SummaryDetection of circulating plasma cells (PCs) in multiple myeloma (MM) patients is a well-known prognostic factor. We evaluated circulating PCs by flow cytometry (FC) in 104 patients with active MM at diagnosis by gating on CD38 + CD45 -cells and examined their relationship with cytogenetic risk.Patients had an average follow-up of 36 months. By using a receiver operating characteristics analysis, we estimated the optimal cut-off of circulating PCs for defining poor prognosis to be 41. Patients with high-risk cytogenetics (n = 24) had poor prognosis, independently of circulating PC levels [PC < 41 vs. PC ≥ 41: overall survival (OS) = 0% vs. OS = 17%, P = not significant (n.s.); progression-free survival (PFS) = 0% vs. 17%, P = n.s.].Patients with standard-risk cytogenetics (n = 65) showed a better prognosis when associated with a lower number of circulating PCs (PC < 41 vs. PC ≥ 41: OS = 62% vs. 24%, P = 0Á008; PFS = 48% vs. 21%, P = 0Á001). Multivariate analysis on the subgroup with standard-risk cytogenetics confirmed that the co-presence of circulating PCs ≥ 41, older age, Durie-Salmon stage >I and lack of maintenance adversely affected PFS, while OS was adversely affected only by lactate dehydrogenase, older age and lack of maintenance. Our results indicate that the quantification of circulating PCs by a simple two-colour FC analysis can provide useful prognostic information in newly diagnosed MM patients with standard-risk cytogenetics.

Little is known about body composition in Parkinson's disease (PD). We studied 35 patients (20 male, 15 female subjects; mean age 69.7+/-5.8 years) with advanced PD by anthropometry, dual-energy X-ray absorptiometry (DEXA), and serum 25-OH vitamin D measurement. Over 70% of patients had a disease duration of more than 4 years; all were on L-dopa treatment. Low levels of serum 25-OH vitamin D were present in 41% of the patients. The mean body mass index (BMI) was 25.3+/-4.3 kg/m(2) (range 17.1-37.3). Mid-arm muscle circumference was below the 10th percentile in 23%. For whole-body mean (+/-SD) bone mineral density, the T score was below -1 SD in 35% of patients, and the Z score was below -1 SD in 24%. Percent fat mass measured with DEXA was 30.6+/-11.4% (range 10.1-45.5) in the overall sample; it was 21.1+/-8.8% (range 10.1-30.4) in male subjects and 38.1+/-9.2% (range 25.8-45.5) in female subjects. We conclude that advanced-stage PD may show excess adiposity coexisting with depletion of lean body mass (sarcopenic obesity), in addition to decreased whole-body bone mineral density associated with low serum 25-OH vitamin D. A low level of physical activity and inadequate exposure to sunlight are likely to be among the putative causes.

The Revised International Staging System (R-ISS) stratifies patients affected by Multiple Myeloma (MM) into three distinct risk groups: R-ISS I [ISS Stage I, Standard-Risk cytogenetics and normal Lactase DeHydrogenase (LDH)], R-ISS III (ISS stage III and either high-risk cytogenetics or high LDH) and R-ISS II (any other characteristics). With the aim to verify whether the three R-ISS groups could be divided into subgroups with different prognostic factors based on the detection of Circulating Plasma Cells (CPCs) at diagnosis, in this retrospective analysis, we evaluated 161 patients with MM treated at our centre between 2005 and 2017. In all, 57 patients (33Á9%) were staged as R-ISS III, 98 (58Á3%) as R-ISS II and six (3Á6%) as R-ISS I. CPCs were detected in 125 patients (74Á4%), while in 43 patients (25Á6%) no CPCs were seen. Our analysis revealed that Overall Survival (OS) and progression-free survival (PFS) rates in R-ISS II patients were higher in the subgroup without CPCs compared to the subgroup with ≥1 CPCs (OS: 44Á7% vs. 16Á3%, P = 0Á0089; PFS: 27Á8% vs. 8Á1%, P = 0Á0118). Our present findings suggest that the detection of CPCs at diagnosis may be used as a further prognostic biomarker to improve the risk stratification of patients with MM staged as R-ISS II.

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. TTP is generally idiopathic, and the association with adenocarcinomas is extremely infrequent [1,2].We report a case of a 69-year-old woman with TTP syndrome secondary to metastatic cancer and normal ADAMTS13 (von Willebrand factor [vWf]-cleaving protease) activity and propose the pathogenetic events underlying this unusual association.The patient was admitted to our hospital because of anemia (hemoglobin level 7.8 g/dl, mean cell volume 90 fl, reticulocyte count was 12%) and thrombocytopenia (platelet count 124 × 10 3 /mm 3 ). Other laboratory findings were: serum aptoglobin <8 mg/dl, lactate dehydrogenase 1,733 UI/l, and alkaline phosphatase 603 UI/l. Partial thromboplastin time and prothrombin time were normal. Direct and indirect Coombs' tests were negative. A peripheral blood smear study demonstrated the presence of schistocytes and erythroid and myeloid precursors, with no evidence of dacryocytes. A diagnosis of TTP was established, and a program of daily plasma exchange started, but TTP did not resolve after 2 weeks.Since the increase of alkaline phosphatase could be a sign of bone disease, total body scintigraphy (99mTc) and bone marrow study were performed to rule out TTP secondary to cancer. In fact, total body scintigraphy revealed an increased diffuse uptake in all bone segments, while bone marrow aspirate and biopsy revealed neoplastic cells, suggesting metastasis of mucin-producing adenocarcinoma.Concomitantly, another set of investigations was performed to find the origin of the neoplasm. Total body computed tomography scan revealed enlarged lymph nodes in the mediastinum; small metastatic lesions of liver, spleen, and bone; and some renal and splenic ischemic lesions. However, these and other investigations (gastroscopy, mammography, and bronchoscopy) could not document any primary neoplastic lesion. A diagnosis of TTP secondary to occult adenocarcinoma diffusely metastatic to bone marrow was established, but a few days later the patient died.Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature [2][3][4][5][6][7]. The mechanism of secondary TTP is different from the idiopathic mechanism. Idiopathic TTP is believed to be caused by the deficiency of ADAMTS13, which results in the accumulation of unusually The Oncologist ® Letter

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