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A clinical and laboratory evaluation of 28 patients with hairy cells leukemia is performed. Twenty-two had splenomegaly and all but one had a pancytopenia with 5 to 70% of hairy cells in blood. A tartrate-resistant acid phosphatase activity was positive in the hairy cells of 11 patients of 14 studied. In all patients a myelofibrosis and a leukemic infiltration were found in a bone-marrow biopsy of iliac crest. Hemodilution by splenomegaly, mild hemolysis and dyshematopoiesis were observed in 10 patients by a 51Cr or 59Fe isotopic exploration. In seven cases an immunological study of the hairy cells was performed, a high percentage of the leukemic cells of these 7 patients had polyclonal surface Ig but without resynthesis of monoclonal S Ig which is a feature usually associated with B lymphocytes. In the blood of these patients normal T and B lymphocytes were decreased. A splenectomy was done in 12 patients (43%) always for severe pancytopenia Splenectomy was not randomised. Spleen weights ranged from 1 085 to 3 600 g. In splenectomised patients the level of hemoglobin, segmented cells and thrombocytes was significantly higher after surgery. The survival rate is better in the splenectomised group (median survival 57 months) than in the non-splenectomised group (median survival 19 months). Infectious diseases were frequent in all patients but less after splenectomy. Fourteen patients died, 8 owing to pancytopenia.

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Plasma exchanges were performed in 5 patients with acute immunoallergic thrombocytopenic purpura (ITP) because of severe haemorrhages and/or inefficient or badly tolerated corticosteroid therapy. Plasma exchanges were carried out with cell separators and were usually well tolerated. They resulted in very rapid increase in platelet levels with disappearance of auto-antibodies and dramatic reduction of haemorrhages, and they brought about, or accelerated, recovery in 3 patients. One patient was slightly improved. In the 5th patient, who had meningeal haemorrhage, plasma exchange was effective in increasing platelet levels but was unable to prevent a fatal outcome. The main indications for plasma exchange seems to be acute ITP with severe, life-threatening haemorrhages.

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Twenty-five patients with Hodgkin's disease and evidence of bone marrow involvement (BM +) were compared with 25 other Hodgkin patients, chosen at random, who had visceral lesions but free bone marrow (BM -). Both groups were observed throughout the same period. The first evaluation showed that bone marrow involvement was more frequent in male patients and that it produced medullary hypoplasia with WBC and platelet counts lower than in BM - patients. However, bone marrow involvement can only be ascertained by medullar biopsy. BM + patients were treated with multiple chemotherapy (mostly MOPP). Positive response was obtained in 64% and complete remission in 50%, as compared with 70% and 50% respectively in BM - patients. There was no significant difference in overall survival rate between the two groups. It remained in plateau at 50.5% after two years in BM + patients and at 63% after 27 months in BM - patients. When complete remission was obtained the survival rate after one year was 90% for BM + patients and 100% for BM - patients. In the absence of complete remission, however, the median survival time was inferior to one year in both groups. The dosage levels and side-effects of chemotherapeutic agents were similar in both groups, but cytopenia was more pronounced in BM + patients.

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Two cases are reported of an apparently distinct type of immune disorder. Beginning with mild anaemia and widespread massive lymphadenopathy, the disease progressed to a fatal autoimmune type haemolytic anaemia. Serum investigation showed polyclonal hypergamma-globulinaemia and some autoantibodies. Repeat lymph node biopsies in each case showed hyperplasia within B lymphocyte territory (follicular hyperplasia and polyclonal plasmacytosis with IgG predominance) and atrophy of T dependent areas. Dilatation of lymph sinuses, vascular proliferation, and sclerosis were striking features. This appears to be a new entity, and reasons are given for separating this disease from other pseudotumourous lymph node disorders associated with dysimmunity.

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This work is devoted to the analysis of the nature of lymphoblastic lymphoma/leukemia with convoluted nuclei which were initially described by Barcos and Lukes. Ultrastructural, cytochemical, and immunologic features of tumor cells were investigated in patients chosen according to known morphologic criteria. Through results of the E rosette test, the patients were divided into two groups (E+ and E-). In the E+ group, the predominant features were sex (only men), the mediastinal localization, and the focal positivity of the acid phosphatase reaction. Cytotoxicity tests with rabbit antihuman T-lymphocyte anti-serum confirmed the results of the E rosette test in the 3 patients of the E+ group who were tested and were also positive in 2 patients from the E- group (1 of these 2 patients had the characteristics found in the E+ group and can thus be related to this group; the other patient had none of these characteristics). This raises the question of a leukemia arising from a less differentiated T-cell but this interpretation is limited by the specificity of the anti-T-serum. Ultrastructural study defines more precisely the convoluted aspect but does not at present allow a distinction between the two groups.

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A case of Hodgkin's disease is described which developed into a terminal illness characterized by a malignant proliferation of T-cells. The leukemic cells, after optical and ultrastructural analysis, were distinct from those of myelomonocytic, acute lymphoblastic, chronic lymphocytic as well as prolymphocytic leukemia. Their relationship with the T-cell lineage seemed to be confirmed by a highly positive E-rosette test and by cytochemistry which showed focal positivity of acid phosphatase. The importance of this T-cell malignant proliferation is discussed, especially with regard to cellular interactions in Hodgkin's disease.

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The diagnostic and prognostic value of the assay of lysozyme in serum and urine was appreciated in 184 cases of acute leukemia. The levels were decreased in the lymphoblastic, mainly of the non B-non T type, and undifferenciated varieties, markedly raised in the monoblastic and myelo-monocytic varieties, while in the myeloblastic ones they were found normal, decreased or slightly increased, and, on the average, significantly higher in the well differenciated than in the poorly differenciated types. For a given cytological type, the level of lysozyme is not correlated with the frequency of the induction of complete remission. However, in the acute myeloblastic leukemia, a significantly higher frequency of infection during or after the induction treatment was observed in the cases presenting initially without a raised serum lysozyme level.

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The 2 patients have cold agglutinin disease with lymphoid nodules in the bone marrow. A splenectomy was done because of the severity of the hemolytic anemia and because of the increasing splenomegaly. Tumoral nodules with the aspect of lymphoplasmocytic sarcoma or immunocytoma were discovered in the spleen. Those tumors can be considered either as a morphologic aspect of the cold agglutinin disease which is yet considered as a kind of chronic lymphoproliferative syndrom or as the proliferation of an other clone. In this hypothesis, the lymphoplasmocytic sarcoma habe the same signification that the Richter's syndrome in chronic lymphoid leukemia and that the sarcoma appearing in Waldenström's macroglobulinemia or in alpha heavy chain disease.

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The main hematological manifestations and complications seen in 18 cases of Sjögren's syndrome are described: a slight anemia of the inflammatory type, a leucopenia, and/or an eosinophilia occur in about half of the patients. One case of auto-immune hemolytic anemia, one of pernicious anemia, 3 of hypoplastic anemia--one of them drug-induced--, and one of thrombocytopenic purpura have also occurred. The clinical and biological manifestations related to the disturbed immunologic status are also frequent: hyperglobulinemic purpura. Raynaud's syndrome due to cryoglobulinemia, rheumatoid factor, anti-nuclear factor. In 4 patients, the disease presented with or evolved into lymphadenopathy and/or splenomegaly; the initial "pseudo-lymphoma" diagnosis corresponded to different aspects or courses: in one cases, malignancy appeared soon, another has evolved in a chronic lympho-proliferative disease, a third one had the histologic features of angio-immunoblastic lymphadenopathy. These various manifestations, which may reveal the Sjögren's syndrome, are discussed in the light of the litterature data.

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The diagnosis value of various methods used to detect or confirm a Sjögren's syndrome are discussed. The Schirmer's test, generally abnormal, is a good test for detection, although not specific. A subnormal concentration of tears lysozyme is seen in about half of the patients, as so as the presence of anti-salivary ducts antibodies, or significant aspects in lip biopsy. The tests that are most frequently disturbed are the salivary immunoglobulin assay and the fonctionnal study of salivary glands with 99m technetium tracer but they are not specific. These abnormalities are neither constant, not specific: for this reason, there are frequently some problems in the diagnosis or the nosologic definition of Sjögren's syndrome. The occurrence in the same subject of many of these abnormalities makes however this diagnosis highly probable even in patients without functionnal troubles.

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An association of pernicious anaemia. Hashimoto's thyroiditis, Sjögren's syndrome and chondrocalcinosis in a 68 years old women is described. Such an observation, does not seem to have been reported previously; the frequency of the different associations and the specificity of the antibodies observed in each cases are discussed.

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The "dU suppression" test, studying thymine-DNA synthesis from deoxyuridine is abnormal in folate and vitamin B12 deficiency, these two vitamins being involved in this reaction. We have done it in 7 cases of anaemia by folate deficiency, 12 cases of anaemia by vitamin B12 deficiency, in 3 cases of combined deficiency, in 3 cases of acute myeloïd leukaemia, 2 cases of sideroblastic anaemia and 2 cases of intoxication by antifolate. This test is very sensitive: it is abnormal even when there is an anaemia related to a vitamin deficiency with only very light morphologic abnormalities and can discriminate the cases, mainly in myelodysplastic syndromes, where folate deficiency is only an epiphenomen in the genesis of anaemia. The abnormalities of the "dU suppression" are quite corrected by added folate derivatives in folate deficiency. However, cobalamin derivatives did not correct completely the test in vitamin B12 deficiency except when a folate compound is also added. The "dU suppression" test becomes normal about one week after starting vitaminotherapy in vitamin B12 deficiency.

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Human lymphocytes from peripheral blood, bone marrow spleen and lymph nodes were cultured. Continuous phytoheamagglutinin (PHA) stimulation was used, first during a 24 h liquid preincubation, then during a 5 day culture in methylcellulose. In normal donors a rapid colony formation took place, with a mean of 124+/-82 colonies per 1 times 10(5) preincubated lymphocytes. Cells from such colonies were studied by cytology, scanning electron microscopy and rosette formation techniques; arguments favour the hypothesis that these could be T lymphocytes. Neither granulocytes nor macrophages could be grown, and no lymphoid colony formation occurred without PHA stimulation. The same technique was applied to patients with various lymphoproliferative disorders. Significant colony suppression was observed in nearly every case of chronic lymphatic leukaemia; the number of colonies was reduced in some patients with acute lymphatic leukaemia, lymphosarcoma, dysglobulinaemia and Hodgkin's disease. This lymphoid culture method should be applied to a larger number of patients to determine whether it has a classification value and/or prognostic significance. When colonies were grown in pathological states, rosette formation was identical to that of normal donors; colony formation could be due to persisting normal lymphocytes.

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Criteria reputed to be of grave prognostic significance were studied in 94 patients suffering from Hodgkin's disease at stages I and II, after laparotomy and splenectomy. The parameters studied (age, general signs, histological type, mediastinal involvement) are less prognostic factors than indications of unrecognised extension of the disease: almost 1/3 of unrecognised lesions in the presence of one of the criteria; almost 2/3 with two or more. Exploratory laparotomy revealed lesions unsuspected on the basis of clinical evaluation only in 10% of patients with no criteria of poor prognosis. The long term prognosis of the disease depends, in fact, not upon the existence of these criteria but upon the method used in initial treatment. Five year survival and cure rates are significantly better in patients treated with combined polychemotherapy (MOPP) and radiotherapy than in those treated with radiotherapy alone. These results would indicate that routine laparotomy should be abandoned in patients with a localised clinical staging if it is decided to begin treatment with a combination of radiotherapy and chemotherapy. So-called prognostic factors could then be used simply to vary the intensity of the treatment prescribed.

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A G-6PD deficiency was detected in a Negro patient from Senegal suffering from congenital nonspherocytic hemolytic anemia. The main characteristics of this variant were: profound defect of G-6PD activity in the red cells, decreased immunologic specific activity, fast electrophoretic mobility, decreased Km-G-6P and normal Km-NADP+, normal inhibition by ATP and NADPH, slightly increased utilization of the substrate analogues, slightly biphasic pH curve, high heat lability, subnormal activation energy. The characteristics of this variant being unique, it was called 'G-6PD Hôtel Dieu.'

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Our aim in this work has been to obtain precisions on the bleeding mechanism during fibrinolysis induced by streptokinase in the rabbit. Haemostasis was studied in vivo by the method oh Honour and Russell. The injection of 40 000 u/kg of streptokinase in 10 minutes induces rapidly fibrinolysis, which lasts for approximately one hour which is not accompanied by changes either in coagulation factors, or in platelet function. In vivo : . the adhesion of platelets to the vascular lesion is normal; . platelet aggregation is normal as is the formation of intravascular thrombi; . on the other hand the intra mural platelet thrombus is unstable. The embolization of the intravascular thrombi is responsible for new hemorrhages which can be correlated with the intensity of fibrinolysis. Thus, even in the absence of fibrinogenolysis, of fibrinogen degradation products FPD, of abnormal thrombin generation and of altered platelet function, the fibrinolytic activity induced by streptokinase could alone be responsible for bleeding episodes, occuring during thrombolytic treatment by streptokinase in man.

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