Giant Cell Arteritis: Focusing on Current Aspects From the Clinic to Diagnosis and Treatment.

Giant cell arteritis (GCA) is a granulomatous arteritis involving large arteries, particularly the aorta and its major proximal branches, including the carotid and temporal arteries. GCA involves individuals over 50 years old. The etiopathogenesis of GCA may involve a genetic background triggered by unknown environmental factors (eg infections), the activation of dendritic cells as well as inflammatory and vascular remodeling.

Epididymo-Orchitis in Bechet's Disease: A Review of the Wide Spectrum of the Disease.

 Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by orogenital ulcers and skin lesions; serious manifestations also include ocular, large vessel, gastrointestinal and neurological involvement. Genetic and unknown environmental factors modify the wide clinical spectrum of the disease. During the long clinical course of the disease, testicular and epididymal involvement has been reported, with scrotal pain and swelling being the most common symptoms.

Nuclear grade plus proliferation grading system for invasive ductal carcinoma of the breast: validation in a tertiary referral hospital cohort.

Objectives: For patients with invasive breast cancer, management decisions are informed by tumor grade according to the Nottingham Grading System (NGS), either on its own or as part of the Nottingham Prognostic Index (NPI). A system retaining the nuclear grade element but substituting the two subjective components, mitosis count and tubule formation, of the NGS with a proliferation index based on Ki-67 (MIB-1) has been proposed (nuclear grade plus proliferation [N+P] grading).

Methods: We validated the prognostic value of this grading system on a population of 322 women.

Expression, prognostic significance and mutational analysis of protein tyrosine phosphatase SHP-1 in chronic myeloid leukemia.

The protein tyrosine phosphatase SHP-1 dephosphorylates BCR-ABL1, thereby serving as a potential control mechanism of BCR-ABL1 kinase activity. Pathways regulating SHP-1 expression, which could be exploited in the therapeutics of TKI-resistant chronic myeloid leukemia (CML), remain unknown. Moreover, the questions of whether there is any kind of SHP-1 deregulation in CML, contributing to disease initiation or evolution, as well as the question of prognostic significance of SHP-1, have not been definitively answered.

Diffuse Calcifications of the Spleen in a Woman with Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease, which affects a wide variety of organs including the spleen. Splenic involvement in SLE includes conditions such as splenomegaly, hyposplenism, infarction, and spontaneous rupture. However, only a few cases of splenic calcifications in patients with SLE have been reported.

High Frequency of Thyroid Disorders in Patients Presenting With Neutropenia to an Outpatient Hematology Clinic STROBE-Compliant Article.

Granulopoiesis abnormalities have been described in association with thyroid disorders (TD). However, data regarding systematic evaluation of adult neutropenia and concurrent or prior TD are scarce. To investigate the frequency of TD among patients presenting with neutropenia, and the immunophenotypic and immunologic profile of neutropenic patients with concomitant thyroidopathy.

Cytomegalovirus meningoencephalitis after rituximab treatment for primary central nervous system lymphoma.

The efficacy and safety of rituximab against B-cell lymphomas is well established. However, there has been an increased incidence of infectious complications after rituximab treatment, mostly hepatitis B reactivation and progressive multifocal leukoencephalopathy. This is the case of a 67-year-old patient with primary central nervous system lymphoma, who developed cytomegalovirus meningoencephalitis after receiving high-dose chemotherapy and rituximab.

Gastrointestinal manifestations of Behçet's disease: advances in evaluation and management.

Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by orogenital ulcers and skin lesions; serious manifestations also include ocular, large vessel, gastrointestinal and neurological involvement. Genetic and unknown environmental factors customise the wide clinical expression of the disease. Gastrointestinal involvement is not unusual, albeit with a highly variable frequency among different ethnic populations.

Effects of anthracyclines on aortic distensibility in patients with lymphomas: a prospective study.

Introduction: Anthracyclines have been widely used in the treatment of haematological malignancies. Their major adverse effect is cardiomyopathy, but their effect on vascular elasticity has not been completely elucidated. The aim of the present study was to investigate the effects of anthracyclines on aortic elastic properties in patients with lymphomas.

The presence of CD55- and/or CD59-deficient erythrocytic populations in patients with rheumatic diseases reflects an immune-mediated bone-marrow derived phenomenon.

Background: Complement has the potential to provoke severe impairment to host tissues, as shown in autoimmune diseases where complement activation has been associated with diminished CD55 and/or CD59 expression on peripheral blood cell membranes. The aim of this study was to evaluate the presence of CD55- and/or CD59-deficient erythrocytic populations in patients with different rheumatic diseases and to investigate possible correlations with clinical or laboratory parameters.

Material And Methods: CD55 and CD59 expression was evaluated in erythrocytes of 113 patients with rheumatic diseases, 121 normal individuals, and 10 patients with paroxysmal nocturnal hemoglobinuria (PNH) using the Sephacryl gel microtyping system.

Survivin messenger RNA levels in Epstein-Barr virus-positive patients with leukemic low-grade B-cell lymphomas expressing the latent membrane protein 1: evidence of apoptotic function?

Background: Epstein-Barr virus (EBV) is a ubiquitous pathogen that chronically infects B lymphocytes and is implicated in the pathogenesis of lymphoproliferative diseases. Latent membrane protein 1 (LMP1), the major oncoprotein of the virus, has been shown to inhibit apoptosis and trigger survivin expression in malignant cell lines. LMP1 expression has been detected in patients with chronic lymphocytic leukemia, but its properties have not been studied in patients with low-grade B-cell lymphomas.

Evidence for regulation of oxidative stress by latent membrane protein 1 oncoprotein in patients with low-grade leukemic B cell lymphoma with latent Epstein-Barr virus infection.

The role of latent Epstein-Barr virus (EBV) infection in the pathogenesis of low-grade B cell non-Hodgkin lymphoma (B-NHL) has not been studied. We therefore investigated the incidence of latent EBV infection in a group of patients with leukemic low-grade B-NHL, as well as the incidence of viral latent membrane protein 1 (LMP1) oncoprotein expression in the same patient group. Furthermore, in an attempt to elucidate the role of this viral oncoprotein in non-EBV-related lymphomas, we correlated the expression of LMP1 with the level of oxidative stress, a parameter related to apoptosis.

The lung in the spectrum of antiphospholipid syndrome.

Patients with antiphospholipid syndrome may develop various lung manifestations. The lung complications that have been described so far are pulmonary thromboembolic disease, pulmonary hypertension, acute respiratory distress syndrome, primary thrombosis of large and small lung vessels, diffuse alveolar haemorrhage, fibrosing alveolitis and postpartum syndrome. Clinicians should be aware of these conditions as in most of these cases, timely diagnosis and treatment is needed.

Adalimumab or cyclosporine as monotherapy and in combination in severe psoriatic arthritis: results from a prospective 12-month nonrandomized unblinded clinical trial.

Objective: To assess the efficacy and safety of adalimumab or cyclosporine (CYC) as monotherapy or combination therapy for patients with active psoriatic arthritis (PsA), despite methotrexate (MTX) therapy.

Methods: A prospective 12-month, nonrandomized, unblinded clinical trial of 57, 58, and 55 patients who received CYC (2.5-3.

Kawasaki disease: current aspects on aetiopathogenesis and therapeutic management.

Kawasaki disease (KD) is a vasculitis that affects mainly children of 6 months to 4 years old. It is important to be early recognised so as to limit the inflammatory cascade that may lead to aneurysmatic dilatations of coronary arteries. The causative agent of KD has not been still indentified and the aetiopathogenetic theories are based on epidemiologic, laboratory and histological data.

Serum anti-Saccharomyces cerevisiae antibodies in Greek patients with Behcet's disease.

We tested 59 Greek patients with Behcet's Disease (BD) for serum anti-Saccharomyces cerevisiae antibodies. No increase of these antibodies was detected in the cases compared to 55 healthy unrelated blood donors from the same population. This finding is in contrast with the correlation between Saccharomyces cerevisiae antibodies and BD as reported in other populations.

Behavior of coagulation factors and normal inhibitors of coagulation during the acute phase of myocardial infarction.

Acute coronary syndromes are characterized by increased endothelial activation. The aim of this study was to investigate the behavior of coagulation factors V, VII, VIII and normal inhibitors antithrombin III and protein C during the acute phase of myocardial infarction. Thirty-six patients (27 men, nine women) and 35 normal individuals were studied during the acute phase of myocardial infarction, in the first 24 h.

Evaluation of current therapeutic strategies in Behçet's disease.

Behçet's disease (BD) is a chronic relapsing vasculitis with multifunctional pathogenesis. The mucocutaneous and ocular lesions are the commonest manifestations, but BD also affects the musculoskeletal, intestinal, cardiac, and central nervous system. BD therapy is based on the suppression of the inflammatory process, using immunomodulating and immunosuppressive agents.

Etiopathogenesis of Behçet's disease with emphasis on the role of immunological aberrations.

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder of unknown origin consisting of oral aphthous ulcers, ocular symptoms, skin lesions, and genital ulcerations. It has many features in common with systemic vasculitides and is more prevalent in countries along the ancient Silk route. Immune-mediated mechanisms play a major role in the pathogenesis of the disease, and inflammatory mediators are also involved.

Kikuchi's lymphadenopathy: a relatively rare but important cause of lymphadenopathy in Greece, potentially associated with the antiphospholipid syndrome.

Kikuchi-Fujimoto disease is a form of reactive lymphadenopathy, which was firstly described in Japan, but is uncommon in the Western world. We retrospectively reviewed the medical records of nine cases of adult or adolescent Kikuchi's disease diagnosed in a single Haematology Unit in Athens, Greece between 1990 and 2006. The median age of the patients was 25 years (14-40) and 8/9 were females.

The role of the Platelet Function Analyzer (PFA)-100 and platelet aggregometry in the differentiation of essential thrombocythemia from reactive thrombocytosis.

Introduction: The most crucial component of all diagnostic criteria for essential thrombocythemia (ET) has been the exclusion of reactive thrombocytosis (RT). Our aim was to evaluate the diagnostic performance of the PFA-100 collagen-epinephrine (CEPI) cartridge test and epinephrine-induced aggregometry individually, but mainly combined, in the differentiation of ET from RT.

Materials And Methods: 26 patients with ET and 25 with RT were studied.

Primary adult-onset macrophage activation syndrome with multisystemic tissue phagocytosis.

The histiocyte disorders are divided into the following 3 categories according to the specific lineage of the histiocytes involved and their biological behavior: the dendritic cell-related disorders, which include Langerhans cell histiocytosis and dermal dendrocyte disorders; the macrophage cell disorders, hemophagocytic lymphohistiocytosis being the main entity; and the malignant histiocyte disorders. We present a case of a 36-year-old woman who was referred to our hospital because of fever of unknown origin, lethargy, anemia, and impaired hepatic function. Following a thorough investigation, we diagnosed extensive histiocyte-mediated phagocytosis in many areas (skin, liver, bone marrow), without any identifiable cause.

Soft tissue gas gangrene: a severe complication of emphysematous cholecystitis.

Soft tissue gas gangrene with myonecrosis is a severe complication of traumatic and non-traumatic conditions with a potentially lethal outcome. Emphysematous cholecystitis is a complication of acute cholecystitis, which is characterized by air accumulation in the gallbladder wall and is reported in the literature as a rare causative factor of soft tissue gas gangrene. Here we report 4 patients who developed soft tissue gas gangrene as a complication of emphysematous cholecystitis.