[Chronic herpes of the pyodermatitis vegetans type in chronic cutaneous lymphoid leukemia]. / Herpès chronique à type de pyodermite végétante au cours d'une leucémie lymphoïde chronique avec localisations cutanées.

The authors report a case of chronic herpes virus infection of the face which developed in a 70-year old man already affected with chronic lymphocytic leukaemia of the B-cell type (CLL-B) with specific cutaneous localisations. Immunodepression was indicated only by marked hypogammaglobulinaemia. Cell-mediated immunity was preserved. The cutaneous lesions of the face were chronic and presented as pyodermatitis vegetans. A one-week course of acyclovir administered by intravenous infusion in doses of 5 mg/kg three times a day resulted in rapid and dramatic cure, but this result proved transient, since the virus infection relapsed 2 1/2 months later. The new episode also was successfully treated with a second course of acyclovir. The herpes virus infection had developed only on those skin areas that were specifically affected by the leukaemia; after treatment and eradication of the virus, massive lymphocytic infiltration of the dermis persisted in these areas. Involvement of the skin is rare in CLL-B and has been reported mainly in CLL-T. The lesions most frequently encountered are tuberous and papular lesions and infiltrated plaques on the forehead and ears. The pyodermatitis vegetans presentation is unusual. The reasons why viral skin lesions develop on those caused by leukaemia are unknown.

[The hemophilic femoropatellar joint]. / L'articulation fémoro-patellaire hémophilique.

The knee is the joint most frequently involved in haemophilia. Femoro-tibial damage in chronic arthropathies has been perfectly described. By contrast, lesions of the patella (with the exception of "squaring") have been reported in only a few scanty publications. An attempt was made to define the characteristics by clinical and radiological study (including in addition to lateral films, an analysis of 30, 60 and 90 degrees axial views, even though these were often made difficult by flexion deformity and joint limitation). Femoro-patellar involvement was seen to be very frequent with various lesions: rarely "squaring", and more often patellar erosions or femoro-patellar incongruence (either pre-existing dysplasia or secondary to haemarthrosis) and sometimes actual incarceration (described as "locked patella" by anglo-saxon authors). These concepts are left to have practical consequences in the management of physiotherapy, synoviorthesis and surgery.

[Our experince with antiglobulin consumption (Dixon test) in the study of antibodies bound to platelets]. / Notre expérience de la consommation de l'antiglobuline (test de Dixon) dans la recherche des anticorps fixés sur les plaquettes.

We measured the quantity of IgG bound to platelets (IgGP) by the antiglobulin consumption test (Dixon technic). In controls, the IgG level did not exceed 10 X 10(-15) g for one platelet. The amount of IgGP was often incraeased (more than 10 X 10(-l5) g) in some autoimmune diseases as lupus erythematosus, chronic lymphocytic leukemia and chronic active hepatitis. Among 26 patients presenting an idiopathic thrombocytopenic purpura (ITP) with a low number of platelets, 21 (77%) had a high titer of IgGP. In 6 ITP in remission, the IgGP titer was normal. After a review of the different technics detecting the IgG bound to platelets, we explain why we choose the antiglobulin consumption. This test is excellent for the research of anti-platelets auto-antibodies in ITP. Yet, the reaction remains negative in a minority of ITP. Several explanations are possible: low quantity of IgGP, presence of other anto-antibodies as IgM or IgA, cellular auto-immunity anti-platelets without antibodies, non immunologic ITP.

[Serum beta-2-microglobulin in multiple myeloma. Practical value]. / Bêta-2-microglobuline sérique au cours du myélome multiple. Intérêt pratique.

The concentration of serum, beta-2-microglobulin (S beta 2m) and the ratio measured S 2m/theoretical S beta 2m M/TH were evaluated in 69 patients with multiple myeloma (MM) and compared with healthy subjects and with patients with a benign monoclonal gammapathy. The concentration of S beta 2m and the ratio S beta 2m M/TH are higher in multiple myeloma than in controls and are strongly correlated with the plasma cell mass On the other hand, no difference was noted concerning the S beta 2m and the ration S beta 2m M/TH between benign monoclonal gammopathy and multiple myeloma at stage I of Durie and Salmon. Under chemotherapy, the variations in concentrations of S beta 2m and of the ratio S beta 2m M/TH are strongly correlated with those of the tumour mass. Beta 2m is not very useful for the differential diagnosis between benign monoclonal gammapathy and multiple myeloma. Beta 2m is very useful in the prognosis of MM, permitting one to assess accurately the tumour mass and the response to treatment, particularly in cases of deficiency of the usual markers.

[Therapeutic failures in early stages of Hodgkin's disease : causes and prevention (author's transl)]. / Les formes précoces de la maladie de Hodgkin : analyse des causes d'échecs, attitudes thérapeutiques.

A retrospective study of 65 patients with Hodgkin's disease (clinical stage I: 11 cases; stage II: 54 cases) included an analysis of the causes of therapy failure. Twenty-five of the 65 patients had been treated by regional irradiation (thoracic mantle or inverted Y field) restricted to only one side of the diaphragm; 27 patients had received the same irradiation followed by chemotherapy (MOPP). The subjects were irradiated on both sides of the diaphragm; 3 of these had received the same radiotherapy followed by chemotherapy (MOPP). Relapses in the irradiated fields were rare. Relapses in the areas bordering the irradiated fields were definitely the result of faulty delivery. Better evaluation of the precise extent of the disease, particularly by laparotomy, would lead to improved initial treatment (radio- or chemotherapy). The availability of improved irradiation techniques and a better choice of indications, particularly concerning the timing for chemotherapy, should result in maximal reduction of therapeutic failures in the early stages of Hodgkin's disease.

[Deaf-mutism, lymphedema of the lower limbs and hematological abnormalities (acute leukemia, cytopenia) with autosomal dominant transmission]. / Surdi-mutite, lymphoedeme des membres inferieurs et anomalies hematologiques (leucose aigue, cytopenies) a transmission autosomique dominante.

Report of a syndrome constituted from sensorineural deaf mutism, lymphoedema of lower limbs with early onset and haematological anomalies (aucte myeloblastic leukaemia, cytopenia) in four individuals (three boys and two girls from two generations). This observation suggest autosomal dominant transmission, however recessive transmission cannot be formelly excluded.

[Multiple myeloma of bones. Retrospective study of prognostic factors based on a series of 243 patients]. / Myélome multiple des os. Etude rétrospective des facteurs pronostiques à partir d'une série de 243 malades.

The authors have made a retrospective study of 243 cases of multiple myelomas, most of them treated with alkylating agents. The average age at the time of diagnosis was 65 years (+ 10). The ratio of the sexes was 1 : 1. Seventy two per cent of the patients had a bone rating of 2 or 3 according to the criteria of Durie and Salmon. Fifty-eight per cent of the patients were anemic (Hg 12 g per cent), with less than 15 per cent being leucopenic or thrombopenic. Hypercalcemia (105 mg 0/00) was noted in 27 per cent of cases. The distribution of immunochemical types is as follows : IgG, 51.5 per cent; IgA, 28 per cent; pure Bence Jones, 20.5 per cent. The respective percentages of kappa and lambda light chains were 64.2 per cent and 35.8 per cent. In this series, nearly 80 per cent of the patients were at phase III according to the classification of Durie and Salmon, and 30.8 per cent at phase B. The median survival, including all phases, increased from 8 months without alkylating agent, to 20 months with melphalan and/or cyclophosphamide. The factors influencing the survival of the patients treated were age, the hemoglobin level, calcemia, renal deficiencies, the immunochemical type, the stage of the disease, and the response to alkylating treatment. The value of these different prognostic factors is discussed.