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Thromboses are the most important preventable risk factors for morbidity and mortality in myeloproliferative neoplasms (MPN). We here performed a retrospective cross sectional study of patients with a diagnosis of Philadelphia negative MPN and a prior history of thrombosis, analyzed from electronic charts. Among a cohort of 260 patients with MPNs (78PV, 102ET, 80 MF), forty four were found triple negative for JAK-2, calreticulin and MPL gene mutations. Sixty-nine (26.54%) patients (29F, 40M) had a personal past clinical history of arterial or venous thrombosis. Among patients with thrombosis, 13(18.8%) cases (11ET, 2MF) were triple negative (median age:60 years). Most events, in particular in triple negative patients, occurred within 18 months before overt MPN . At the time of thrombosis diagnosis, only a minority of patients showed blood tests abnormalities suggestive of MPN or indicating hematological consultation(Figure). Triple negative MPNs may represent a distinct entity with peculiar clinical course and risk factors.

Infradiaphragmatic Hodgkin's disease is rare, making up 5-12% of cases in clinical stages I and II; consequently, several questions concerning prognosis and treatment strategy remain to be answered. The aim of this study was to analyze the clinical and prognostic characteristics and outcome of this condition.A series of 282 patients with CS I-II Hodgkin's disease (HD) was investigated. In 31 patients the disease was confined below the diaphragm (BDHD), and in the remaining above the diaphragm (ADHD). The presenting features and outcomes were compared in the two groups.The BDHD group was older (p0.0002), had a higher frequency of males (p0.08) and a different histological subtype group distribution (p0.0001). Stage II BDHD patients had a worse overall survival rate (OS) than stage II ADHD patients (68.8% vs 86.6% at 8 years, p0.01) if age is not considered; patients with more than 40 years of age, in fact, had the same survival rates as those with ADHD. BDHD patients with intra-abdominal disease alone had worse prognostic factors and OS (p = 0.12) than patients with inguinal-femoral nodes.Although BDHD patients present distinct features, they have the same OS and relapse-free survival rate as age-adjusted ADHD patients. According to our experience patients with stage I peripheral BDHD respond well to radiotherapy-based regimens. Those with stage II and or intra-abdominal disease are more challenging; chemotherapy or a combined therapy seem to be more suitable approaches for these patients.

Background. Essential thrombocythemia (ET) and primary myelofibrosis (MF) are myeloproliferative neoplasms characterized by the overproduction of mature cells such as platelets (ET) or early bone marrow fibrosis due to scarring induced by highly proliferating myeloid progenitors and pathological stimulation of local fibroblasts (MF). Somatic mutations in CALR gene have recently identified in the majority of JAK2-V617F and MPL negative ET and MF patients. In this study we evaluated the frequency and type of CALR mutations and their clinical and hematological features. Methods. A total of 54 patients, 29 ET and 25 MF patient, was included in this study. All patients were JAK2 V617F and MPL negative. We registered clinical and hematological characteristics of patients i.e. age, hemoglobin level, white blood cell count, platelet count, International Prognostic Scoring System (IPSS), risk of thrombosis. Samples were collected from peripheral blood and DNA was extracted by using the QIAamp DNA mini kit (QIAGEN); CALR mutations were analyzed by direct sequencing method. Results. CALR mutations were present in 20.4 % of patients (4 ET; 7 MF). Four types of CALR mutations were detected; type 1 (p.L367fs*46) was isolated in 6 MF patient, type 2 (p.K385fs*47) was isolated in 3 ET patient; we also found 2 deletion mutations (p.E371fs*49 and D373fs*47), which are less common deletions, in the remaining patients. Patients carrying CALR mutations were younger (mediane age: 50 vs 65; p=0.2) than CALR negative patients. Furthermore, they did not show thrombosis and IPSS high risk. Conclusions. Our observations are in agreement with the findings of literature. We can assert an improved outcome of CALR mutated patients and we can also speculate a possible protective role of CARL mutations given the absence of thrombosis events and of IPSS high risk. However, the cohort of patients with myeloproliferative disease need to be implemented to draw final conclusion.

Twenty-four patients with immune thrombocytopenic purpura (ITP) were treated with high doses of intravenous immunoglobulin (Ig). Increase in platelets was observed in all the patients. The maximum value was reached between the seventh and the twelfth day of treatment. The mean number of platelets was 18 x 10(9)/l before the treatment and 150 x 10(9)/l after the treatment. The effect was transient; in fact, a fall in the platelets was observed after 18 days of treatment. Our study confirms that high doses Ig is an effective treatment of ITP, but the high cost and the temporary effect limit its use only in those cases in which other treatments are ineffective or contraindicated.