Outcome of allogeneic hematopoietic stem cell transplantation for mycosis fungoides and Sézary syndrome

Although allogeneic hematopoietic stem cell transplantation (HSCT) has been reported to provide prolonged remission of relapsed/refractory mycosis fungoides (MF) and Sézary syndrome (SS), its role has not been fully evaluated. Here, the outcomes of allogeneic HSCT for patients with MF/SS were retrospectively evaluated by using the registry database of the Japan Society for Hematopoietic Cell Transplantation. Forty-eight patients were evaluable and enrolled in the analysis. Median age was 45.5 years. Eighteen patients (38%) received myeloablative conditioning, and 33 (69%) received HSCT from an alternative donor. Disease status was complete or partial response in 25% of the patients and relapsed or refractory in the others. At the time of analysis, 18 patients were alive, with a median follow-up of 31.0 months (range, 3.8-31.1). Three-year overall survival (OS) and progression-free survival (PFS) were 30% (95%CI, 16-45%) and 19% (95%CI, 9-31%), respectively. Disease progression was not observed later than 17 months after transplantation. Both disease status and performance status at transplant significantly affected OS and PFS. Although our findings suggest that allogeneic HSCT provides long-term PFS in patients with MF/SS, the timing of transplantation should be decided carefully based on the disease status and the patient's condition in order to improve the outcome.     

Neurological and neurocognitive functions from intrauterine methylmercury exposure

In the 1950s, large-scale food poisoning caused by methylmercury was identified in Minamata, Japan. Although severe intrauterine exposure cases (ie, congenital Minamata disease patients) are well known, possible impacts of methylmercury exposure in utero among residents, which is likely at lower levels than in congenital Minamata disease patients, are rarely explored. In 2014, the authors examined neurological and neurocognitive functions among 18 exposed participants in Minamata, focusing on fine motor, visuospatial construction, and executive functions. More than half of the participants had some fine motor and coordination difficulties. In addition, several participants had lower performance for neurocognitive function tests (the Rey-Osterrieth Complex Figure test and Keio version of the Wisconsin card sorting test). These deficits imply diffuse brain damage. This study suggests possible neurological and neurocognitive impacts of prenatal exposure to methylmercury among exposed residents of Minamata.     

Intralymphatic histiocytosis comprises M2 macrophages in superficial dermal lymphatics with or without smooth muscles

Intralymphatic histiocytosis represents a rare reactive disorder, which is characterized by the accumulation of macrophages within lymphatic vessels and observed predominantly in upper extremities. The infiltration and preferential M2 differentiation of macrophage are observed in chronic lymphedema, and lymphedema is considered a causative factor of intralymphatic histiocytosis. However, what causes accumulation of histiocytes in the lymphatic vessels remains unclear, and investigation regarding the characteristics of the macrophages has not been evaluated. We present a case of intralymphatic histiocytosis, in which immunohistochemical staining for both macrophages and lymphatic vessels was performed to evaluate the nature of macrophages within lymphatic vessels and to determine the causative factor. Aggregated macrophages were shown to be M2 macrophages positive for CD68, CD163 and CD206 but negative for inducible nitric oxide synthase. Thick lymphatic vessels positive for D2‐40 and α‐SMA in the superficial dermis were observed. We speculate that chronic lymphedema leads to hypertrophy of lymphatic vessels with smooth muscle in the superficial dermis, which may be a kind of malformation, and these lymphatic vessels produce some chemokines that induce intralymphatic aggregation of macrophages.     

Acute Coronary Syndrome Does Not Have a Negative Impact on Outcomes after Coronary Artery Bypass Grafting in Patients with Left Main Disease

Purpose: Early and long-term outcomes of coronary artery bypass grafting (CABG) in patients with left main disease (LMD) with acute coronary syndrome (ACS) have never been assessed.Methods: Between September 2004 and April 2012, 459 patients with LMD underwent first-time isolated CABG. Of those, 191 patients had ACS and 268 did not. Early and late postoperative outcomes were compared between two groups.Results: Patients in the LMD+ACS group were older and more likely to be female. Left ventricular ejection fraction was lower in the LMD+ACS group. In both groups, bilateral internal thoracic artery grafts were used in over 90% of patients and off-pump technique in over 95%. Operative death rate was not significantly different between the groups (LMD+ACS: 2.1% vs. LMD–ACS: 0.4%). Log-rank test revealed that the actuarial survival rate (79.2 ± 3.7% vs. 81.5 ± 3.5%) and freedom from major adverse cardiac and cerebrovascular events (MACCE) (69.2 ± 4.2% vs. 67.0 ± 4.1%) were similar between groups at 7 years. Multivariate analyses demonstrated that ACS was not identified as an independent predictor of operative death, late mortality, and late MACCE.Conclusion: ACS did not have a negative impact on early and late outcomes of CABG in patients with LMD.