Abstract:
:Hepatic involvement in familial Mediterranean fever (FMF) ranges from a nonspecific increase in liver enzymes to cryptogenic cirrhosis, and the liver is mostly involved in patients bearing the M694V MEFV mutation in homozygosis. A 44-year-old Jewish woman with FMF developed nonalcoholic steatohepatitis during colchicine treatment (2,5 mg per day), confirmed by both elastography and liver biopsy. Therefore, combined therapy with the interleukin-1 (IL-1) blocking agent canakinumab (150 mg every four weeks) and colchicine (at a reduced dose of 1.5 mg per day) was started. Three months later, transaminases became normal, and after further six months, there was a marked improvement of liver fibrosis. IL-1 blockade has the power to halt or mitigate liver involvement in FMF patients. However, further experience is required to assess its therapeutic potential in the most severe patients with the hepatic disease who are partially responsive to long-term prophylaxis with colchicine.
journal_name
Mediterr J Hematol Infect Disauthors
Massaro MG,Pompili M,Sicignano LL,Pizzolante F,Verrecchia E,Vecchio FM,Rigante D,Manna Rdoi
10.4084/MJHID.2020.059subject
Has Abstractpub_date
2020-09-01 00:00:00pages
e2020059issue
1issn
2035-3006pii
mjhid-12-1-e2020059journal_volume
12pub_type
abstract:INTRODUCTION:Bone marrow aspiration and biopsy still remains as one of the vital tests for confirmation of diagnosis of visceral Leishmaniasis. The aim of the present study is to assess the relative frequency of common, uncommon and atypical hematological findings in cases of Visceral Leishmaniasis. MATERIALS #ENTITYS...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2011.035
更新日期:2011-01-01 00:00:00
abstract::Sickle cell disease (SCD) is an extremely heterogeneous disease that has been associated with global morbidity and early mortality. More effective and inexpensive therapies are needed. During the last five years, the landscape of the pharmacotherapy of SCD has changed dramatically. Currently, 54 drugs have been used o...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2020.010
更新日期:2020-01-01 00:00:00
abstract::The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis.Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Chronic myeloprolifera...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2011.063
更新日期:2011-01-01 00:00:00
abstract::POEMS syndrome is a rare, chronic and disabling condition. The causes of this condition remain unknown; however, chronic overproduction of proinflammatory cytokines appears to be a major contributor. Early diagnosis is essential to start treatment before the clinical state of the patient becomes compromised. A complet...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2017.051
更新日期:2017-09-01 00:00:00
abstract::Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder predominantly of the lymph nodes, which mostly occurs in children and young adults typically presenting with lymphadenopathy. Our case is of a 63 year-old African-American male who presented with subjective fever, weight loss, bilateral ax...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type:
doi:10.4084/MJHID.2013.022
更新日期:2013-04-10 00:00:00
abstract::This retrospective study was conducted to analyze clinical and laboratoristic parameters to individuate specific differences and facilitate differential diagnosis between Measles and Kawasaki Disease (KD) at first evaluation in an emergency room. We found similar clinical features as duration of fever and number of KD...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2018.033
更新日期:2018-05-01 00:00:00
abstract:Objective:This study focused on the efficacy and safety of thalidomide for patients with β-thalassemia in a multicenter trial. Methods:Patients with non-transfusion-dependent thalassemia (NTDT) or transfusion-dependent thalassemia (TDT), who were unable to pursue conventional therapy with transfusion and chelation, we...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2020.021
更新日期:2020-05-01 00:00:00
abstract::Human coronaviruses (HCoVs) commonly cause mild upper-respiratory tract illnesses but can lead to more severe and diffusive diseases. A variety of signs and symptoms may be present, and infections can range in severity from the common cold and sore throat to more serious laryngeal or tracheal infections, bronchitis, a...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2020.042
更新日期:2020-07-01 00:00:00
abstract::One case of acute panmyelosis with myelofibrosis (APMF) is here reported. A 45 year old male presented with abrupt onset of rapidly progressing low backache, weakness and pancytopenia. On examination there was no organomegaly. Peripheral blood examination revealed normocytic normochromic red blood cells with 10% circu...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type:
doi:10.4084/MJHID.2013.042
更新日期:2013-06-04 00:00:00
abstract::The primary objective of this review was to update and discuss the current concepts and the results of the treatment of acute lymphoblastic leukemia (ALL) in adolescents and young adults (AYA). After a brief consideration of the epidemiologic and clinicobiologic characteristics of ALL in the AYA population, the main r...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2014.052
更新日期:2014-07-02 00:00:00
abstract::In the last 15 years, highly active antiretroviral therapy (HAART) has determined a dramatic reduction of both morbidity and mortality in human immunodeficiency virus (HIV)-infected subjects, transforming this infection in a chronic and manageable disease. Patients surviving with HIV in the developed world, in larger ...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2010.034
更新日期:2010-01-01 00:00:00
abstract::In Primary Myelofibrosis (PMF), megakaryocyte dysplasia/hyperplasia determines the release of inflammatory cytokines that, in turn, stimulate stromal cells and induce bone marrow fibrosis. The pathogenic mechanism and the cells responsible for progression to bone marrow fibrosis in PMF are not completely understood. T...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2018.068
更新日期:2018-11-01 00:00:00
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journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2015.013
更新日期:2015-02-20 00:00:00
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journal_title:Mediterranean journal of hematology and infectious diseases
pub_type:
doi:10.4084/MJHID.2016.050
更新日期:2016-10-20 00:00:00
abstract:INTRODUCTION:Follicular lymphoma (FL) is an indolent lymphoproliferative disorder of B-cells with variable clinical behavior. It is the second most common subtype of Non-Hodgkin lymphoma in western countries but reported to have a lower incidence in Asia. MATERIALS AND METHODS:Cases of FL diagnosed in the Department o...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2016.060
更新日期:2016-11-01 00:00:00
abstract:Background:Although bronchoalveolar lavage (BAL) measurements of galactomannan antigen (GM) seems to be more sensitive than serum testing to detect invasive fungal infection (IFI), a consensus on the most appropriate diagnostic threshold of the BAL GM test is still unclear. Moreover, there is uncertainty as to whether ...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2019.065
更新日期:2019-11-01 00:00:00
abstract:INTRODUCTION:Sickle cell disease (SCD) is one of the leading causes of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the time challenging its man...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2017.037
更新日期:2017-06-20 00:00:00
abstract::We describe the case of a 17- year old female who developed fatal haemophagocytic syndrome (HPS) one month following acute infection caused by Epstein-Barr virus (EBV). Despite initiation of treatment and reduction of EBV load, laboratory signs of HPS as severe cytopenia, hypofibrinogenemia, hyperferritinemia and hype...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type:
doi:10.4084/MJHID.2012.008
更新日期:2012-01-01 00:00:00
abstract::MTB ranks as the first worldwide pathogen latently infecting one third of the population and the second leading cause of death from a single infectious agent, after the human immunodeficiency virus (HIV). The development of vigorous and apparently appropriate immune response upon infection with M. tuberculosis in huma...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2014.027
更新日期:2014-04-07 00:00:00
abstract::Therapy for hemophilia has evolved in the last 40 years from plasma-based concentrates to recombinant proteins and, more recently, to non-factor therapeutics. Along this same timeline, research in adeno-associated viral (AAV) based gene therapy vectors has provided the framework for early phase clinical trials initial...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2020.069
更新日期:2020-09-01 00:00:00
abstract::Kikuchi-Fujimoto Disease (KFD), also known as histiocytic necrotizing lymphadenitis [corrected], is a benign, self-limiting disease that manifests primarily as cervical lymphadenopathy but may include low-grade fever, headache, and fatigue. There is a higher incidence of KFD in women aged 20-35 years and in Asian popu...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type:
doi:10.4084/MJHID.2014.001
更新日期:2014-01-01 00:00:00
abstract::The introduction of newer cytotoxic monoclonal antibodies and chimeric antigen receptor modified T cells is opening a new age in the management of B-lineage adult acute lymphoblastic leukemia. This therapeutic change must be very positively acknowledged because of the limits of intensive chemotherapy programs and allo...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2015.001
更新日期:2015-01-01 00:00:00
abstract::The B-cell receptor (BCR) signaling inhibitors ibrutinib and idelalisib are revolutionizing the treatment of chronic lymphocytic leukemia (CLL) and other B-cell malignancies. These oral agents, both alone and in combination with other drugs, have shown remarkable clinical activity in relapsed or refractory CLL across ...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2016.011
更新日期:2016-02-10 00:00:00
abstract:BACKGROUND:Beta thalassemia is an inherited hemoglobin disorder resulting in a severe, chronic anemia requiring life-long blood transfusion that induces iron overload. Silymarin is a flavonoid complex isolated from Silybin marianum with a strong antioxidant activity, inducing an hepatoprotective action, and probably, a...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2013.065
更新日期:2013-11-04 00:00:00
abstract::Acquired aplastic anemia (aAA) is a severe and rare disease, characterized by hematopoietic bone marrow failure and peripheral cytopenia. The pathophysiology is immune mediated in most cases, activated T1 lymphocytes have been identified as effector cells. The disease can be successfully treated with combined immunosu...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2009.026
更新日期:2009-12-26 00:00:00
abstract::Tenofovir is widely used as first-line treatment of HIV infection, although its use is sometimes complicated by a reversible proximal renal tubulopathy.We report the case of a 45-year-old woman with chronic HIV infection and personality disorder, who after 12 months of tenofovir, complained of fatigue, diffuse bone pa...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type:
doi:10.4084/MJHID.2012.025
更新日期:2012-01-01 00:00:00
abstract:INTRODUCTION:IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia and hypoxia, chronic liver disease, iron overload and other associated endocrinopathies, e.g. growth hormone deficiency (GHD). Few data are available in the literature regarding adult TM patients and growth di...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2014.074
更新日期:2014-11-01 00:00:00
abstract:Background:Alpha Thalassemia (α-thal) is a heterogeneous group of hereditary alterations caused by deletions that affect alpha regulatory genes, and the 3.7Kb deletion is the most frequent worldwide. The prevalence ranges from 20% and 35% in Brazil, depending mainly on race, predominant in Afro-descendants. Purpose:Th...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2021.001
更新日期:2021-01-01 00:00:00
abstract::Patients infected with human immunodeficiency virus (HIV) are at increased risk for developing both non-Hodgkin's lymphoma (NHL) and Hodgkin's lymphoma (HL). Even if this risk has decreased for NHL after the introduction of combination antiretroviral therapy (cART), they remain the most common acquired immune deficien...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章,评审
doi:10.4084/MJHID.2019.004
更新日期:2019-01-01 00:00:00
abstract:Introduction:The aim of the study was to enumerate the clinical, hematological, and molecular spectrum of G6PD deficiency in malaria endemic regions of south west Odisha. Methods:Diagnosis of G6PD deficiency was made by using the Di-chloroindophenol Dye test in two south west districts (Kalahandi and Rayagada) of Odis...
journal_title:Mediterranean journal of hematology and infectious diseases
pub_type: 杂志文章
doi:10.4084/MJHID.2020.015
更新日期:2020-03-01 00:00:00