The T-TAS appears to be sensitive mainly to plasma VWF concentration and the presence of large multimers. Failure of the PL and AR chips to become occluded points to a lack of large VWF multimers, or type 1 VWD with VWF levels below 10 U dL . Although the T-TAS does not assure a precise VWD diagnosis, it does point us in the right direction, and thus seems a useful global preliminary test.

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Introduction Severe plasminogen (PLG) deficiency causes ligneous conjunctivitis, a rare disease characterized by the growth of fibrin‐rich pseudomembranes on mucosal surfaces; gums involvement leads to ligneous gingivitis (LG). Specific therapy for LG is not available yet. We report a prophylactic treatment with enoxaparin and fresh frozen plasma (FFP) for invasive dental procedures in a patient with LG, and a review of literature on LG treatment. Methods A 43‐year‐old female with LG was studied. In order to prevent LG recurrence after dental care, FFP before and the day after the procedure, and enoxaparin were administered in addition to proper minimally invasive dentistry techniques and implant surgery. Results Plasminogen deficiency was confirmed by reduced PLG antigen (25 μg/mL) and activity (20%) levels, and genetic analysis. PLG levels rose to 46% after FFP transfusion and returned to baseline after 48 hours. Minimally invasive dental procedures and implants were performed. Small gingival pseudomembranes developed soon thereafter in some cases but disappeared within a few weeks; no bleeding complications were observed. Conclusions In our patient with LG, the adoption of combined haematological and dentistry protocols appeared to be safe and effective in preventing abnormal gingival pseudomembranes growth after dental interventions, maintaining a healthy periodontal condition.

With more recent modalities of immunosuppression, splenectomy is now rarely considered in refractory/relapsed thrombotic thrombocytopenic purpura (TTP). However, the surgical approach had shown convincing evidences of high efficacy in the pre-rituximab era and therefore may still represent a lifesaving option in selected challenging cases. To define the characteristics of subjects who may benefit from splenectomy may ease clinical decision making. In this paper we describe the clinical and laboratory data of 2 multiple relapsing TTP cases who successfully underwent splenectomy in the pre-rituximab era. Whereas high anti-ADAMTS13 antibody titre and low ADAMTS13 activity never correlated with remission and relapse, a drop in the ADAMTS13 antigen level was always associated with the acute phase, whereas levels consistently returned to normal following splenectomy, heralding long term remission. Splenectomy may therefore be considered in refractory TTP cases associated with increased ADAMTS13 antigen clearance, irrespective of persistence of inhibitory antibodies.

ARTICLES 147 HemostasisThe impaired capacity of von Willebrand factor to carry factor VIII is identified as type 2N von Willebrand's disease. R854Q is the most common type 2N mutation, and almost the only one identified in Italy. This aim of this study was to ascertain whether R854Q mutations in a cohort of Italian patients with type 2N von Willebrand's disease originated from a single event or recurrent events. Thirteen unrelated Italian families were investigated, analyzing the von Willebrand factor gene haplotype associated with the R854Q mutation. A common haplotype emerged in all the families, extending from single nucleotide polymorphisms rs2166902 to rs216293 over 48.2 kb and including five intragenic markers. This haplotype is infrequent in the healthy Italian population (17% versus 100%, P<0.0001) and each genetic marker within the said haplotype is similarly rare. These data strongly suggest a founder effect, with a single R854Q mutation event being the cause of the type 2N von Willebrand's disease in our cohort of patients. Using DMLE+ software and the mathematical model of Bengtsson and Thomson, it was estimated that the R854Q mutation occurred from 10,000 to 40,000 years ago, which is consistent with the short dimension of the haplotype shared by our patients. Together with the fact that the R854Q mutation seems to be limited to Caucasian populations, these findings suggest that a single mutational event took place after human populations moved from Africa towards Europe. -access paper. doi:10.3324/haematol.2012.066019 A common ancestor more than 10,000 years old for patients with R854Q-related type 2N von Willebrand's disease in Italy ©2013 Ferrata Storti Foundation. This is an open ABSTRACT Manuscript

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a 29-yearold Moldovan woman was referred to our center with a platelet count of 4 × 10 9 /L and bleeding symptoms such as epistaxis and petechiae. She had been diagnosed with immunethrombocytopenia (ITP) in 2002. Ever since her childhood, her thrombocytopenia had been associated with hemolytic anemia (Evans syndrome) requiring blood transfusions, and complicated by HCV-related hepatitis (anti-HCV antibodies, HCV-RNA 1257491 IU/ml, genotype 1b). Previous therapy for ITP, based on prednisone, intravenous immunoglobulin (IVIg) and azathioprine had been ineffective. In 2006, she underwent splenectomy, and achieved a stable complete remission (CR), maintained with a low daily dose of prednisone (2.5 mg).She also suffered from Sjögren's syndrome, with severe dry eyes, positive ANA and ENA anti-SSA titers, and monoclonal gammopathy of undetermined significance IgGK without Bence-Jones proteinuria. Definitive diagnosisDr. Vianello: We performed a bone marrow examination, which was normal; platelet-associated autoantibodies against GpIIb/IIIa and GpIb were identified. An abdominal CT scan performed during hospitalization at our center was negative for accessory spleen. A definitive diagnosis of secondary and refractory ITP was established.