Lupus mastitis in systemic lupus erythematosus: a rare condition requiring a minimally invasive diagnostic approach.

Breast involvement is a rare event in SLE patients. The most frequent presentation is lupus panniculitis with skin erythema, tenderness, and parenchymal nodules. However, when breast masses are detected in SLE patients without significant superficial inflammation, it is mandatory to rule out breast carcinoma. Here, we report the case of a 47-year-old woman with an 18-year-long history of SLE, who presented with a suspicious breast mass. Since surgical trauma has been reported to be able to exacerbate breast inflammation in lupus mastitis, an ultrasound-guided minimally invasive Mammotome biopsy was performed to obtain tissue samples for histological and immunohistochemical examinations. Histology was consistent with lupus mastitis. The patient was already on mycophenolate mofetil and hydroxychloroquine. At the latest follow-up visit 6 years later, no progression of the breast lesion was observed.

Direct or reverse correlations within the expression of activation, differentiation or T-B cooperation molecules on chronic lymphocytic leukemia B cells.

AIM: B-cell chronic lymphocytic leukemia (B-CLL) is characterized by homogeneous coexpression of CD19, CD23 and CD5, and poor expression of membrane Ig. The aim of this study was to evaluate, in B-CLL patients and in healthy subjects by flow cytometry, B cell expression of surface molecules involved in cell activation, differentiation, T-B cooperation and apoptosis. METHODS: The study population consisted of 29 patients (16 men and 13 women; mean age: 66.5 years) with B-CCL. The control group consisted of 16 sex- and age-matched healthy subjects. The results are reported as percentages and mean fluorescence intensity (MFI) of CD19+ cells coexpressing each analyzed molecule. RESULTS: We found that the lymphocyte activation markers, CD69, CD25 and CD11c, were more expressed in B-CLL patients than controls. CD38 and CD95 expressions were higher on normal B lymphocytes than leukemic B cells. Finally, CD80 and CD86, molecules involved in T-B cooperation, showed an inverse expression between lymphocytes of B-CLL patients and healthy subjects. CD80 was higher on normal than leukemic B cells, while CD86 expression was higher on CLL B cells. Linear regression analysis showed a positive correlation between CD80 and CD95 expression on leukemic B cells; a reverse correlation was observed between CD69 and CD11c. CONCLUSION: These results suggest that common mechanisms may regulate the simultaneous expression of CD80 and CD95 or the reverse expression of CD69 and CD11c, respectively, in different stages of B cell activation and/or differentiation.

Herpes zoster infection and Ogilvie's syndrome in non-Hodgkin's lymphoma with hypogammaglobulinemia.

The case of a 43-year-old male with non-Hodgkin's lymphoma (stage IV B), and hypo-IgG and IgM, who developed acute colonic pseudo-obstruction or Ogilvie's syndrome during chemotherapy, is presented. The simultaneous occurrence of a unilateral segmental vesicular rash indicative of herpes zoster infection suggests an etiopathogenetic relationship between the colonic pseudo-obstruction and herpetic involvement of the motor celiac sympathetic ganglia. The rapid resolution of the abdominal dilation and the functional recovery from the colonic pseudo-obstruction after anti-viral therapy is also consistent with the diagnostic hypothesis.

[Late onset immunodeficiency with hypo-IgG and hyper-IgM, T CD4+ lymphocytopenia and vitiligo]. / Immunodeficienza ad insorgenza tardiva con ipo-IgG e iper-IgM, linfocitopenia T CD4+ e vitiligo.

The Authors report the clinical case of a patient with a deficit of humoral immunity who developed infections since puberty. The serum levels of IgG and IgA decreased progressively in the fourth decade of life, while serum IgM increased. Moreover, the patient developed a marked CD4+ T lymphocytopenia and a meager B lymphocytopenia, vitiligo, positivity for anti-SSA/Ro autoantibodies and granulomatous phlogosis of the knee. The heterogeneity of the clinical and laboratory data suggests that this patient might present an overlap immunodeficiency syndrome with some of the clinical and immunological features typical of the hyper-IgM syndrome (in the X-linked or autosomal forms) and others that can be referred to a nosologically distinct humoral immunodeficiency such as the common variable immunodeficiency.

Lymphocyte subset reconstitution after HLA-identical placental blood transplantation (PBT) or PBT plus bone marrow transplantation (BMT) in three children with beta-thalassemia major.

The kinetics of circulating lymphoid cells were evaluated in three children suffering from beta-thalassemia major after HLA-identical sibling placental blood transplant (PBT) in one patient and placental blood plus bone marrow transplantation (BMT) in two patients. Recovery of the main lymphocyte subsets, as determined by phenotype analysis of circulating PBMCs, was complete within 2 months after transplant. NK (CD56+) cells were the first to appear in peripheral blood, followed by T (CD3+, CD2+, CD7+) and B (CD19+) cells. Of the T lymphocytes, the CD8+ were the first to reconstitute, but recovery of CD4+ cells was also rapid and within 6 months these T cells reached normal values. The expression of CD57 by NK or T cells was slightly delayed. The evaluation of RA and RO isoform expression of the CD45 molecule showed a prevalence of the CD45RA antigen with a ratio of 2-3:1. In the PBT only patient, T cells expressing the CD45RO antigen prevailed in the early post-transplant period. Severe or chronic GVHD was not observed. This experience demonstrates that reconstitution of lymphocyte subsets is successful in genetic hematological diseases after transplantation of HLA-identical placental blood or placental blood plus bone marrow from healthy or heterozygous siblings. Bone Marrow Transplantation (2000) 26, 743-747.

A 'de novo' arisen case of angioedema C1-inhibitor deficiency dependent: possible mutagenic effect of azathioprine?

It is reported that a C1-inhibitor (CI-INH) deficiency dependent angiodema case arose 'de novo' in a child without a family history of this disease. His mother was undergoing immunosuppressive therapy (50 mg of azathioprine plus 8 mg of methyl-prednisolone daily) during pregnancy, uninterrupted for seven years because of a kidney transplant. All the other known causes of acquired C1-INH deficiency were excluded. An involvement of an azathioprine-induced C1-INH gene mutation is hypothised.

Functional characteristics of cord blood T lymphocytes after lectin and anti-CD3 stimulation. Differences in the way T cells express activation molecules and proliferate.

Newborns are more susceptible than adults to infections, which suggests a relative immaturity of the immune system early after birth. Cord blood T cells differ significantly both in surface phenotype and function from adult T cells. We examined the proliferation and expression of activation molecules by lymphocytes isolated from umbilical cord blood or peripheral blood of adults. The lymphocytes were cultured for 5 days in the presence of phytohemagglutinin, concanavalin A, or anti-CD3 monoclonal antibody. Cord blood T cells expressed the CD45RA molecule, while a low proportion expressed the RO isoform, a marker of primed or activated lymphocytes. Furthermore, more than 95% of neonatal lymphocytes bear the CD38 molecule, but do not express the CD57 molecule. After stimulation by phytohemagglutinin or concanavalin A, the lymphocytes from newborns were activated and proliferated as efficiently as adult T cells. Anti-CD3 did not cause neonatal lymphocytes to proliferate, but these cells expressed activation molecules, such as HLA-DR antigens and the receptor for interleukin-2 and transferrin. The relevance of these findings to tolerance induction in immature cord blood T cells is discussed.

Ascitic fluid-associated lymphocytes in patients with metastatic ovarian cancer. Analysis of lymphocyte subsets and of phenotypical changes induced by "in vitro".

Ascitic fluid and peripheral blood samples, from seven patients affected by peritoneal carcinomatosis secondary to ovarian tumors, were analyzed to examine the phenotypic properties of ascitic fluid-associated lymphocytes (AFAL) and then asses T-cell activities in the presence of different interleukins. The AFALs showed a relative enrichment of CD2+, CD3+, CD5+, CD4+ T cells compared to the peripheral blood samples, while the contrary was true for CD56+ and CD57+ cells. The same phenomenon was observed in both CD3+, CD56+ and CD2+, CD56+ cell populations. Fewer CD71+ and CD25+ cells were found in the ascitic fluid suspensions whilst the opposite was true for HLA-DR+ lymphocytes. The data obtained from the in vitro incubation of AFALs in the presence of IL-1 (or), IL-2, IL-4, IL-6 or IFN showed that IL-1, IL-2 and IFN- can induce a two-fold increase in the percentages of HLA-DR+ T lymphocytes. IL-6, IL-2 and IL-1 seemed to have a fairly modest effect on CD25. A better survival of CD56+ cells was found only in the presence of IL-4 and IL-6. Even though preliminary and based on a limited number of patients, our results would seem to invite caution in instituting intraperitoneal administration of one or more cytokines for the treatment of such lesions in humans.

Monoclonal expansion of immunoglobulin not-secreting CD5+ CD11c+ CD38+ B-cells in a rare case of chronic lymphoplasmacytoid leukaemia.

We present the clinical and immunological features of a rare case of chronic lymphoid leukaemia with lymphoplasmacytoid morphology. The patient was first admitted suffering from weakness, pallor, dyspnoea, marked splenomegaly, hepatomegaly and systemic lymphadenopathy and panhypogammaglobulinaemia. White blood cell count revealed important leukocytosis (220 x 10(9) WBC/l) with 2% neutrophils and 98% lymphoid cells showing lymphoplasmacytoid features, while lymphoid cells of identical morphology severely infiltrated the bone marrow and lymph nodes. The disease, initially controlled by non aggressive chemotherapy over a period of 30 months, later evolved to a clinical and haematological picture suggestive of Richter's syndrome. Immunophenotyping of the leukaemic cells demonstrated a monoclonal expansion of B-cells bearing surface markers of typical CLL (CD5, CD19, CD20, CD21, CD22, CD23, CD24, CD40 and low density IgM+IgD/kappa) and also the CD11c and CD38 antigens. A proportion of these cells expressed activation markers (CD25, CD69 and CD71). Following in vitro activation with TPA or PWM, the cells responded by weak incorporation of 3H-TdR but failed to secrete immunoglobulins. These findings confirm the broad morphological, phenotypical and clinical spectrum of chronic lymphoid leukaemias.