Another side of lupus: A case report of lupus presenting with cutaneous vasculitis.

Systemic lupus erythematosus (SLE) is a systemic autoimmune complex disease that affects any organ, characterized by immune complex formation and autoantibody production. Lupus vasculitis begins at a young age. These patients generally have a longer disease duration. Ninety percent of cases in lupus-associated vasculitis present with cutaneous vasculitis. Disease activity, severity, organ involvement, response to treatment and drug toxicity determine the frequency of outpatient control in lupus. Depression and anxiety are observed more frequently in SLE than in the normal population. Our case, it is an example of the patient's disruption of controls due to psychological trauma and that lupus can cause serious cutaneous vasculitis. In addition, psychiatric evaluation of lupus cases from the time of diagnosis may have a positive effect on the prognosis.

The development of ankylosing spondylitis in a patient with familial hemophilia.

Hemophilia is a rare inherited disease which causes bleeding due to Factor VIII or Factor IX deficiency. It is usually X-linked recessive and typically affects males. Arthropathy occurs as a result of cartilage damage and chronic synovitis due to recurrent intra-articular bleeding in hemophilic patients and is mostly seen in the knee, shoulder, hip and ankle joints. There are many other diseases that cause chronic synovitis ankylosing spondylitis (AS), which is a subtype of spondyloarthropathies that cause chronic low back pain, more common in men younger than 45 years of age. In addition to axial involvement, peripheral arthritis, uveitis, enthesitis and dactylitis can be seen. Although the etiology is not fully known, genetic and environmental factors are responsible for the pathogenesis. In this study, we aimed to present congenital hemophilia and AS coexistence in a 22-year-old male patient.

Relation of whole-body metabolic tumor volume and total lesion glycolysis on fluorodeoxyglucose PET/computed tomography with clinical and laboratory parameters in newly diagnosed multiple myeloma.

OBJECTIVE: This study aims to evaluate the relationships of metabolic fluorodeoxyglucose PET/computed tomography (FDG PET/CT) parameters such as whole-body metabolic tumor volume (WB MTV), WB-total lesion glycolysis (TLG), and bone marrow (BM)-mean standard uptake value (SUVmean) with clinical stage and other prognostic biomarkers in newly diagnosed multiple myeloma (MM) patients. METHODS: Patients who underwent pretreatment PET/CT with the diagnosis of MM were evaluated retrospectively. The number of focal lesions, WB MTV, WB TLG, and BM SUVmean values were measured on FDG PET/CT images. Clinical stages and prognostic laboratory parameters were recorded the pretreatment period. RESULTS: WB MTV and WB TLG values were significantly higher in patients with more than three focal lesions on FDG PET/CT scan (all P < 0.001). According to the Revised International Staging System (R-ISS), all WB MTV, WB TLG, and BM SUVmean values are significantly higher in patients with stage 3 disease than in stages 1-2 ( P = 0.027, P = 0.019, P = 0.001, respectively). Serum creatinine level is positively correlated with WB MTV, WB TLG, and BM SUVmean values ( P = 0.020, P = 0.004, P < 0.001, respectively). In addition, the ß2 microglobulin level, an essential biochemical prognostic parameter, was positively correlated with the BM SUVmean value ( P = 0.013). CONCLUSION: The BM SUVmean, WB MTV, and WB TLG values, which reflect FDG avid WB tumor burden, are associated with prognostic biomarkers and R-ISS stage in newly diagnosed MM patients. It contributes to the identification of high-risk patients at the pretreatment staging.

Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience.

The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17-77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7-183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.

Is CONUT score a prognostic index in patients with diffuse large cell lymphoma?

Background/aim: The aim of the study was to evaluate the effect of Controlling Nutritional Status (CONUT) score on the prognosis in patients with diffuse large B-cell lymphoma (DLBCL). Materials and methods: The present study was a retrospective study. The CONUT score was calculated based on serum albumin, total cholesterol and lymphocyte levels. This study included a total of 266 patients, 131 (49.2%) were female and 135 (50.8%) were male. The median follow-up period was 51 months (range: 1­190). Results: The median age was 64 years. The cut off CONUT was 1.5. There was a significant difference between patients with high (≥ 2) or low (< 2) CONUT scores in terms of overall survival (OS) and progression-free survival (PFS). The 5-year OS and PFS in patients with high CONUT score was 52.1% and 49.7%. The 5-year OS and PFS in patients with low CONUT score was 79.8% and 75.6% (p < 0.001). In the multivariate analysis for OS, age ≥ 65 years (HR = 1.80, p = 0.028), Eastern Cooperative Oncology Group (ECOG) > 1 (HR = 2.04, p = 0.006), stage IIIA­IVB disease (HR = 2.75, p = 0.001) and the CONUT score (HR = 1.15, p = 0.003) were found statistically significant. In the multivariate analysis for PFS, age ≥ 65 years (HR = 2.02, p = 0.007), stage IIIA­IVB disease (HR = 2.42, p = 0.002) and the CONUT score (HR = 1.19, p = 0.001) were found to be significant parameters. Conclusion: High CONUT score reduces OS and PFS in DLBCL. CONUT score is an independent, strong prognostic index in patients with DLBCL.

Efficacy of CLARA in recurrent/refractory acute myeloid leukaemia patients unresponsive to FLAG chemotherapy.

We hereby report our multicentre, retrospective experience with CLARA in patients with fludarabine/cytarabine/G-CSF (FLAG) refractory AML. The study included all consecutive R/R AML patients, who received CLARA salvage during October 2010-October 2015 period. All patients were unresponsive to FLAG salvage chemotherapy regimen and did not undergo previous allo-HCT. A total of 40 patients were included. Following CLARA 5 (12.5%) patients experienced induction mortality and 10 (25%) patients achieved CR. 25 (62.5%) patients were unresponsive to CLARA. 7 (17.5%) out of 10 patients in CR received allo-HCT. Median overall survival of patients who achieved CR after CLARA was 24.5 months (8.5-54.5) and 3 months (2.5-5), in patients who underwent and didn't allo-HCT, respectively. Our results indicate that CLARA may be good alternative even in FLAG refractory AML patients and can be used as a bridge to allo-HCT, who have a suitable donor and able to tolerate the procedure.

Autologous stem cell transplantation and stem cell mobilization kinetics in elderly patients with B cell non-Hodgkin lymphoma.

As known, the world population is aging and as the life span increases the number of advanced-age lymphomas also shows an upward trend. Autologous hematopoietic stem cell transplantation (HSCT) is the standard treatment modality in chemotherapy-sensitive relapsed or refractory aggressive lymphomas. Increased morbidity and mortality related to both the transplant itself and comorbid diseases can be observed in elderly lymphoma patients. Patients who are 65 years or older and underwent autologous HSCT with B-cell non-Hodgkin lymphoma were retrospectively included in our study. In terms of survival analysis, median follow-up was 34.5 months (8-159) while the overall survival (OS) was 58%. In the univariate analysis of prognostic data in OS, patients who were referred to transplantation with complete response had a statistically significant survival advantage (p=0.043). In terms of the effect of pre-transplant conditioning regimens on survival, BEAM regimen yielded better results, though not statistically significant. Age, number of chemotherapy cycles received before mobilization and radiation therapy had no significant effect on the CD34 (+) cell count in the final product (p=0.492, 0.746 and 0.078 respectively). In conclusion, autologous HSCT is a practicable treatment modality that provides survival advantage in suitable advanced-age patients with a diagnosis of B-cell non-Hodgkin lymphoma.

Bone mineral density in geriatric patients with toxic nodular goiter.

BACKGROUND AND AIMS: We aimed to assess bone mineral density (BMD) in geriatric patients with hyperthyroidism caused by TNG. MATERIALS AND METHODS: Bone mineral density was measured at the lumbar spine and proximal femur, using dual-X-ray absorptiometry (DXA) in 90 patients with TNG (mean age; 69.2 ± 4.4 years) and compared with those in 42 age-matched healthy subjects (mean age; 68.40 ± 4.3 years). Serum levels of TSH, fT3, fT4, 25-OH vitamin D and PTH were measured. RESULTS: BMD was significantly lower at total spine (0.904 ± 0.1 vs. 1.114 ± 0.1 g/cm(2)) and total hip (0.850 ± 0.1 vs. 1.079 ± 0.1 g/cm(2)) in male patients with TNG in comparison to the healthy men (p = 0.001, p < 0.001). Postmenopausal women with TNG had lower BMD measurements at total lumbar spine (0.754 ± 0.1 vs. 0.870 ± 0.2 g/cm(2), p < 0.001) and total hip (0.765 ± 0.1 vs. 0.831 ± 0.2 g/cm(2), p < 0.001) in comparison to the healthy women. T scores of total lumbar spine and hip were lower in patients with subclinical hyperthyroidism compared to the control group, respectively (-1.9 ± 1.7 vs -0.8 ± 1.8, p = 0.007; -1.09 ± 1.2 vs. -0.02 ± 1.6, p = 0.001). While serum levels of fT3 and fT4 revealed a negative correlation with T score of BMD measurements at the total spine and hip, TSH levels were positively correlated. We did not find a difference in serum calcium, phosphorus, 25-OH vitamin D and PTH levels between the two groups (p > 0.005). CONCLUSION: Geriatric patients with hyperthyroidism secondary to TNG had reduced BMD at the total spine and hip. Thus, we suggest to investigate bone mineral density in geriatric patients with TNG.

Serum macrophage migration inhibitory factor levels in Hashimoto's thyroiditis; a case control study.

OBJECTIVE: The cell-mediated immune process by CD4+ and CD8+ lymphocyte subsets of T-cells has a major role in the pathogenesis of Hashimoto's thyroiditis (HT). However, the exact mechanisms of initiation and progression of thyroid autoimmunity have not been completely clarified yet. Macrophage migration inhibitory factor (MIF) is commonly recognized as playing vital roles in various autoimmune diseases. Ee aimed to investigate serum MIF levels in subjects with HT and correlate them with the level of thyroid hormones and autoantibodies. MATERIALS AND METHODS: This study included 93 patients with untreated Hashimoto's thyroiditis and 53 healthy controls. We measured serum levels of TSH, free T4 (FT4), free T3 (FT3), anti-thyroglobulin autoantibody (TGAb) and anti-thyroid peroxidase autoantibody (TPOAb) in all patients and thyroid ultrasonography was performed. The concentration of MIF was measured using enzyme-linked immunosorbent assay (ELISA) method. RESULTS: We enrolled 93 patients with HT (mean age; 31.3 ± 11.1 years), and 53 healthy control group (mean age; 29.3 ± 8.5 years) in the current study. The patient group consisted of 52 with euthyroid autoimmune thyroiditis, 31 with subclinical hypothyroidism and 10 with overt hypothyroidism. Serum levels of MIF were higher in patients with overt hypothyroidism (6300.9 ± 2504.3 pg/ml) than the euthyroid patients (3955.2 ± 3013.6 pg/ml) (p = 0.036). CONCLUSION: MIF increases in overt hypothyroidism due to the Hashimoto's thyroiditis. Further investigations are needed to explore the role of MIF in pathogenesis of Hashimoto's thyroiditis.

Alterations of thyroid volume and nodular size during and after pregnancy in a severe iodine-deficient area.

OBJECTIVE: The effects of pregnancy on thyroid nodules were investigated in a few number of studies. We aimed to evaluate the prevalence of thyroid nodules, the changes in size, volume and number of nodules during pregnancy and after delivery in pregnant women. DESIGN AND METHODS: This prospective study was performed in a severe iodine-deficient area and included 83 pregnant women (mean age 30·4 ± 5·5 years). We evaluated thyroid hormone levels, ultrasound examination of thyroid and urine iodine concentration (UIE) at each trimester and at 3-month post-partum period (PP). All patients with thyroid nodules >1 cm underwent fine-needle aspiration biopsy (FNAB) after the last visit at the PP. RESULTS: Twenty-six women had thyroid nodules on thyroid ultrasonography at the first trimester. The volume of single/dominant nodule showed enlargement during pregnancy and remained at the PP; however, it was not significant (first trimester: 0·83 ± 0·8 ml; second trimester: 0·92 ± 1 ml; third trimester: 0·99 ± 1·2 ml; PP: 0·92 ± 1·2 ml). The maximum diameter of single/dominant nodule in the third trimester of pregnancy (12·6 ± 5·4 mm) was greater than the first trimester (11·9 ± 4·8 mm) (P = 0·002). The number of nodules did not change during pregnancy. The mean TV increased during pregnancy and remained 3 months after delivery (P < 0·001), and the maximum value of TV was reached in the third trimester (14·2 ± 7·9 ml). FNAB results revealed a 6·6% prevalence of malignancy among the nodules. CONCLUSIONS: Thyroid nodules were present in 30·1% of pregnant women. While size of the single/dominant thyroid nodule increased significantly during pregnancy, the number of nodules did not change.

Routine Screening for Cushing's Syndrome Is Not Required in Patients Presenting with Obesity.

Background. Cushing's syndrome (CS) is a relatively unusual condition that resembles many of the phenotypic features of obesity. Our aim was to evaluate the frequency of CS in obese patients. Materials and Methods. This study included 354 consecutive patients (87.9% female, age 37.8 ± 13.4 years) who presented with simple obesity. All the patients were evaluated for the clinical signs of CS. Lipid parameters, fasting glucose (FPG) and insulin, 75 gr oral glucose tolerance test, basal cortisol and ACTH were measured. 1 mg overnight DST was performed. Results. The mean weight of the patients was 102.4 ± 20.1 kg and BMI 40 ± 7.35 kg/m(2). 34.5% of the patients were hypertensive. 36.2% of the patients had central obesity, 72% dorsocervical fat accumulation, 28.8% abdominal striae and 23.2% acne. 49.4% of the women had hirsutism. 46.5% had prediabetes and 12.0% had type 2 diabetes, 72.6% had dyslipidemia. The mean cortisol and ACTH levels were as follows: 9.28 ± 3.53 µ g/dL and 17.02 ± 10.43 pg/mL. Seven patients failed to suppress plasma cortisol to less than 1.8 µ g/dL. Biochemical confirmation tests were performed in these patients and 2 of them were diagnosed glucocorticoid-secreting adrenal adenoma. Conclusions. Routine screening for CS in obese patients is not required.