Background Although patients with familial Mediterranean fever (FMF) are symptom-free between the attacks, subclinical inflammation may continue. Vitamin D (vit D) plays an important role in the modulation of immune system and might also influence the course of FMF. Endothelial dysfunction is previously demonstrated in Behcet's disease (BD) and vit D is implicated to modulate endothelial functions. Objectives We aimed to evaluate the status of serum 25(OH)Vit D3 levels and its association with disease activity, endothelial function and carotis intima media thickness (CIMT) in patients with FMF. Methods Sixty-two FMF (F/M: 42/20, mean age: 33.5 years), 43 ankylosing spondylitis (AS) (F/M: 19/24, mean age: 40.9 years) patients and 46 healthy controls (HC) (F/M: 26/20, mean age: 34.7 years) were studied. Serum 25(OH)Vit D3 levels were measured by HPLC method. Endothelial function was evaluated by brachial artery flow mediated dilatation (FMD) and CIMT with B-Mode ultrasound. The vitamin D-deficient BD patients received 1000 IU Vitamin D3 daily for 3 months. At the end of the third month of treatment, the subjects were re-evaluated for the same parameters (CIMT and FMD). Results The mean (SD) disease duration was 8.2 (7.8) vs 8.4 (6.5) years (p>0.05) in FMF and AS patients, respectively. All FMF patients were on attack-free period and all of them were on colchicine treatment. A significant difference was observed between the levels of 25(OH)Vit D3 between the groups [median (range): 11.3 (2.7-48.3), 12.7 (4-37) and 56 (14-125), FMF, AS and HC, respectively), p=0,001]. CIMT and FMD measurements were similar between groups (p>0.05). Serum 25(OH)Vit D3 levels were not observed to correlate with CIMT or FMD measurements. A significant increase of 25(OH)VitD3 levels after replacement therapy [11.3 (2.7-48.3), vs 30 (13-72) p=0.001] was observed after 3 months of therapy. Also, after replacement therapy, CIMT measurements improved (1,04 vs 0.50, p=0.001) and FMD measurements improved (4,9 vs 10,2, p=0.01) Conclusions A high presence of Vitamin D deficiency was observed in FMF patients from Turkey. Although impaired vascular endothelial function did not correlate with vitamin D levels, vitamin D deficiency may influence the course of the attacks and be related to some colchicine-resistant cases. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5994

Neutrophil-lymphocyte ratio (NLR), an indicator of inflammation, has been lately demonstrated as a prognostic factor and an indicator of disease activity in various diseases. However, the effects of NLR have not been investigated in mycosis fungoides (MF) patients yet. The aim of this study is to investigate the relationship between the NLR and treatment demand (systemic PUVA and/or chemotherapy), time to treatment, progression in stage, and time to progression in stage in MF patients. The data of 117 patients, who were followed with the diagnosis of MF at the Department of Dermatology in Istanbul Training and Research Hospital between April 2006 and January 2016, were analyzed retrospectively. The cutoff score for NLR was determined as 2 according to the median NLR level which was 1.96. At the time of diagnosis, the median age of patients was 54 years (range, 21-90) with 62 (53 %) female and 55 (47 %) male. Seventy-seven (65.8 %) patients required treatment during follow-up. Sixty-three (53.8 %) patients showed progression in disease stage. There was no significant difference in treatment demand, time to treatment, progression in stage, and time to progression in stage in patients with a NLR ≥ 2 and NLR < 2 (p = 0.331, 0.987, 0.065, and 0.119, respectively). It seems that there is no association between the NLR and treatment demand, time to treatment, progression in stage, and time to progression in stage in MF patients.

Background: We evaluated the frequency of subnormal erythropoietin levels, JAK2V617F positivity and polycythemia vera (PV) in patients who did not meet WHO 2008 criterion for hemoglobin levels but were suggested to be investigated for PV in 2016 revision. Materials and Methods: We assessed the data of 92 patients, who were further evaluated with JAK2V617F mutation and serum erythropoietin (EPO) levels and bone marrow biopsy, if necessary. We also compared this patient group with 20 patients whose Hgb>18.5 g/dL for men and >16.5 g/dL for women. Results: Nine patients (45%) in the higher hemoglobin group were JAK2V617F positive, while 4 patients (4.3%) in the lower hemoglobin group were JAK2V617F positive (p<0.001). The number of patients with serum EPO levels <4.3 mIU/mL was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=7, 7.6%) (p<0.001). Finally, the number of patients who received a diagnosis of PV was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=9, 9.8%) (p<0.001). Conclusion: We found a substantial increase in patients who were candidates for testing for PV with the introduction of WHO 2016 criteria; these patients were diagnosed with PV with a rate (9.8%) that cannot be underestimated.

Abstract. T-cell acute lymphoblastic leukemia (ALL) is an aggressive hematological malignancy, accounting for ~25% of all adult cases of ALL. We herein report a case of T-cell ALL exhibiting aberrant CD34, CD56, CD33 and CD117 expression in addition to T-cell markers, which did not respond to induction treatment. A 55-year-old woman was admitted to our hospital with a sore throat unresponsive to medication for 1 month. The laboratory examination revealed pancytopenia and the peripheral blood smear examination revealed blast cells. On flow cytometric analysis, the blast cells were found to be positive for cytoplasmic CD3, CD2, CD5, CD7, CD34, CD56, CD33 and CD117, and negative for myeloperoxidase, CD13, CD11b, CD15, CD19, CD79a, CD22 and CD10. The patient was diagnosed with T-cell ALL according to the 2008 World Health Organisation classification. The patient did not respond to Hyper-cyclophosphamide, vincristine, adriamycin and dexamethasone (CVAD) course A treatment and succumbed to the disease during Hyper-CVAD course B treatment. To the best of our knowledge, this is the first report of aberrant co-expression of the natural killer cell marker CD56, myeloid cell markers CD117 and CD33 and stem cell marker CD34 in a patient with T-cell ALL. This appears to be associated with an unfavorable outcome, despite the use of intensive chemotherapy.

IntroductionThe prevalence of Sjögren's syndrome (SS) in patients with the diagnosis of SpA has been reported to be higher than normal population. Yet, the vice-versa is unclear. In this study, we aimed to investigate the prevalence of IBP, radiologic sacroiliitis and SpA in patients with primary SS.Methods85 patients followed at the rheumatology clinics of the Marmara and Kocaeli Universities with the diagnosis of primary SS between November 2011 and August 2012 were included in this study. The control group consisted of 100 age-and gender-matched patients. Inflammatory back pain and axial SpA were diagnosed according to the assessment of spondylo arthritis International Society (ASAS) criteria.Results83 patients were (97%) female and 2 (3%) were male. Mean age of the patients was 49.1 (±11) years. Mean disease duration was 7.3 (±4) years. The patient and control groups were comparable in terms of age and gender (p > 0.05). Inflammatory back pain was observed in 21 (24.7%) of 85 primary SS patients and in 4 (4%) of 100 control subjects (p < 0.001), radiographic sacroiliitis was demonstrated in 9 (10.5%) of primary SS patients and 2 (2%) of the control subjects (p = 0.025). Remaining SpA findings were not encountered in either group.Conclusioninflammatory back pain and radiologic sacroiliitis is increased in patients with primary SS. Whether IBP, SI joint inflammation and radiologic sacroiliitis is due to the co-existence of SpA and primary SS or IBP is an underdiagnosed clinical feature of SS deserves further studies of large patient numbers.

Objective: Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by isolated thrombocytopenia. While first-line treatments focus on inhibiting autoantibodies and platelet destruction, second- and third-line treatments include splenectomy and thrombopoietin receptor agonists. In this study, we aimed to compare the efficiency and toxicities of splenectomy and eltrombopag as second-line treatments in ITP. Materials and Methods: We retrospectively analyzed patients who were diagnosed with ITP and followed between 2015 and 2020. Patients who underwent splenectomy or received eltrombopag treatment as second-line or further therapy were included. For subgroup analyses, patients were further stratified according to whether they received eltrombopag in the second or third line of treatment. Results: There were 38 patients in the splenectomy group and 47 patients in the eltrombopag group. The mean age of patients in the splenectomy and eltrombopag groups was 43.2 and 50.5 years, respectively. Time to response was significantly shorter in the splenectomy arm (p=0.001). However, response rates at the 3 rd , 6 th , 12 th , and 24 th months did not exhibit a statistically significant difference between groups; nor did total duration of response and adverse events. Response rates at the 1 st , 3 rd , 6 th , 12 th , and 24 th months and the total duration of response did not exhibit a statistically significant difference between eltrombopag subgroups. Eltrombopag treatment was ceased for 20 patients after a median of 54.1 months (range: 1-151). Among them, 12 patients (60%) did not experience a loss of response. Conclusion: Comparing the splenectomy and eltrombopag arms, even though time to achieve response was in favor of the splenectomy group, this advantage disappeared when overall response rates and response rate at the 2 nd year were considered. Using eltrombopag in the second or third line of therapy does not yield any difference in terms of time to achieving response.

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The neutrophil-lymphocyte ratio (NLR), which is a cheap and easily available indicator of inflammation, has lately been demonstrated to be a prognostic factor of various solid malignancies as well as hematological malignancies such as diffuse large B-cell lymphoma (DLBCL), Hodgkin's lymphoma (HL), multiple myeloma (MM), and peripheral T-cell lymphoma (PTCL) (1-6). However, the relationship between NLR and clinicopathological parameters during the diagnosis of follicular lymphoma (FL) has not been investigated to date. Methods The data of 21 patients who were diagnosed with FL between December 2011 and May 2015 were retrospectively analyzed. The data, gathered from the medical records, including gender; age; lactate dehydrogenase (LDH) levels; presence of B symptoms; Ann Arbor stage; FLIPI score; extranodal, bone marrow, hepatic, and splenic involvements; tumor grade; treatment status; and follow-up period, were recorded. NLR was calculated on the basis of the complete blood count of patients prior to biopsy. The study protocol was approved by the local ethics committee. The median age of the cohort was 57 years (range, 43-78), with 11 males and 10 females. Three (14.3%) patients had stage I-II disease and 18 (75.7%) had stage III-IV disease. The tumor characteristics were determined as grade 1-2 in 11 (52.4%) patients, grade 3A in 8 (38.1%), and grade 3B in 2 (9.5%). LDH levels were above normal in 12 (57.1%) patients. The median FLIPI score was 2 (range, 0-4). B symptoms were present in 7 (33.3%) patients. Extranodal involvement was observed in 9 (42.9%) patients. One (4.8%) patient presented with hepatic involvement, 6 (28.6%) with splenic involvement, and 8 (38.1%) with bone marrow involvement. Thirteen (62%) patients received treatment. During follow-up (range, 2-41 months), all the patients were alive. Only 1 patient who was treated progressed to aggressive disease. Therefore, we did not evaluate overall survival and progression-free survival of the cohort. The median NLR was 2.01 (range, 0.46-4.59), and the cutoff score for NLR was determined to be 2. NLR was >2 in 11 (52%) patients. No correlation was found between NLR and LDH; FLIPI score; disease stage; tumor grade; treatment necessity; presence of B symptoms; and presence of extranodal, bone marrow, hepatic, and splenic involvement (p>0.05) (Table 1). Discussion There is increasing evidence that neutrophils, a constitutive member of cancer-related inflammation, contribute to tumor progression and metastasis (7, 8). On the contrary lymphocyte subtypes Relationship between Neutrophil-Lymphocyte Ratio and Clinicopathological Parameters in Follicular Lymphoma Objective: We aimed to investigate the relationship between the neutrophil-lymphocyte ratio (NLR) and clinicopathological parameters during the diagnosis of follicular lymphoma (FL). Methods: The data of 21 patients who were diagnosed with FL between December 2011 and May 2015 were retrospectively analyzed. The median NLR was 2.01 (range, 0.46-4.59), and the cutoff score for NLR was determined to be 2....