Neutrophilic leukocytosis in advanced stage polycythemia vera: hematopathologic features and prognostic implications

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nodal peripheral t cell lymphoma with secondary granulomatous cutaneous involvement

Sir, The presence of a granulomatous pattern has rarely been described in cutaneous lymphomas, mycosis fungoides (MF) being the variant more frequently reported (1 – 4). The aetiology of granulomatous reactions remains unclear and its prognostic value is still disputed, although a favourable influence on outcome has been hypothesized (5). We report here the case of an 87-year-old man with nodal peripheral T-cell lymphoma (PTL) with secondary cutaneous involvement, who clinically presented with multiple eruptive papular and nodular skin lesions on the upper part of the body and histologically showed a strong granulomatous reaction in the skin

Homing of peripherally injected bone marrow cells in rat after experimental myocardial injury

Background and objectives: significant progress has been achieved during the past 10 years in cell transplantation and recent research has focused on the possibility of improving ventricular function after myocardial infarction. Most studies in the field of cardiac tissue repair are performed by direct intramyocardial injection of cells of different origin. Since this approach requires a surgical intervention, in this study we investigated the feasibility of non-invasive administration of bone marrow mononuclear cells (BMMNCs) by assessing the fate of peripherally injected, purified, labeled cells in cryodamaged hearts. Design and methods: ten donor and ten recipient inbred isogenic adult (4 weeks old) Fisher rats were used as models to mimic autologous transplantation. Myocardial damage was obtained in recipient rats by placing a frozen metal probe on the anterior left ventricular wall for 15 seconds (freeze-thaw injury technique). BMMNCs were purified and labeled with a red fluorescent cell dye. Seven days after the injury about 15-25x10(6) cells were infused through the femoral vein of recipient rats. Seven days after the infusion, the heart, lungs, liver, kidneys, spleen and thymus were harvested to track transplanted cells. RESULTS: Labeled cells were found only in the injured area of the heart and not in the normal tissue, and a limited number of cells were identified in the spleen of all the animals. Most of the labeled cells in the infarcted area were Thy-1(+) and some were CD34(+). Interpretation and conclusions: our data suggest that peripherally injected BMMNCs can traffic through the circulation to the site of damage; we hypothesize that tissue injury leads to the priming of a cytokine cascade acting as chemoattractant for the infused cells

Mediastinal lymphadenopathies and skin lesions in a 49-year-old Sinhalese man

Leprosy is a neglected disease sporadically reported in high-income countries. Skin lesion and peripheral nerve involvement represent most common manifestations. Mediastinal lymphadenopathy in the absence of superficial lymph nodes involvement is very rare. Atypical or rare clinical presentations of disease may delay diagnosis and therapy and cause potential life-threatening manifestations and disabilities. We describe the case of a 49-year-old Sinhalese man who was admitted to our hospital with a one-month history of peripheral neurological symptoms and skin lesions on lower limbs. CT scan showed the presence of mediastinal lymphadenopathies without lung parenchymal and superficial lymph nodes involvement. Endobronchial ultrasound-guided transbronchial needle aspiration showed the presence of granulomas while skin biopsy revealed dermo-hypodermic granulomas with perineural lymphohistiocytic inflammatory reaction. Fite-Faraco staining demonstrated the presence of acid-fast bacilli in both lymph nodal and skin biopsy and polymerase chain reaction was positive for Mycobacterium leprae. Multibacillary leprosy was then diagnosed

Blast Transformation in Myeloproliferative Neoplasms: Risk Factors, Biological Findings, and Targeted Therapeutic Options

Myeloproliferative neoplasms represent a heterogenous group of disorders of the hematopoietic stem cell, with an intrinsic risk of evolution into acute myeloid leukemia. The frequency of leukemic evolution varies according to myeloproliferative neoplasms subtype. It is highest in primary myelofibrosis, where it is estimated to be approximately 10–20% at 10 years, following by polycythemia vera, with a risk of 2.3% at 10 years and 7.9% at 20 years. In essential thrombocythemia, however, transformation to acute myeloid leukemia is considered relatively uncommon. Different factors are associated with leukemic evolution in myeloproliferative neoplasms, but generally include advanced age, leukocytosis, exposure to myelosuppressive therapy, cytogenetic abnormalities, as well as increased number of mutations in genes associated with myeloid neoplasms. The prognosis of these patients is dismal, with a medium overall survival ranging from 2.6–7.0 months. Currently, there is no standard of care for managing the blast phase of these diseases, and no treatment to date has consistently led to prolonged survival and/or hematological remission apart from an allogeneic stem cell transplant. Nevertheless, new targeted agents are currently under development. In this review, we present the current evidence regarding risk factors, molecular characterization, and treatment options for this critical subset of myeloproliferative neoplasms patients

Severe chronic diarrhea and maculopapular rash: A case report

Systemic mastocytosis (SM) is a heterogeneous disease of the bone marrow characterized by abnormal growth, accumulation and activation of clonal mast cells (MCs). We report a case of SM with multi-organ involvement. A 30-year-old man presented with diarrhea, flushing, maculopapular rash with itching and weight loss. The upper and lower gastrointestinal endoscopies showed macroscopic involvement of stomach and duodenum; mucosal samples from stomach, duodenum, colon and distal ileum showed mucosal infiltration by large, spindle-shaped MCs with abnormal surface molecule expression (CD2 and CD25), a picture fully consistent with SM, according to the World Health Organization diagnostic criteria. A computed tomography scan showed diffuse lymphadenopathy, hepatosplenomegaly and diffuse small bowel involvement. Bone marrow aspirate and biopsy were diagnostic for SM; serum tryptase levels were increased (209 ng/mL, normal values < 20 ng/mL). The conclusive diagnosis was smouldering SM. There were no therapeutic indications except for treatment of symptoms. The patient was strictly followed up because of the risk of aggressive evolution

A network between Italian transplantation Centers supported by the Ministry of Health to expand cadaveric donor pool for organ transplantation (SITF Project).

The research program "Innovative strategies to expand cadaveric donor pool for liver transplantation" (SITF Project) has been formally approved in 2002 by Italian Ministry of Health (IMH) as a two-years experimental project. The main goals of the SITF Project are to improve matching criteria for split liver transplantation (SLT) in both pediatric and adult recipients, to promote an increase of the SLT/full-size liver transplantation ratio at the national level, and to establish shared criteria for SLT in two adult recipients. The original executive plan of SITF Project prefigured the participation of both the Italian transplantation Centers with more advanced experience in the field of SLT, and institutional partnership such as Nord Italia Transplant (NITp) and Italian National Transplant Center. A first Web public area concerning the SITF Project has been activated, as well as a reserved Web area with the aim to shared cadaveric donors and patients in the waiting lists between Operative Units involved in the research program. For this objective, a specific Oracle-based database able to perform an automatic matching between a single cadaveric donor and two potential adult recipients has been released in the beta version. The SITF Project represents a new approach in the management of SLT for two adults recipients in Italy, and a model for a functional network between Italian transplantation Centers

Impact of different donor age cut-offs on adult recipient survival after liver transplantation: a single-center analysis.

The increasing number of patients awaiting liver transplantation (LT) has forced to use cadaveric donors (CD) with suboptimal characteristics. Of these, donor age is the most investigated parameters. Although excellent outcomes were observed for LT performed by using CD aged >60 years, European Liver Transplant Registry (ELTR) found an increased risk by using CD with >55 years, and the Italian National Transplant Center has recently assumed the CD age >60 years as a potential risk factor for LT. In this study, a single-center analysis has been carried out by stratifying CD for three different age cut-offs (=55/>55, 60, and =65/>65 years), and then evaluating their effect on LT outcome. Although no significant difference in 6-month and 1-year patient or graft survival occurred after stratification for each donor age cut-off, a better survival was observed in LT performed with livers procured from CD >55 years. A significant increase in cold ischemia time (CIT) was observed in LT performed with grafts procured from CD =55 and =65 years (P = 0.007), and an inverse correlation between overall CIT and donor age occurred (r = -0.300; P = 0.0022). However, no impact on 1-year patient survival was found by introducing in univariate logistic regression models CIT, as well as overall donor age, recipient age, and ELTR diagnostic categories. The results of this study suggests the suitability of CD >55 years for LT, and the need to further investigate the cut-off value for CIT-related increasing risk