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A letter to the editor is presented that examines the association between sarcoidosis and haematological malignancies.

The outcomes of 55 consecutive haemato-oncology patients admitted to the intensive care unit (ICU) were retrospectively analysed. Twenty-eight patients were admitted following haemopoietic stem cell transplantation (HSCT). Thirty-nine patients were admitted with respiratory failure, and all patients required respiratory support. Seventeen patients survived to be discharged from ICU, with an actuarial 1-year survival of 18%. Overall survival between patients who received intensive chemotherapy and those who underwent allogeneic HSCT was not significantly different (19% vs. 10%, P = 0·19). None of the nine myeloablative HSCT recipients survived (median survival: 9 d). Six of the 15 reduced-intensity conditioned HSCT recipients survived beyond 1 year (median survival: 1050 d, range: 438–1437). [ABSTRACT FROM AUTHOR]

Bone marrow (BM) is the preferred graft source for hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) compared with mobilized peripheral blood stem cells (PBSCs). We hypothesized that this recommendation may not apply to those regions where patients present later in their disease course, with heavier transfusion load and with higher graft failure rates. Patients with SAA who received HSCT from an HLA-matched sibling donor from 1995 to 2009 and reported to the Center for International Blood and Marrow Transplant Research or the Japan Society for Hematopoietic Cell Transplantation were analyzed. The study population was categorized by gross national income per capita and region/countries into 4 groups. Groups analyzed were high-income countries (HIC), which were further divided into United States-Canada (n = 486) and other HIC (n = 1264); upper middle income (UMIC) (n = 482); and combined lower-middle, low-income countries (LM-LIC) (n = 142). In multivariate analysis, overall survival (OS) was highest with BM as graft source in HIC compared with PBSCs in all countries or BM in UMIC or LM-LIC (P < .001). There was no significant difference in OS between BM and PBSCs in UMIC (P = .32) or LM-LIC (P = .23). In LM-LIC the 28-day neutrophil engraftment was higher with PBSCs compared with BM (97% versus 77%, P = .002). Chronic graft-versus-host disease was significantly higher with PBSCs in all groups. Whereas BM should definitely be the preferred graft source for HLA-matched sibling HSCT in SAA, PBSCs may be an acceptable alternative in countries with limited resources when treating patients at high risk of graft failure and infective complications., (Copyright © 2016 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.)

Although myeloid sarcomas (MS) are frequently associated with acute myeloid leukaemia (AML), the occurrence of mediastinal MS is a much rarer event. The authors describe a distinct group of three AML patients with mediastinal MS and complex cytogenetics presenting at their centre over a 7-year period. Clinical features consistent with superior vena caval obstruction were noted at presentation in two of the three patients. Mediastinal mass was detected on routine chest radiography, and biopsies confirmed the diagnosis of MS. One patient relapsed after consolidation chemotherapy and died from progressive disease. Two patients underwent allogeneic haemopoietic stem cell transplant, but succumbed to transplant related complications. Review of mediastinal MS over the last 20 years shows that a significant proportion of patients have complex cytogenetic abnormalities and a poor long-term prognosis. Early and accurate diagnosis is essential and patients should be managed along the lines of high risk AML.

Alemtuzumab is a humanised monoclonal antibody against CD52 used as an immunosuppressive agent in allogeneic HSCT. Regimens including alemtuzumab for allogeneic haematopoietic stem cell transplant (HSCT) conditioning have been associated with an increased incidence of viral complications. Patients with prior toxoplasma exposure undergoing alemtuzumab containing HSCT could therefore be expected to be at a higher risk of toxoplasma reactivation. We conducted a retrospective review of 220 alemtuzumab based allogeneic HSCT performed over a 4 year period, of which 202 were reduced intensity conditioning (RIC) HSCT. A total of 67 patients (30%) in whom the pre-transplant recipient toxoplasma IgG test was positive were classified as high-risk for toxoplasma infection. All patients started trimethoprim/sulfamethoxazole prophylaxis following HSCT when the neutrophil count was > or = 1x10(9)/l. Two high-risk patients developed toxoplasma invasive disease with cerebral involvement at 2 and 4 months post-transplantation respectively. The incidence of toxoplasma disease in the entire cohort and amongst high-risk patients was 0.9% and 3.0% respectively. Despite in vivo T-cell depletion with alemtuzumab, the incidence of toxoplasma disease in our cohort was comparable with previously reported T-cell replete HSCT studies.

The last decade has witnessed a multistep evolution in the understanding of the natural history, clinical manifestations, and some of the molecular mechanisms that underlie the ineffective hematopoiesis and leukemic transformation in the myelodysplastic syndrome (MDS). The international prognostic scoring system, FAB, and WHO classifications have helped define specific subgroups with their characteristic cytogenetic, molecular and immunological abnormalities. Until recently the mainstay of the treatment has been entirely supportive with blood and platelet transfusions. What is increasingly manifest now is the considerable excitement generated by the emergence of novel therapeutic strategies based on painstaking research findings from the laboratories. In Section I, Dr. Alan List reviews the therapeutic strategies with the specific emphasis on the relevance of molecular mechanism of apoptosis and targeted therapies using small molecules. Of particular interest is the excitement surrounding the clinical benefit obtained from potent immunomodulatory derivative (IMiD) of thalidomide CC5013. The review provides an update of the role of small molecule inhibitors of VEGF receptor tyrosine kinase, arsenic trioxide, oral matrix metalloprotease inhibitors, farnesyl transferase inhibitors, and imatinib mesylate in the treatment of MDS subgroups. In Section II, Dr. Steven Gore describes the results of clinical trials of inhibitors of DNA methylation such as 5 azacytidine (5 AC) and 5-aza 2-deoxycytidine (Decitabine). The review also provides an update on the rationale and results obtained from the combination therapy using histone deacetylases (HDAC) and DNA methyltransferase inhibitors in the treatment of MDS. In Section III, Professor Ghulam Mufti and Dr. Aloysius Ho describe the role of bone marrow transplantation with particular emphasis on recent results from reduced-intensity conditioned transplants, exploiting the graft versus leukemia effect without significant early treatment-related mortality. The section provides an update on the results obtained from the manipulation of the host's immune system with immunosuppressive agents such as ALG and/or cyclosporine A.

The therapeutic options for the indolent non-Hodgkin's lymphomas have evolved significantly over the years. However, at present, allogeneic haematopoietic stem cell transplantation (HSCT), despite its significant transplant associated complications, is still the only option which may be delivered with curative potential. The debut of reduced-intensity conditioning regimens has extended the applicability of allogeneic transplants to groups of patients ineligible for conventional myeloablative allografts. However, there are still substantial hurdles to overcome, namely that of relapse, graft-versus-host disease and infection. Whilst follow-up is still relatively limited for a disease group with a median survival of up to 10 years, early results are extremely encouraging and further studies are warranted.