Mediterranean Journal of Hematology and Infectious Diseases

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  • ISSN
    2035-3006

Publications in this journal

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    ABSTRACT: Imatinib was the first signal transduction inhibitor (STI), used in a clinical setting. It prevents a BCR-ABL protein from exerting its role in the oncogenic pathway in chronic myeloid leukemia (CML). Imatinib directly inhibits the constitutive tyrosine kinase activity. Imatinib binds to BCR-ABL kinase domain by preventing the transfer of a phosphate group to tyrosine on the protein substrate and the subsequent activation of phosphorylated protein. As the result, the transmission of proliferative signals to the nucleus is blocked and leukemic cell apoptosis is induced. The FDA has approved imatinib as first-line treatment for newly diagnosed CML in December 2002 following an International Randomized Study (IRIS), initiated in June 2000, comparing imatinib at a single daily dose 400 mg to IFN alpha plus cytarabine in newly diagnosed patients with CML in CP. Results from this study show the outstanding effectiveness of imatinib and its superiority with respect to the rates of complete hematological response (CHR), major and complete cytogenetic response (MCyR, CCyR). Patients randomized to imatinib arm at 8 - year data cut off continue to have a durable hematologic and cytogenetic responses, low progression rates to AP or BC, and remarkable survival outcomes. An overall survival (OS) rate is 85% for patients receiving imatinib (93% when only CML-related deaths and those prior to stem cell transplantation are considered). The results have been confirmed in the last years by several groups. According these cumulative results the rates of CCyR achieved after one year of therapy with imatinib at standard dose ranged from 49% to 77%, and the proportion of patients who achieved major molecular response (MMR) after one year ranged between 18% and 58%. Discontinuation of imatinib has been also tried in patients in MMR, a molecular relapse occurs in about one third of patients, generally within 6 months from imatinib cessation.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014007.
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    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014010.
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    ABSTRACT: Bone marrow examination is an indispensable diagnostic tool to evaluate neoplastic and non neoplastic hematological diseases. To compare bone marrow aspirate with trephine biopsy in hematological disorders. To determine the optimum trephine preprocessing length in lymphoma infiltration. Diagnostic comparison was done between simultaneous bone marrow aspirates and trephine biopsies in 449 patients. Biopsies were fixed in formalin, decalcified in 5.5% EDTA and routinely processed. Concordance rates and validity parameters for aspirate were calculated. Three deeper sections of trephine biopsy, cut at 0.1-0.2 mm intervals, were assessed for lymphoma involvement. Proportion of biopsies showing marrow infiltration by lymphoma cells was plotted against trephine length and correlation was assessed. Aspirate had a high sensitivity for acute leukemia (89.4%) and multiple myeloma (88.5%), moderate for NHL (67.6%) and nonhematopoietic metastases (58.3%) and low for aplastic anemia (38.5%) and Hodgkin lymphoma (5%). Aspirate has no role in granulomatous myelitis and myelofibrosis. Lymphoma positivity increased with trephine length, with maximum positivity (68.9%) seen in 17-20 mm group and no further gain beyond 20 mm. (lymphoma positivity ≤16mm=40.3% and ≥17mm=66.1%, p=0.0011). Aspirate has a high specificity; its sensitivity depends upon the type of disease. Apart from few conditions, in which aspirate alone is sufficient, biopsy is mandatory in most. Preprocessing trephine length of 17-20 mm examined at multiple deeper levels was found optimal for assessing lymphoma positivity.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014002.
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    ABSTRACT: The use of interferon-a (INF) in chronic myeloid leukemia, when it started in the 80s, was considered as a breakthrough in the therapy of this disease; INF administered alone or in combination with aracytin was the standard choice for treatment for Chronic Myeloid Leukemia (CML) patients unfit for bone marrow transplantation. With the appearance of the first Tyrosine Kinase Inhibitor (TKI) (imatinib) and based on the results of the pivotal IRIS trial, imatinib monotherapy was the new treatment of choice for CML, according to the ELN recommendations. The possibility of combining INF with imatinib, for obtaining better therapeutic responses in CML patients has been already tested and reported. The current challenge is the combined use of second generation TKIs with pegylated -IFN, in order to minimize failures to therapy and increase the number of CML patients with deep molecular responses, who may be able to discontinue lifelong treatment.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014006.
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    ABSTRACT: The first treatment of chronic myeloid leukemia (CML) included spleen x-radiation and conventional drugs, mainly Busulfan and Hydroxyurea. This therapy improved the quality of life during the chronic phase of the disease, without preventing nor significantly delaying the progression towards advanced phases. The introduction of allogeneic stem cell transplantation (alloSCT) marked the first important breakthrough in the evolution of CML treatment, because about 50% of the eligible patients were cured. The second breakthrough was the introduction of human recombinant interferon-alfa, able to achieve a complete cytogenetic remission in 15% to 30% of patients, with a significant survival advantage over conventional chemotherapy. At the end of the last century, about 15 years ago, all these treatments were quickly replaced by a class of small molecules targeting the tyrosine kinases (TK), which were able to induce a major molecular remission in most of the patients, without remarkable side effects, and a very prolonged life-span. The first approved TK inhibitor (TKI) was Imatinib Mesylate (Glivec or Gleevec, Novartis). Rapidly, other TKIs were developed tested and commercialized, namely Dasatinib (Sprycel, Bristol-Myers Squibb), Nilotinib (Tasigna, Novartis), Bosutinib (Busulif, Pfizer) and Ponatinib (Iclusig, Ariad). Not all these compounds are available worldwide; some of them are approved only for second line treatment, and the high prices are a problem that can limit their use. A frequent update of treatment recommendations is necessary. The current treatment goals include not only the prevention of the transformation to the advanced phases and the prolongation of survival, but also a length of survival and of a quality of life comparable to that of non-leukemic individuals. In some patient the next ambitious step is to move towards a treatment-free remission. The CML therapy, the role of alloSCT and the promising experimental strategies are reviewed in the context of the new therapeutic goals.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014005.
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    ABSTRACT: With the advent of target therapies, imatinib became the mainstay for treatment of chronic myeloid leukemia. However, despite the brilliant results obtained with this drug, more than 30% of patients discontinue therapy in long-term due to several reasons, including failure and/or intolerance. Second-generation tyrosine kinase inhibitors (TKIs) are more potent drugs and have expanded inhibition against a broad spectrum of mutations resistant to imatinib. Both nilotinib and dasatinib have demonstrated in vitro and in vivo clinical activity against different types of mutations and various forms of resistance. However, patients with T315I mutation do not obtain an advantage from these drugs and a third generation inhibitor ponatinib, a pan-BCR drug, was tested with significant results. In this review, we report the results of second-and third-generation TKIs tested as second or third line therapy in patients resistant and/or intolerant to previous inhibitors.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014003.
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    ABSTRACT: Tuberculosis (TB) is widespread in Africa, but weak health systems in developing countries, often display poor quality of care with delays in case identification, irrational therapy and drug shortage, clinical mismanagement, unnecessary expenditures for patients, reduced adherence and increased mortality. Public-private partnership has demonstrated to increase TB case detection, but less is known about its effects on quality of care, mortality and costs for hospitalized TB patients.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014049.
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    ABSTRACT: Classic Hodgkin Lymphoma (cHL) has a unique histology since only a few neoplastic cells are surrounded by inflammatory accessory cells that in the last years have emerged as crucial players in sustaining the course of disease. In addition, recent studies suggest that the abnormal activity of these inflammatory cells (such as deregulation in regulatory T cells signaling, expansion of myeloid derived suppressor cells, HLA-G signaling and natural killer cells dysfunction) may have prognostic significance. This review is focused on summarizing recent advanced in immunological defects in cHL with translational implications.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014039.
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    ABSTRACT: Kikuchi-Fujimoto Disease (KFD), also known as histiocytic lymphadenitis, 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 populations. A PubMed search revealed 590 articles that described KFD. Of these, 22 cases have been fully described in the United States. Ten of the 22 (45%) patients were male and 12 (55%) were female, with 20% Caucasian, 20% Asian American, and the remaining 60% of other ethnic backgrounds. In this study, we describe an additional 3 cases of KFD and discuss the diagnosis, pathology, and management of KFD.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014001.
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    ABSTRACT: β Thalassemia major is characterized by hemolytic anemia, ineffective erythropoiesis and hemosiderosis. About 4% of the world population carries a Thalassemia gene. Management includes blood transfusions and iron chelation. However, this treatment is costly, and population screening may be significantly more cost beneficial. The purpose of the current study is to analyze the cost of running a prevention program for β Thalassemia in Israel and to compare it to the actual expenses incurred by treating Thalassemia patients. THREE COST PARAMETERS WERE ANALYZED AND COMPARED: the prevention program, routine treatment of patients and treatment of complications. An estimation of the expenses needed to treat patients who present with complications was calculated based on our ongoing experience in treating deteriorating patients. The cost of preventing one affected newborn was $63,660 compared to $1,971,380 for treatment of a patient during 50 years (mean annual cost: $39,427). Thus, the prevention of 45 affected newborns over a ten year period represents a net saving of $88.5 million to the health budget. Even after deducting the cost of the prevention program ($413.795/year), the program still represents a benefit of $76 million over ten years. Each prevented case could pay the screening and prevention program for 4.6 years.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014012.
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    ABSTRACT: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment for sickle cell anemia (SCA). We report our experience with transplantation in children with the Black African variant of SCA and the effects of transplant on erythroid compartment in bone marrow (BM).
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014054.

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