The aim of this study was to evaluate the long-term response to rituximab in patients with chronic and refractory immune thrombocytopenic purpura (ITP). Adults with ITP fail to respond to conventional therapies in almost 30% of cases, developing a refractory disease. Rituximab has been successfully used in these patients. We used rituximab at 375 mg/m 2 , IV, weekly for a total of four doses in 18 adult patients. Complete remission (CR) was considered if the platelet count was >100 × 10 9 /l, partial remission (PR) if platelets were >50 × 10 9 /l, minimal response (MR) if the platelet count was >30 × 10 9 /l and <50 × 10 9 /l, and no response if platelet count remained unchanged. Response was classified as sustained (SR) when it was stable for a minimum of 6 months. Median age was 43.5 years (range, 17 to 70). Median platelet count at baseline was 12.5 × 10 9 /l (range, 3.0 to 26.3). CR was achieved in five patients (28%), PR in five (28%), MR in four (22%), and two patients were classified as therapeutic failures (11%). Two additional patients were lost to follow-up. The median time between rituximab therapy and response was 14 weeks (range, 4 to 32). SR was achieved in 12 patients (67%). There were no severe adverse events during rituximab therapy. During follow-up (median, 26 months; range, 12 to 59), no other immunosuppressive drugs were used. In conclusion, rituximab therapy is effective and safe in adult patients with chronic and refractory ITP. Overall response rate achieved is high, long term, and with no risk of adverse events.
Gershon and Kondo described CD8+ Treg lymphocytes as the first ones with regulating activity due to their tolerance ability to foreign antigens and their capacity to inhibit the proliferation of other lymphocytes. Regardless, CD8+ Treg lymphocytes have not been fully described—unlike CD4+ Treg lymphocytes—because of their low numbers in blood and the lack of specific and accurate population markers. Still, these lymphocytes have been studied for the past 30 years, even after finding difficulties during investigations. As a result, studies have identified markers that define their subpopulations. This review is focused on the expression of cell membrane markers as CD25, CD122, CD103, CTLA-4, CD39, CD73, LAG-3, and FasL as well as soluble molecules such as FoxP3, IFN-γ, IL-10, TGF-β, IL-34, and IL-35, in addition to the lack of expression of cell activation markers such as CD28, CD127 CD45RC, and CD49d. This work also underlines the importance of identifying some of these markers in infections with several pathogens, autoimmunity, cancer, and graft-versus-host disease as a strategy in their prevention, monitoring, and cure.
Several prognostic factors have been recognized in patients with multiple myeloma (MM). Among the most important are: the serum levels of beta2-microglobulin, albumin, and LDH; the labeling index; and an abnormal karyotype. Patients with amyloidosis (AL) have poor prognosis; however, little is known concerning the prognostic significance of AL associated to MM. In 201 consecutive patients with de novo MM, we performed a fat-pad biopsy needle aspiration (FPBNA) that was stained with Congo red. Sixty eight (34%) patients had AL and a poorer prognosis disease: lower performance status, presence of B symptoms, higher LDH and calcium values, and worse response to chemotherapy. Cox regression model for overall survival detected three variables having independent prognostic significance: the presence of AL (RR = 3.4, P < 0.004), serum albumin levels <3.5 g/dl (RR 3.2, p < 0.005), and patients not achieving complete remission or very good partial remission (RR 2.9, p < 0.02). In 28% of patients with de novo MM, FPBNA was useful to detect incidental amyloidosis. During follow-up, 69% of these patients had symptoms of AL. Excluding 16 patients with obvious symptoms of AL at diagnosis, overall survival was worse in patients who developed later symptoms of AL. MM-associated AL represents a poorer prognosis disease even in the absence of symptoms at diagnosis, and this specific association may be considered as an independent high-risk prognostic factor. The routine study of periumbilical fat-pad tissue should be mandatory in all patients with MM.
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