Repeated successful use of eltrombopag in chronic primary immune thrombocytopenia: description of an intriguing case.

Thrombopoietin receptor agonists (TPO-RAs) are used as effective alternative treatments in ITP patients unresponsive to first-/second-line therapies. TPO- RAs can also be used to normalize platelet count to safely perform invasive pro- cedures and chemotherapy, in case of malignancies. In few responsive patients, TPO-RAs can be suspended maintaining a sustained respons

Clinical characteristics and outcome of patients with autoimmune hemolytic anemia (AIHA) uniformly defined as primary by a diagnostic work-up

Primary autoimmune hemolytic anemia (P-AIHA) is a relatively uncommon and hetereogeneous disease characterized by the destruction of red blood cells due to anti-erythrocyte autoantibodies (AeAbs) in the absence of an associated disease [1–3]. Secondary AHIA is frequently associated with lymphoproliferative diseases (LD) in particular, chronic lymphocytic leukemia, aggressive or indolent lymphomas, autoimmune disorders, malignancies other than lymphoid, and infections [1,2,4]. On the hypothetical assumption that in a significant proportion of cases defined as P-AIHA the clinical heterogeneity could be due to an ignored associated disease, we retrospectively analyzed the clinical characteristics and outcome of patients with a diagnosis of P-AIHA based on a diagnostic work-up aimed at excluding or identifying an associated disease. ..

Management of elderly and unfit patients with chronic lymphocytic leukemia

Introduction: About 75% of patients with chronic lymphocytic leukemia (CLL) are more than 65 years at the time of diagnosis. Treatment of the elderly remains complicated due to multiple factors, such as comorbidities, decline in functional reserve and fitness. Since chronological age by itself cannot properly predict life expectancy and treatment tolerance, an accurate assessment of the fitness status is of crucial importance for an optimal treatment choice. Areas covered: This review will discuss the most relevant aspects concerning the issues experienced in the management of elderly/unfit patients with CLL. The most frequently observed age-related toxicities, fitness assessments, supportive care measures and treatment options for elderly patients and for patients who are deemed unfit will be discussed. Literature search methodology included examination of PubMed index. Expert commentary: During the last decade, different trials focusing on elderly/unfit patients have investigated more tolerable chemoimmunotherapy schedules and, more recently, the activity and safety of chemo-free regimens. Chlorambucil combined with an anti-CD20 monoclonal antibody has shown clinical activity with a relatively good profile of toxicity. The recent introduction of the B-cell receptor antagonists, ibrutinib and idelalisib, and other targeted drugs in development (e.g. venetoclax), is broadening the therapeutic armamentarium of elderly CLL patients

Kajian Perbedaan Konsentrasi Larutan Garam Pada Perendaman Rgh Dan Vaksin Terhadap Kelulushidupan Dan Pertumbuhan Benih Ikan Lele Sangkuriang (Clarias Gariepinus)

Berdasarkan data KKP (2013), pencapaian produksi ikan lele pada tahun 2013 mampu melampaui target. Ikan lele merupakan ikan yang mudah dibudidayakan, sehingga banyak dilakukan penelitian agar didapatkan benih lele dengan pertumbuhan dan kelulushidupan yang lebih baik, serta tahan terhadap serangan penyakit. Penelitian ini bertujuan untuk mengetahui pengaruh konsentrasi larutan garam yang berbeda dan konsentrasi yang terbaik pada perendaman rGH dan vaksin terhadap kelulushidupan dan pertumbuhan benih lele sangkuriang. Penelitian ini dilaksanakan pada tanggal 13 November 2014 – 15 Februari 2015 di Satuan Kerja Pembenihan dan Budidaya Ikan Air Tawar (SATKER PBIAT), Siwarak, Ungaran, Semarang. Ikan uji yang digunakan adalah benih lele sangkuriang umur 12 hari. Metode yang digunakan adalah eksperimental dengan rancangan acak lengkap (RAL) 4 perlakuan dan 3 ulangan, yaitu: (A) perlakuan tanpa larutan garam, (B) konsentrasi 0,5%, (C) konsentrasi 1,0% dan (D) konsentrasi 1,5%. Pemeliharaan ikan dilakukan selama 42 hari. Variabel data yang diamati meliputi kelulushidupan, SGR, panjang mutlak, FCR, EPP dan kelulushidupan setelah uji tantang. Analisa data dengan menggunakan anova untuk mengetahui pengaruh perlakuan yang berbeda nyata, apabila hasil yang didapatkan berbeda nyata, maka dilanjutkan dengan uji duncan untuk mengetahui perlakuan yang terbaik. Hasil penelitian menunjukkan perlakuan terbaik adalah perlakuan C, dengan nilai kelulushidupan (87,00±1,00%), SGR (7,79±0,03%), nilai panjang mutlak (6,75±0,15cm), nilai FCR (0,71±0,01) dan nilai EPP (140,28±1,25%), sedangkan untuk kelulushidupan setelah uji tantang didapatkan hasil yang tidak berbeda nyata, dimana: A (93,33±5,77%), B (96,67±5,77%), C (96,67±5,77%) dan D (96,67±5,77%). Kesimpulan dari penelitian ini ialah pemberian konsentrasi larutan garam yang berbeda berpengaruh sangat nyata terhadap kelulushidupan, SGR, panjang mutlak, FCR dan EPP, namun tidak berpengaruh nyata terhadap kelulushidupan setelah uji tantang. Konsentrasi larutan garam terbaik pada penelitian ini adalah 1,0%. Based on data from KKP (2013), the achievement of the production of catfish in 2013 was able of exceeding the target. Catfish is a fish that easily cultivated, so a lot of research done to get catfish\u27s seed with better survival and growth, and it can resistant to attack of deseases. This research was aimed to find out the effect of different salt solution concentrations and the best concentration from immersion of rGH and vaccine for survival rate and growth of sangkuriang catfish\u27s seed. This research was conducted on November 13th, 2014 – February 15th, 2015 at Satuan Kerja Pembenihan dan Budidaya Ikan Air Tawar (SATKER PBIAT), Siwarak, Ungaran, Semarang. The fish that used for this research is sangkuriang catfish\u27s seed aged 12 days. Completely randomized design (CRD) was used in this research with four treatments and three replication, which treatments are: (A) without salt solution concentration, (B) Using concentration 0.5%, (C) Concentration 1.0% and (D) Concentration 1.5%. The Fishes are maintained for 42 days. Observational variable are survival rate, SGR, absolute length, FCR, EPP and survival rate after challenge test. Data analysis using anova to know the effect of treatment is significantly different, if the result is significantly different, then continue with duncan test to know the best treatment. The best result is treatment C, with survival rate (87.00±1.00%), SGR (7.79±0.03%), absolute length (6.75±0.15cm), FCR (0.71±0.01), EPP (140.28±1.25%), and for survival rate after challenge test are not significantly different, where: treatment A (93.33±5.77%), B (96.67±5.77%), C (96.67±5.77%) and D (96.67±5.77%). The conclusion of this research is giving of salt solution with different concentration take significantly effect for survival rate, SGR, absolute length, FCR and EPP, but did not take significantly effect for survival rate after challenge test. The best salt solution concentration is 1.0%

Ciona intestinalis interleukin 17-like genes expression is upregulated by LPS challenge

In humans, IL-17 is a proinflammatory cytokine that plays a key role in the clearance of extracellular bacteria promoting cell infiltration and production of several cytokines and chemokines. Here, we report on three Ciona intestinalis IL-17 homologues (CiIL17-1, CiIL17-2, CiIL17-3). The gene organisation, phylogenetic tree and modelling supported the close relationship with the mammalian IL-17A and IL-17F suggesting that the C. intestinalis IL-17 genes share a common ancestor in the chordate lineages. Real time PCR analysis showed a prompt expression induced by LPS inoculation suggesting that they are involved in the first phase of inflammatory response. In situ hybridization assays disclosed that the genes transcription was upregulated in the pharynx, the main organ of the ascidian immune system, and expressed by hemocytes (granulocytes and univacuolar refractile granulocyte) inside the pharynx vessels

Prednisone vs high-dose dexamethasone in newly diagnosed adult primary immune thrombocytopenia: a randomized trial

A debate exists regarding which type of corticosteroids (standard-dose prednisone [PDN] or high-dose dexamethasone [HD-DXM]) is the best first-line treatment for adult patients with newly diagnosed untreated primary immune thrombocytopenia (pITP). An ad hoc study compared PDN with HD-DXM in newly diagnosed untreated patients with pITP (aged >= 18 but <= 80 years, platelet count of <= 20 or >20 but <50 x 10(9)/L, and bleeding score of >= 8). Patients were randomised to receive PDN 1 mg/kg per day from days 0 to 28 (Arm A) or HD-DXM 40 mg per day for 4 days, every 14 days, for 3 consecutive courses (Arm B). Fifty-nine of 113 patients (52.2%) were randomized to Arm A and 54 of 113 (47.8%) to Arm B. In evaluable patients, total initial responses (complete response [CR], partial response [PR], minimal response [MR]) were 44 of 56 (78.57%) in Arm A and 46 of 49 (93.88%) in Arm B at days 42 and 46, respectively (P = 0.0284). Total final responses (at day 180 from initial response) were 26 of 43 (60.47%) in Arm A and 23 of 39 (58.97%) in Arm B (P = 0.8907). Total persistent responses (at 12 months from initial response) were 25 of 31 (80.65%) in Arm A and 20 of 36 (55.56%) in Arm B (P = 0.0292). Seven relapses occurred. Median follow-up was 44.4 months. Overall survival was 100% at 48 months, overall disease-free survival was 81.11% at 48 months from day 180. PDN and pulsed HD-DXM were well tolerated; HD-DXM allows effective initial responses but less long lasting than PDN. This trial was registered at www.clinicaltrials.gov as #NCT00657410

Alterations in the α2 δ ligand, thrombospondin-1, in a rat model of spontaneous absence epilepsy and in patients with idiopathic/genetic generalized epilepsies

Objectives Thrombospondins, which are known to interact with the α2δ subunit of voltage-sensitive calcium channels to stimulate the formation of excitatory synapses, have recently been implicated in the process of epileptogenesis. No studies have been so far performed on thrombospondins in models of absence epilepsy. We examined whether expression of the gene encoding for thrombospondin-1 was altered in the brain of WAG/Rij rats, which model absence epilepsy in humans. In addition, we examined the frequency of genetic variants of THBS1 in a large cohort of children affected by idiopathic/genetic generalized epilepsies (IGE/GGEs). Methods We measured the transcripts of thrombospondin-1 and α2δ subunit, and protein levels of α2δ, Rab3A, and the vesicular glutamate transporter, VGLUT1, in the somatosensory cortex and ventrobasal thalamus of presymptomatic and symptomatic WAG/Rij rats and in two control strains by real-time polymerase chain reaction (PCR) and immunoblotting. We examined the genetic variants of THBS1 and CACNA2D1 in two independent cohorts of patients affected by IGE/GGE recruited through the Genetic Commission of the Italian League Against Epilepsy (LICE) and the EuroEPINOMICS-CoGIE Consortium. Results Thrombospondin-1 messenger RNA (mRNA) levels were largely reduced in the ventrobasal thalamus of both presymptomatic and symptomatic WAG/Rij rats, whereas levels in the somatosensory cortex were unchanged. VGLUT1 protein levels were also reduced in the ventrobasal thalamus of WAG/Rij rats. Genetic variants of THBS1 were significantly more frequent in patients affected by IGE/GGE than in nonepileptic controls, whereas the frequency of CACNA2D1 was unchanged. Significance These findings suggest that thrombospondin-1 may have a role in the pathogenesis of IGE/GGEs