- Successful therapy of Pyoderma gangrenosum with a JAK2 inhibitor. [Letter]
- BJBr J Dermatol 2018 Feb 16
- Pyoderma gangrenosum (PG) is a rare, solitary or multiple, chronically progressive, painful, destructive, sterile neutrophil inflammation of unexplained aetiology and pathogenesis, which often remain...
Pyoderma gangrenosum (PG) is a rare, solitary or multiple, chronically progressive, painful, destructive, sterile neutrophil inflammation of unexplained aetiology and pathogenesis, which often remains resistant to treatment. An autoimmunological and autoinflammatory genesis is discussed, which is the main reason why there are so many immunosupressant therapies available. PG may occur in association with inflammatory and haematological disorders, such as Crohn's disease, myeloma, leukaemia, lymphoma and polycythemia vera (PV), as it is the case in our patient presented here. This article is protected by copyright. All rights reserved.
- The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum. [Journal Article]
- JDJAMA Dermatol 2018 Feb 14
- CONCLUSIONS: Although clinical presentation in this large cohort was similar between different age groups, disease associations varied by age. The findings of this study may allow for a more focused, age-specific evaluation of patients with pyoderma gangrenosum.
- A patient case highlighting the myriad of cutaneous adverse effects of prolonged use of hydroxyurea. [Journal Article]
- DODermatol Online J 2017 Nov 15; 23(11)
- CONCLUSIONS: We report a case that exemplifies the cutaneous adverse effects of chronic hydroxyurea therapy. Although many cases improve after drug discontinuation, strict photoprotection and ongoing surveillance are indicated given the recently proposed premalignant potential of dermatomyositis-like eruptions and the aggressive nature of hydroxyurea-induced nonmelanoma skin cancer.
- Injection Testosterone and Adverse Cardiovascular Events: A Case-crossover Analysis. [Journal Article]
- CEClin Endocrinol (Oxf) 2018 Feb 15
- CONCLUSIONS: Testosterone injections were uniquely associated with short-term risk of acute cardio- and cerebrovascular events in older adult men following injection receipt. This article is protected by copyright. All rights reserved.
- Aquagenic Pruritus in Polycythemia Vera: Clinical Characteristics. [Journal Article]
- ADActa Derm Venereol 2018 Feb 13
- Aquagenic pruritus is one of the main clinical features of polycythemia vera. The aim of this study was to analyse the clinical characteristics of aquagenic pruritus. The study group comprised 102 pa...
Aquagenic pruritus is one of the main clinical features of polycythemia vera. The aim of this study was to analyse the clinical characteristics of aquagenic pruritus. The study group comprised 102 patients with molecularly confirmed polycythemia vera. Demographic data, data on disease history, polycythemia vera status and treatment modalities were collected. Moreover, various clinical features of aquagenic pruritus (including intensity, localization, quality, descriptors) and the most common factors responsible for its aggravation or alleviation were examined. Aquagenic pruritus was observed in 41.2% of individuals, mean duration 6.6 ± 8.6 years, and its onset was noticed in the majority of patients (52.4%) before the diagnosis of polycythemia vera. The mean intensity of aquagenic pruritus was 4.8 ± 1.9 points (visual analogue scale). One-third of patients with aquagenic pruritus avoided any contact with water. Antipruritic treatment was received only by 3 patients. Aquagenic pruritus seems to be an important, but frequently neglected, symptom in patients with polycythemia vera.
- Thromboembolism prophylaxis in patients with Philadelphia-negative myeloproliferative neoplasms - clinical practice among Nordic specialists. [Journal Article]
- EJEur J Haematol 2018 Feb 10
- CONCLUSIONS: The results of this study highlight the importance of considering all risk factors for thrombosis and an optimal collaboration with the primary health care sector. This article is protected by copyright. All rights reserved.
- Distinguishing myelofibrosis from polycythemia vera and essential thrombocythemia: The utility of enumerating circulating stem cells with aberrant hMICL expression by flow cytometry. [Journal Article]
- IJInt J Lab Hematol 2018 Feb 10
- CONCLUSIONS: Enumeration of circulating hMICL+ stem cells by FCM can discriminate between MPN phenotypes and holds potential for monitoring disease evolution.
- The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. [Review]
- BCBlood Cancer J 2018 Feb 09; 8(2):15
- The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloprol...
The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloproliferative neoplasms (MPNs), the revised document includes seven subcategories: chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia-not otherwise specified and MPN, unclassifiable (MPN-U); of note, mastocytosis is no longer classified under the MPN category. In the current review, we focus on the diagnostic criteria for JAK2/CALR/MPL mutation-related MPNs: PV, ET, and PMF. In this regard, the 2016 changes were aimed at facilitating the distinction between masked PV and JAK2-mutated ET and between prefibrotic/early and overtly fibrotic PMF. In the current communication, we (i) provide practically useful resource tables and graphs on the new diagnostic criteria including outcome, (ii) elaborate on the rationale for the 2016 changes, (iii) discuss the complementary role of mutation screening, (iv) address ongoing controversies and propose solutions, (v) attend to the challenges of applying WHO criteria in routine clinical practice, and (vi) outline future directions from the perspectives of the clinical pathologist.
- The 2014 BCSH criteria and the 2016 WHO criteria for essential thrombocythemia: a comparison in a large-scale cohort. [Journal Article]
- EJEur J Haematol 2018 Feb 05
- CONCLUSIONS: The BCSH criteria diagnosed ET in a broader range of patients encompassing a significant number of patients who would otherwise be diagnosed as prePMF or MPN-u. This article is protected by copyright. All rights reserved.
New Search Next
- JAK inhibitors for the treatment of myeloproliferative neoplasms and other disorders. [Review]
- FF1000Res 2018; 7:82
- JAK inhibitors have been developed following the discovery of the JAK2V617F in 2005 as the driver mutation of the majority of non- BCR-ABL1 myeloproliferative neoplasms (MPNs). Subsequently, the sear...
JAK inhibitors have been developed following the discovery of the JAK2V617F in 2005 as the driver mutation of the majority of non- BCR-ABL1 myeloproliferative neoplasms (MPNs). Subsequently, the search for JAK2 inhibitors continued with the discovery that the other driver mutations ( CALR and MPL) also exhibited persistent JAK2 activation. Several type I ATP-competitive JAK inhibitors with different specificities were assessed in clinical trials and exhibited minimal hematologic toxicity. Interestingly, these JAK inhibitors display potent anti-inflammatory activity. Thus, JAK inhibitors targeting preferentially JAK1 and JAK3 have been developed to treat inflammation, autoimmune diseases, and graft-versus-host disease. Ten years after the beginning of clinical trials, only two drugs have been approved by the US Food and Drug Administration: one JAK2/JAK1 inhibitor (ruxolitinib) in intermediate-2 and high-risk myelofibrosis and hydroxyurea-resistant or -intolerant polycythemia vera and one JAK1/JAK3 inhibitor (tofacitinib) in methotrexate-resistant rheumatoid arthritis. The non-approved compounds exhibited many off-target effects leading to neurological and gastrointestinal toxicities, as seen in clinical trials for MPNs. Ruxolitinib is a well-tolerated drug with mostly anti-inflammatory properties. Despite a weak effect on the cause of the disease itself in MPNs, it improves the clinical state of patients and increases survival in myelofibrosis. This limited effect is related to the fact that ruxolitinib, like the other type I JAK2 inhibitors, inhibits equally mutated and wild-type JAK2 (JAK2WT) and also the JAK2 oncogenic activation. Thus, other approaches need to be developed and could be based on either (1) the development of new inhibitors specifically targeting JAK2V617F or (2) the combination of the actual JAK2 inhibitors with other therapies, in particular with molecules targeting pathways downstream of JAK2 activation or the stability of JAK2 molecule. In contrast, the strong anti-inflammatory effects of the JAK inhibitors appear as a very promising therapeutic approach for many inflammatory and auto-immune diseases.