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Introduction: Primary plasma cell leukemia (PPCL) is one of the most aggressive hematological malignancies. The prognosis of PPCL patients remains poor, although some improvements have been made in recent years. Areas covered: In this review recent clinical and biological advances in PPCL are reported. Some recommendations for the practical management of these patients are provided, with a particular focus on the role of novel agents and transplant procedures. A brief description of the currently ongoing clinical trials with new drugs is also enclosed. Expert opinion: PPCL still represents a difficult challenge for all hematologists. Here the authors provide a personal view on how the current, generally unsatisfactory results in this neoplastic disorder could be improved. In particular, dedicated studies exploring alternative therapies are necessary and eagerly awaited. Such studies should possibly be based on new biological information that could be of help in identifying novel genetic biomarkers for risk stratification and new actionable molecular targets.

Primary plasma cell leukemia (PPCL) is a rare and aggressive variant of multiple myeloma. This disease is associated with a very poor prognosis, and unfortunately it has not significantly improved during the last three decades. Autologous stem cell transplantation is generally recommended in eligible patients, but survival in transplanted PPCL patients is significantly lower than that of multiple myeloma. Recent preliminary data indicate that new drugs, in particular lenalidomide and bortezomib, could significantly improve the clinical outcome of PPCL, increasing response rate and duration, as well as survival. In this review we report an updated literature analysis about the current therapeutic scenario of PPCL, with a particular focus on the use of novel agents., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Iron overload is a frequent consequence in transfusion-dependent myelodysplastic syndromes (MDSs), which often requires iron chelation therapy (ICT). Interestingly, ICT may sometimes induce a hematologic improvement that leads to significant reduction or complete interruption of blood transfusions. This phenomenon has been recently described in MDS treated with the new oral chelator deferasirox. Here we briefly review the literature about this phenomenon and discuss the possible biological mechanisms underlying hematologic effects of deferasirox in MDS, starting from a new paradigmatic case in whom both hemoglobin level and platelet count improved, inducing transfusion-independence, soon after starting the treatment with deferasirox., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Background and Purpose: Physical therapy is frequently offered to community-dwelling frail elderly as part of home care rehabilitation programs. A better knowledge of predictors of rehabilitative success could allow a better targeting of limited resources. The purpose of this study is to evaluate the association of medical indicators of health status on functional recovery during rehabilitation of frail elderly living in the community., Subjects: Subjects included 598 patients aged 70 years or more admitted consecutively to a home care rehabilitation program from January 2004 to December 2004., Methods: A comprehensive geriatric multidisciplinary evaluation was offered to all patients, based on the Minimum Data Set for Home Care (MDS-HC) assessment form. Predictors of functional recovery were identified by a multiple logistic regression model. Data analyses were based on the items contained in the MDS-HC form., Results: An improvement in ADL score was seen in 33% of patients, while the others remained unchanged or worsened. In multivariate analysis the negative predictors of functional improvement were as follows: cognitive impairment (OR 0.67; CI 0.60-0.74), depression (OR 0.89, CI 0.82-0.96), visual (OR 0.32, CI 0.21-0.50) and hearing impairment (OR 0.42, CI 0.27-0.67), and urinary (OR 0.21, CI 0.14-0.33) and bowel incontinence (OR 0.16, CI 0.10-0.26)., Conclusions: Cognitive impairment, depressed mood, sensory impairment, and incontinence are key factors that need to be assessed in order to individuate patients at risk of failure in rehabilitation. A targeted intervention in these areas could offer the opportunity of improving rehabilitation outcome.

In a patient with Behcet's syndrome the finding of five times the normal level of serum transaminase accompanied by weakness may be correlated to the syndrome itself. We performed needle biopsy of the liver in our patient which showed piecemeal necrosis and portal lympho-monocytic infiltration extending into the lobule. It is interesting that HLA B5 and B27 antigens were present. Simultaneous occurrence of chronic active hepatitis and Behcet's syndrome may imply the same autoimmune pathogenesis, as we suggest in this work.

Relapsing polychondritis (RP) is a rare disease characterized by auricular, nasal and respiratory tract chondritis, ocular inflammation, inflammatory polyarthritis and cardiovascular abnormalities. Here we describe a patient with a five-year history of mild nasal and auricular chondritis, which suddenly developed into a severe aortic insufficiency with rest dyspnea. The pathogenesis and the management of this rare connective tissue disease are discussed.

Haematic concentrations of catecholamines were found to be higher in cirrhotic patients with ascites, than cirrhotic patients without ascites and controls. In compensated and decompensated cirrhosis, different forms of sympathetic nervous activity were observed. The high catecholamine values in cirrhotic patients and the activation of the renin angiotensin-aldosterone system suggest the use of beta-blockers to reduce sodium-water retention.

Two patients with Behçet's syndrome had severe esophageal involvement. Both presented the complete clinical pattern (oral, genital, and eye lesions) of Behçet's syndrome. In both, esophageal lesions were associated with other gastrointestinal manifestations, in the first a transient episode of ulcerative ileitis, and in the second patient colitis. In both, the differential diagnosis with inflammatory bowel disease was very difficult. Histology of esophageal lesions was nonspecific; no vasculitis was found. Both patients improved on steroid therapy. Esophageal lesions also improved endoscopically and histologically with steroid therapy. When there is obvious gastrointestinal involvement in Behçet's syndrome, the differential diagnosis from inflammatory bowel disease is very difficult.