CD23 antigen density is related to serum gamma globulin level, bone marrow reticulin pattern, and treatment in B chronic lymphocytic leukemia.

The CD23 antigen density was evaluated by a cytofluorometric technique in 55 patients with chronic lymphocytic leukemia. The quantification method was based on the use of biological standards in indirect immunofluorescence. The CD23 antigen density was correlated with the percentage of CD23 positive cells, but antigen density appeared to be a more informative parameter. CD23 antigen density was lower in stage B than in stages A or C patients, and higher in patients undergoing chemotherapy or previously treated than in untreated patients. There was a significant negative correlation between CD23 antigen density and serum gamma globulin and IgG levels, that existed only in patients in an advanced stage of the disease. CD23 antigen density was higher in patients with abnormal bone marrow reticulin pattern. Serum gamma globulin level was lower in these patients, as well as in patients with prognostically unfavorable histologic bone marrow infiltration pattern. These data emphasize the interest of antigen density as an additional parameter and the complex relationship between CD23 expression, hypogammaglobulinemia, bone marrow histologic findings, and treatment in chronic lymphocytic leukemia.

Non-Hodgkin's lymphoma associated with primary Sjögren's syndrome.

OBJECTIVES: To evaluate the prevalence, the incidence and clinical presentation of non-Hodgkin's lymphoma associated with primary Sjögren's syndrome. METHODS: Sixty-two patients with primary Sjögren's syndrome were analyzed retrospectively in an open investigation. RESULTS: Of 62 patients with primary Sjögren's syndrome, 4 of them (6.4%) developed non-Hodgkin's lymphoma (6.9 cases per 1000 per year). All of them were women. Non-Hodgkin's lymphoma always developed after the onset of primary Sjögren's syndrome with a time interval ranging from 3 to 27 years. Pathological findings showed two diffused mixed small and large cell cleaved lymphomas and two diffused large cell cleaved lymphomas. Three cases had extra-nodular localizations. All of these 4 patients are still alive and in complete remission 2 to 8 years after the diagnosis of non-Hodgkin's lymphoma. CONCLUSION: This study confirms the association of non-Hodgkin's lymphoma in primary Sjögren's syndrome. These non-Hodgkin's lymphomas frequently had extra-nodal localizations. Good sensitivity to treatment, when necessary, provided good prognosis.

A study of 15 cases of primary mediastinal lymphoma of B-cell type.

Fifteen cases of pure supradiapragmatic lymphoma with initial prominent antero-superior mediastinal involvement displaying a B-cell pattern of reactivity were studied. These cases occurred in six men and nine women with a median age of 33 years at diagnosis (range, 23 to 75 years). Supradiapragmatic peripheral lymphadenopathies were present in three cases, and intrathoracic extension to the lung, pericardium, or pleura was possible. In five cases a thymic origin was obvious. All cases exhibited a B-cell pattern of differentiation, with a great variety of histopathologic aspects associated with a high frequency of fibrosis and/or necrosis. Hodgkin's disease was initially misdiagnosed in four cases. The evolution was purely local, with extrathoracic extension in five cases, at the ultimate phase of the disease. The prognosis appeared to be poor with only five patients still alive at a median survival time of 16 months. A complete chemoresistance and radio-resistance was observed in seven cases; only two complete remissions were achieved with aggressive chemotherapy. Prolonged remission could be achieved after surgical reduction of the mass. Primary B-cell mediastinal lymphoma appears to be a distinct clinical entity with local evolution and resistance to therapy. A new therapeutic regimen, which could include surgery in some cases, should be found for this disease.

Acute monocytic leukemia with B cell markers expression following B chronic lymphocytic leukemia.

A case of chronic lymphocytic leukemia (CLL) treated with chlorambucil, followed by the development of an acute monoblastic leukemia, is described. Cytofluorometric quantitative immunophenotype was determined during the blastic phase. Whereas small lymphocytes displayed a CD19+; CD24+; CD37+; CD5+ phenotype, the blastic population exhibited, besides CD13, CD14 and CD15 positivity, which is usually noted in such a monoblastic leukemia, definite CD9, CD10, CD22, CD24, CD37, CD5 and CD4 staining. Such results argue against a complete independence between the two clones, although their similarity could not be demonstrated.

Specific 5 alpha-dihydrotestosterone receptor and 5 alpha-reductase activity in human amniotic fluid cells.

A macromolecular component which specifically binds tritium-labeled-dihydrotestosterone is present in cultured amniotic fluid cells. The androgen-binding complex is characterized by a 3.6S sedimentation coefficient, an apparent dissociation constant of 1 nmol/L, a mean binding capacity of 243 +/- 140 fmol/mg of DNA, and a specificity for testosterone and dihydrotestosterone. Similar properties have been reported for the androgen receptor of the fetal genital skin fibroblast, which suggests that the tritium-labeled-dihydrotestosterone-binding component in amniotic fluid cells is the androgen receptor. Amniotic fluid cell monolayers incubated with serum-free medium containing testosterone are able to transform testosterone into dihydrotestosterone. The 5 alpha-reduced product has been characterized by thin-layer chromatography and capillary column gas-liquid chromatography. Androgen receptors and 5 alpha-reductase activity are expressed in amniotic fluid cells. The prenatal diagnosis of 5 alpha-reductase deficiency or complete androgen insensitivity syndrome (testicular feminization) is thus theoretically possible and obviously prenatal testing would be indicated in the family at high risk.

[Study of caryotypes in 52 cases of chronic lymphocytic B-cell leukemia]. / Etude du caryotype de 52 leucémies lymphoïdes chroniques B.

The karyotypes of 103 cases of B cell chronic lymphocytic leukemia were studied following stimulation or establishment of continuous cell lines using the Epstein-Barr virus. In 52 patients metaphases suitable for cytogenetic analysis were obtained; 30 revealed normal karyotypes and 22 abnormal karyotypes. The most frequently encountered abnormalities were 6 cases of extra chromosome 12, 4 cases of structured aberrations concerning chromosome 14, including 2 t(11;14), 4 translocations concerning chromosome 11, and 3 cases of extra chromosome 3. No relationship appears to exist between the abnormalities observed and the clinical profile or prognosis.

[Idiopathic pulmonary hemosiderosis and celiac disease in a child. Case report]. / Hémosidérose pulmonaire idiopathique et maladie coeliaque chez l'enfant. Une observation.

An enteropathy with almost total atrophy of the villi was discovered in a 13-year old girl with idiopathic pulmonary haemosiderosis. Coeliac disease was strongly suspected but could not be proven. This case draws attention to the possible association of an idiopathic pulmonary haemosiderosis with a coeliac disease.

Isochromosome 17q in cell lines of two cases of B cell chronic lymphocytic leukemia.

The cytogenetic study of lymphocytes stimulated by EB virus in two cases of B-CLL revealed an isochromosome 17q. This abnormality, well known in CML during the stage of blastic transformation, may not be specific to myeloid proliferation as it is also observed in malignant lymphomas and B- or T-CLL. The anomaly does not appear to have the same prognostic nature as in CML.

Karyotype analysis of B-lymphocytes transformed by Epstein-Barr virus in 21 patients with B cell chronic lymphocytic leukemia.

The karyotypes of 21 patients with chronic B cell leukemia were studied using lymphoblastoid cell lines obtained with the aid of the Epstein-Barr virus. Ten patients had a normal karyotype and eleven patients, an abnormal. There is no single characteristic anomaly, but certain types were more frequent, i.e., 14q+ as a result of a translocation 11;14, trisomy 12, and an isochromosome 17q. Abnormalities in these same three chromosomes have been found in other hematologic malignancies. It is postulated that loci with control cell proliferation are present on chromosomes 12, 14, and 17.

11;14 translocations in lymphoproliferative disorders. An analysis of three cases.

Our paper reports 3 cases of (11;14) (q13;q32) translocation during two B-cell chronic lymphocytic leukemias and one B-cell diffuse centrocytic malignant lymphoma. Relating to this subject, we briefly review observations of 11;14 translocations during lymphoid hemopathies.

[Hepatitis caused by Campylobacter coli (author's transl)]. / Hépatite à Campylobacter coli.

Campylobacter coli is known to cause ulcerous enterocolitis, but hepatitis has not yet been reported. A 50-year-old woman without history of liver disease was admitted with diarrhoea, fever, poor general condition, subicterus and enlarged liver. Campylobacter coli was grown in haemoculture, and a specific antibiotic treatment resulted in complete cure. The results of haemoculture, the necrosis and polymorphonuclear infiltrates found in liver biopsies, the return to normal of biochemical tests and liver size under antibiotic therapy and the absence of any other cause of acute or chronic liver disease are strong arguments in favour of the hepatitis being caused by Campylobacter coli in this patient.

[Mastocytosis and leukemia (author's transl)]. / Mastocytoses et leucémies.

The authors report a case of Ph 1 positive chronic myelocytic leukemia associated with cutaneous mastocytosis, occuring lately in a 75 years old man. 12 similar cases (associations mastocytosis chronic or acute leukemia) are described in the literature.

[Partial trisomy 21 with 45 chromosomes due to translocation of two chromosomes 21 onto a chromosome 14 : 45,XX-14,-21,+t(14q21q21q) (author's transl)]. / Trisomie 21 partielle à 45 chromosomes par translocation de deux 21 sur le 14 : 45,XX,-14,-21,+t(14q21q21q).

A patient with the phenotype of trisomy 21 and increased activity of superoxide dismutase A is reported with partial trisomy for the distal portion of 21q. The exceptional feature in this case is a 45-chromosome karyotype due to the translocation of two chromosomes 21 onto the distal end of 14q.

[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.

[B-immunoblast lymphosarcoma 8 years after a malignant reticulosis]. / Lymphosarcome à immunoblastes b huit ans après une réticulose maligne.

A girl presented with a malignant reticulosis in the early months of life. For 4 years she was treated with repeated courses of irradiation and chemotherapy, mostly cyclophosphamide. After 6 years of complete remission she developed a rapidly progressive B cell lymphoma. The contribution of the various forms of treatment to the development of the second malignancy are discussed.

[Angio-immunoblastic lymphadenopathy]. / La lympho-adénopathie angioimmunoblastique.

A report is presented on four cases of angioimmunoblastic lymphadenopathy. In this recently individualized entity, adenomegaly with fever and cutaneous eruption is associated with polyclonal hypergammaglobulinemia and autoimmunization, especially of anti-erythrocytic type. The lymph nodes are homogenized by a lymphoplasmo-immunoblastic granuloma with angiogenesis. The disease could be attributed to hyperplasia of B lymphocytes with a deficit of T cells, which would explain the autoimmunization. It appears in some cases to be triggered by accidents of drug intolerance. Prognosis is poor and the course of the disease is nearly always fatal in the long or short term. It is difficult to ascertain whether prolonged remissions, either spontaneous or therapeutic, can be considered actual cures.