Monoclonal gammopathy associated with dermatological manifestations are a well-recognized complication. These skin disorders can be associated with infiltration and proliferation of a malignant plasma cells or by a deposition of the monoclonal immunoglobulin in a nonmalignant monoclonal gammopathy. These disorders include POEMS syndrome, light chain amyloidosis, Schnitzler syndrome, scleromyxedema and TEMPI syndrome. This article provides a review of clinical manifestations, diagnostics criteria, natural evolution, pathogenesis, and treatment of these cutaneous manifestations.

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BackgroundEarly assessment of cognitive symptoms is an issue in geriatrics. This study investigated the delay from the onset of cognitive symptoms to initial clinical assessment and its associations with patients’ sociodemographic and clinical characteristics.MethodsThis is a cross-sectional retrospective study using medical chart review of 316 patients referred for assessment to a university-affiliated memory clinic. Symptom duration was self-reported by patients/carers. Severity of symptoms assessed by the MoCA and FAST instruments was compared according to delay duration (≥3 years vs. <3 years) using chi-squared tests. Logistic regression was used to determine the association between patients’ characteristics and long symptom duration (≥3 years).ResultsAt the initial assessment, 29.4% of patients reported experiencing cognitive symptoms for ≥3 years. They were more likely to have MoCA scores ≤17 (47.8 vs. 34.1%; p=.023) and FAST scores ≥5 (21.5 vs. 10.8%; p=.012). They were also significantly older than 75 years (75–84 yr: OR=2.22 [95%CI: 1.11–4.41]; ≥85 yr: 4.36 [2.08–9.11]), presented more depressive symptoms (2.37 [1.40–4.02]), and were less likely to live alone (0.55 [0.31–0.96]).ConclusionsA significant proportion of patients had cognitive symptoms for years when initially assessed, which delayed diagnosis and management. Stigma, depression, and compensatory help from carers may contribute to this delay.

Background: Long-term survival in patients progressing after tandem autologousallogeneic stem cell transplant (SCT) has been reported, suggesting a persistent graftvs-myeloma (GvM) effect even after post-transplant progression. Methods: In order to confirm this observation, we updated the results of our previously published cohort of 92 newly diagnosed myeloma patients who received tandem transplant and compared them with 81 contemporary patients who received autologous transplant only. Results: With a median follow-up of 13.1 and 10.2 years, respectively, median overall survival (OS) in the tandem group has not been reached, compared with 6.1 years after auto-SCT (P ≤ .001). Disease progression occurred less frequently after tandem transplant, with an estimated 10-year cumulative incidence of 49% vs 76% (P ≤ .001). Cumulative incidence of extensive chronic graft-vs-host disease (cGVHD) was high at 83%, with modest benefits on OS (60% vs 49%, P = .550) but sharp improvement of progression-free survival (PFS; 55% vs 10%, P = .002) at 10 years associated with development of cGVHD. After first progression, median OS was 5.8 years in tandem and 5.2 years in the auto-group (P = .062); median PFS was also similar. Conclusion: Despite confirmation of better outcomes after upfront tandem transplant, our data do not support persistence of a strong, clinically significant graft-vsmyeloma effect after first progression, emphasizing the need to better characterize the GvM effect.

Allogeneic stem cell transplantation (SCT) remains the best treatment for severe aplastic anemia. The current priority order for selection of a stem cell donor is an HLA-identical sibling, a matched unrelated donor, and a haplo-identical donor when the first two are unavailable. 1 Cord blood (CB) transplant also offers a curative option, although few adult cases have been reported due to insufficient number of progenitor cells leading to high risk of graft failure and infections. 1,2 The use of two CB units may accelerate engraftment but has fallen into disfavor due to higher costs of procurement and significant incidence of acute graft-vs-host disease (GVHD). 3 CB expansion can fasten hematologic reconstitution, but no experience in severe aplastic anemia has yet been reported, likely due to fear of graft rejection and lack of availability of expanded CBs. 3 UM171, a first-in-class pyrimidoindole derivative, allows expansion of hematopoietic stem cells by an average of 35-fold within 7 days by enhancing the human long-term-repopulating hematopoietic SC self-renewal independently of aryl hydrocarbon receptor suppression. 4 With a median follow-up of 18 months, a recent phase I/II study in 22 patients with high-risk malignancies has shown that UM171-expanded CB grafts can lead to prompt engraftment, a

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Major progress in the treatment of multiple myeloma has been made in the last several years. However, myeloma remains incurable and patients with high-risk cytogenetics or advanced stage disease have an even worsen survival. Only allogeneic transplantation may have curative potential in some patients. However, the high non-relapse mortality and incidence of chronic graft-versus-host disease have raised controversy regarding this procedure. In this review, we will address the role of upfront and delayed allogeneic transplant.