1,720,964 research outputs found
Safety and Efficacy of Rituximab and Cyclophosphamide in a Case of Resistant Acquired Hemophilia A in Course of Chronic Lymphocytic Leukemia
No full textAcquired Hemophilia A (AHA) is a rare disease caused by anti-factor VIII autoantibodies. It is usually characterized by clinically significant bleeding at the onset and requires prompt hemostatic and immunosuppressive therapies. Due to its rarity and the lack of randomized trials, its treatment is a great challenge, especially in the relapse/refractory setting. This report presents the case of a patient diagnosed with chronic lymphocytic leukemia who developed a steroid-resistant AHA, successfully managed with aggressive immunosuppressive therapy
UN RARO CASO DI EMOFILIA A ACQUISITA NEL POST-PARTUM
No full textL'emofilia A acquisita è un raro disordine caratterizzato dalla presenza di autoanticorpi diretti contro il fattore VIII circolante. Condizioni sottostanti possono essere evidenziate in circa il 50% dei pazienti (malattie autoimmuni, neoplasie solide, malattie linfoproliferative, gravidanza). Nel periodo post-partum, l'emofilia acquisita si manifesta generalmente tra il primo e il quarto mese dopo il parto (Huth-Kühne et al., Haematol. 2009;94:566-575).
Descriviamo il raro caso di una donna di 34 anni giunta all'osservazione dell’U.O. di Ematologia dell’AOU Pisana circa tre mesi dopo il parto per comparsa di ematomi spontanei agli arti e ginocchio edematoso in assenza di emartri.
Gli esami di laboratorio hanno evidenziato anemia (Hb 10.8 g/dL, Val.Rif.=11.5-15.5 g/dL), con piastrine nella norma (218.000/μL), normale tempo di protrombina (PT ratio=1.0), prolungamento del tempo di tromboplastina parziale attivata (aPTT ratio=3.36, VR=0.85-1.2) con ridotta attività del fattore VIII (0.6 %, VR=50-150%). Nonostante una marcata correzione dell'aPTT dopo mixing con pool di plasmi normali (aPTT ratio=1.09), la ricerca dell'anticorpo specifico contro il fattore VIII, secondo metodo Bethesda modificato, ha restituito un valore di 8,16 UB/mL (VR=assente).
La paziente ha pertanto iniziato terapia steroidea mostrando, nei successivi controlli, costante riduzione dei livelli dell'inibitore fino al valore di 0,88 UB/mL per poi risalire dopo circa due mesi al titolo di 3,8 UB/mL. Vista la scarsa efficacia del trattamento immunosoppressivo di prima linea, la paziente ha iniziato terapia con Rituximab (anticorpo monoclonale anti-CD20) e sospeso gradualmente la terapia steroidea.
Dopo tre settimane di trattamento con Rituximab la paziente ha mostrato normalizzazione dell'aPTT e dei livelli di attività del fattore VIII (102%) con scomparsa dell’anticorpo inibente il fattore VIII. Nei controlli successivi fino ad oggi la paziente ha sempre mostrato normale profilo emocoagulativo.
Nonostante la letteratura riporti una scarsa correlazione tra l’entità delle manifestazioni cliniche ed il livello dell’inibitore, questo caso conferma che la valutazione dei livelli dell’inibitore può essere di aiuto nel valutare l’efficacia della terapia immunosoppressiva in questa tipologia di pazienti
Interference of Monoclonal Gammopathy with Fibrinogen Assay Producing Spurious Dysfibrinogenemia
Full text linkAbnormal coagulation properties indicative of a dysfibrinogenemia were found in the plasma of an asymptomatic 65-year-old male. An immunoglobulin k light chain was found to interfere with Fg functional assay and coagulation tests (activated partial thromboplastin time, prothrombin time, and thrombin time). Steroid therapy reduced the inhibitory effect (after dexamethasone treatment coagulation test and functional Fg value normalized). Spurious dysfibrinogenemia associated with light chain monoclonal gammopathy of undetermined significance was diagnosed
Acute myeloid leukaemia after treatment with 90Y-ibritumomab tiuxetan for follicular lymphoma
No full textWe report here a patient with relapsed follicular lymphoma who developed secondary acute myeloid leukaemia 15 months after radioimmunotherapy (RIT) with 90Y-ibritumomab tiuxetan, the fifth described case to date. We review the literature for the potential causal relationship between RIT and secondary myelodysplastic syndrome/acute myeloid leukaemia. Copyright © 2008 John Wiley & Sons, Ltd
How to treat splenic marginal zone lymphoma (SMZL) in patients unfit for surgery or more aggressive therapies: experience in 30 cases
No full textSplenic marginal zone lymphoma (SMZL) is an indolent disease that typically affects elderly patients. Thanks to its outcome, most patients don’t need any specific therapy and ‘a watch and wait’ policy is frequently employed. Treatment is required in symptomatic cases. Splenectomy remains one of the first line options in patients fit for surgery. The best pharmacological strategy has not yet been identified for poor surgical risk cases. Amongst different possible chemotherapeutic approaches, alkylating agents, alone or in association with Rituximab, could employ in ‘frail’ patients. In the present study, the role of oral cyclophosphamide (100 mg per day for 15 consecutive days, every 30 for a total of six cycles) associated with anti-CD20 monoclonal antibody has been evaluated in 30 newly diagnosed SMZL patients, not fit for splenectomy or more toxic chemotherapic regimens. Overall response rate was 87% (CR 70%; PR 17%). Median PFS was 20 months (range, 1–53), with better outcome for low-risk cases according to IIL score prognostic index. Toxicity profile resulted mild
Disfibrinogenemia indotta da una catena leggera libera kappa delle immunoglobuline
No full textAbstract
Despite several pathological conditions are associated with free light chains (FLC) deposition in human tissues, only few cases of human diseases caused by the specific binding activity of monoclonal FLC are described. A 65-year old male patient, with highly abnormal functional coagulation tests and undetectable functional fibrinogen was admitted to the Hematological Clinic of the University Hospital of Pisa. The same tests were within the reference intervals one year before. After excluding a number of causes for abnormal coagulation tests, we focused on potential causes of acquired dysfibrinogenemia. Due to the presence of abnormal values of FLC, we performed an immunofixation: while serum did not show any detectable monoclonal band, the immunofixation of a plasma sample revealed the presence of monoclonal FLC of kappa type co-migrating with fibrinogen. The serum kappa FLC concentrations were much lower than plasma levels, suggesting that the majority of these FLC were bound to fibrinogen, remaining associated to fibrin after clotting. Bone marrow biopsy showed 4% monoclonal plasma cells producing kappa light chains. The patient was diagnosed as affected by a FLC MGUS. After two courses of dexamethasone, the plasma concentration of kappa FLC decreased substantially and most of the coagulation tests normalized. The nature of the interaction between fibrinogen and kappa FLC is currently under investigation to elucidate the mechanism able to inhibit fibrinogen polymerization
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
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