Is JAK2V617F Mutation the Only Factor for Thrombosis in Philadelphia-Negative Chronic Myeloproliferative Neoplasms?

The most common genetic disorder in Philadelphia negative chronic myeloproliferative neoplasms is the JAK2-V617F mutation. In the present study, we aimed to determine risk factors for thrombosis in patients with essential thrombocytosis and polycythemia vera. We screened the medical records of 101 patients. Risk factors which may predict thrombosis were recorded. Venous thrombosis (VT) before diagnosis was significantly higher in JAK2 positive patients. VT after diagnosis was similar in JAK2 positive and negative groups, and was significantly higher in elderly patients. Treatment places importance on the JAK2 mutation under unmodifiable cardiovascular risk factors such as advanced age after diagnosis.

Decreased interleukin-20 level in patients with systemic sclerosis: are they related with angiogenesis?

In this study, we aimed to evaluate the relation between angiogenesis indicators and T helper 17 cytokine group in patients with systemic sclerosis (SSc) which is a disease characterized by impaired angiogenesis and autoimmune response. In our study, patients with SSc are compared with patients with primary Raynaud's phenomenon (RP) and healthy controls. Forty SSc patients, 18 primary RP cases, and 20 healthy controls were included in our study. The demographic and clinical features of patients with SSc were recorded. The serum levels of vascular endothelial growth factor (VEGF), vascular endothelial (VE)-cadherin, interleukin (IL)-20, IL-22, and IL-23 were assessed. In the SSc group, IL-20 level was significantly lower than in both primary RP group and controls (p values <0.001). VE-cadherin level in SSc was significantly higher than in primary RP (p = 0.016). The IL-22 and IL-23 and VEGF levels of SSc, primary RP, and control groups were similar (p values >0.05). In SSc patients, IL-23 correlated negatively with VEGF (r = -0.36, p = 0.025) and positively with VE-cadherin (r = 0.55, p < 0.001). IL-20 levels in SSc patients correlated with disease duration (r = 0.32, p = 0.044). SSc patients with limited involvement had significantly higher VE-cadherin levels than SSc patients with diffuse involvement (p = 0.044). We observed that IL-20 which is an IL-10 group angiogenesis indicator was observed to be suppressed in SSc, suggesting abnormal angiogenesis.

Secondary amyloidosis in ankylosing spondylitis.

We evaluated the frequency of secondary amyloidosis, associated clinical features, and outcomes in ankylosing spondylitis (AS) patients diagnosed in the last decade. The medical records of AS patients diagnosed at single academic medical center were reviewed for clinical evidence of amyloidosis. During routine follow-up, routine urinalysis was performed at each visit; patients with significant proteinuria underwent rectal biopsy. We diagnosed 8 clinically apparent amyloidosis patients (1.1 %) in our cohort of 730 AS patients (508 males, 222 females). Four patients undergoing hemodialysis were diagnosed secondary amyloidosis. Three patients had nephrotic syndrome and renal dysfunction and one patient had non-nephrotic proteinuria. When AS patients with amyloidosis were compared to AS controls, it was observed that the amyloidosis group was older, had longer disease duration, higher initial BASDAI scores, and ESR values, and more frequent peripheral arthritis (p < 0.05). Logistic regression analysis revealed that the initial BASDAI level was an independent predictor for the development of secondary amyloidosis (OR:2.36). Six patients were administered anti-TNF therapy. The clinical findings resolved in these. In 2 patients with nephrotic syndrome and renal dysfunction, in addition to clinical improvement, there was a decrement in proteinuria; renal function improved or remained stable. Anti-TNF therapy is safe and effective in patients with renal failure, and at an earlier stage, appears effective in improving renal function. The development of proteinuria in AS patients should occasion a search for underlying amyloidosis.