrastructural level, a majority or all blast cells exhibited erythroid differentiation features such as theta granules and ferritin molecules. However, platelet glycoproteins GP Ib, GP IIb, and GP IIIa were also immunologically detected in a fraction (from 14-82%) of the blasts. Since the ultrastructural study indicated that some promegakaryoblasts were also present in three patients, double labeling between erythroid markers (glycophorin A or carbonic anhydrase I) and platelet glycoprotein (Ib or IIIa) was performed and showed a clear overlap between the two kinds of markers. A similar approach was performed at ultrastructural level and indicated that blast cells with ultrastructural erythroid features of differentiation may have three distinct phenotypes, i.e., presence of glycophorin A without platelet glycoproteins or, conversely, the presence of platelet glycoproteins without glycophorin A and coexpression of glycophorin A and platelet glycoproteins. Expression of glycophorin A correlated directly with the differentiation level of the erythroid blasts, whereas platelet glycoproteins were essentially expressed in the more primitive leukemic erythroid cells. The GP Ib synthesized by these blasts was subsequently studied. The GP Ib alpha mRNA analyzed by Northern blot from these erythroid cells was identical in size with that from megakaryocytic cells as was the molecular weight of the GP Ib molecule from both after immunoprecipitation by a monoclonal antibody. Therefore, "in vivo" erythroid leukemic cells may express the main platelet glycoproteins including GP Ib.

Pleomorphic medium-sized T-cell lymphoma following Hodgkin's disease (nodular sclerosis type).

We describe a 32-year-old woman who presented with Hodgkin's disease, nodular sclerosis type II, subtype I, which necessitated several treatments over 11 years. The patient then developed pleomorphic, medium-sized T-cell lymphoma, which had a fatal outcome within 13 months. The role of radiotherapy, splenectomy, and chemotherapy in second tumor induction is compared with other sequential T-cell lymphomas. The significance of rare Epstein-Barr virus-infected cells during the T-cell lymphoma extension is discussed.

[Relapsing polychondritis, thrombosis and antiphospholipid antibodies]. / Polychondrite atrophiante, thromboses et anticorps antiphospholipides.

The authors report a case of relapsing polychondritis associated with antiphospholipid antibodies. The initial manifestations were recurrent venous thrombosis which occurred prior to the chondritis. Antiphospholipid antibodies are found in various diseases, some of which are sometimes associated to relapsing polychondritis. However, antiphospholipid-antibodies have not yet been reported in this disease.

["Transparent circles" lymph glands: an unusual appearance on lymphography in certain proliferative lymphoid syndromes: prognostic value]. / Les ganglions "cerclés transparents". Aspect inhabituel de la lymphographie dans certains syndromes lympho-prolifératifs. Intêrêt pronostique.

The authors describe 27 cases in which a very particular type of lymphographic image was observed, associating disseminated lesions in all the retroperitoneal lymph glands, and an appearance of "circled" glands of very weak density. This image may be rarely observed in Hodgkin's disease and non-Hodgkin type hematosarcomas. It is frequently observed, however, during the course of angio-immunoblastic lymphadenopathy. Whatever the etiology, this image is of particularly poor prognostic significance.

[Hodgkin's disease: analysis of a reduction of chemotherapy]. / Maladie de Hodgkin: analyse d'une réduction de la chimiothérapie.

From April 1972 to September 1979, 121 patients with Hodgkin's disease clinical stages IInA, IB, IIB or III successively received MOPP 6 courses in a first trial and 3 courses in a second trial, prior to extended field irradiation; 118 patients underwent surgical restaging prior to irradiation. Anatomical findings demonstrated that 3 courses of MOPP were as effective as 6 courses to treat occult splenic disease. Comparison between clinical and surgical restaging confirmed the reliability of clinical criteria of complete remission after chemotherapy. After extended field irradiation, actuarial survival and relapse-free survival at 4 years were the same whether the patients had received 6 or 3 courses of MOPP.