Double trouble: myocardial infarction with non-obstructive coronary arteries as a presentation of Hughes syndrome in monozygotic twins.

Myocardial infarction with non-obstructive coronary arteries (MINOCA) is a recently described, clinically significant entity, with prevalence rates ranging from 1% to 14% and a mean of 6% of all patients with myocardial infarction. Antiphospholipid syndrome (APS; Hughes syndrome) is characterized by the presence of antiphospholipid antibodies associated with thrombosis (arterial and/or venous) and/or pregnancy morbidity and could be the cause of MINOCA. Data on genetic predisposition to APS are scarce. The present study describes a unique case of monozygotic twin brothers who, at a young age, developed the same clinical presentation of APS. The diagnosis of APS was later confirmed, along with a diagnosis of systemic lupus erythematosus in one brother.

The cutaneous manifestations are significantly related to cerebrovascular in a Serbian cohort of patients with Hughes syndrome.

Objectives To investigate a possible relationship between cerebrovascular, such as stroke and transient ischaemic attack, and various cutaneous manifestations (livedo reticularis, skin ulcerations, pseudovasculitis lesions, superficial cutaneous necrosis and digital gangrene) in antiphospholipid syndrome (APS). This report is based on a Serbian cohort of APS patients. Methods A total of 508 antiphospholipid syndrome APS patients were assessed: 360 with primary (PAPS) and 148 with APS associated with SLE (SAPS). Antiphospholipid antibodies analysis included detection of anti-cardiolipin (IgG/IgM), anti-ß2glycoprotein I (IgG/IgM) and positive lupus anticoagulant test. Results The prevalence of cutaneous manifestations in our cohort was significantly higher in the SAPS group (76.4% vs. 27.2%, p = 0.0001). In both groups, the most common manifestation was livedo reticularis. The majority of cutaneous manifestations were significantly associated with cerebrovascular events in SAPS and PAPS. Cutaneous manifestations were independent predictors of transient ischaemic attack and stroke in PAPS patients (odds ratio 2.850, 95% confidence interval 1.562-5.202, p = 0.001, odds ratio 1.832, 95% confidence interval 1.024-3.277, p = 0.041, respectively). Conclusion In this cross-section analysis of a large cohort of Serbian APS patients, there was a strong relationship between cutaneous and cerebrovascular manifestations, suggesting a more cautious approach regarding neurological symptoms, especially in PAPS patients with cutaneous manifestations present.

Does the presence of secondary antiphospholipid syndrome in patients with systemic lupus erythematodes accelerate carotid arteries intima-media thickness changes?

Patients with systemic lupus erythematosus (SLE) have an increased risk of atherosclerosis. The aim of our study was to evaluate the importance of secondary antiphospholipid presence (SAPS) in light of carotid artery intima-media thickness (CIMT) changes in SLE patients. Our study included 120 patients with SLE (46.02 ± 13.16 years), 108 women and 12 men divided into two groups: 58 patients with SAPS and 62 SLE patients without SAPS taken as a control group. All patients underwent assessment of CIMT of right and left common carotid artery (CCA) and left and right internal carotid artery (ICA) by Doppler ultrasonography. In SAPS group, 48.3 % patients had significant changes of carotid arteries comparing to 16.1 % patients in control group (p = 0.008). Average CIMT values in left and right CCA and right ICA were significantly higher in SAPS group. No significant relationship between antiphospholipid antibody type and CIMT changes was established. Multivariate regression analysis revealed SAPS as a significant predictor of CIMT changes in SLE patients (p = 0.025). Presence of SAPS in SLE patients is associated with significant CIMT changes. Additional autoimmune burden leads to a need for a more aggressive education and prevention considering standard risk factors in this group of patients.

Survivin expression in patients with newly diagnosed nodal diffuse large B cell lymphoma (DLBCL).

Survivin is one of the inhibitors of apoptosis proteins (IAP) that might play an important role in the pathogenesis of diffuse large B cell lymphoma (DLBCL). The present study was designed to investigate the clinical and prognostic significance of survivin expression in nodal DLBCL. We analyzed lymph node biopsy specimens obtained from 56 patients with newly diagnosed nodal DLBCL, treated with immunochemotherapy (R-CHOP). The expression of survivin was analyzed using the standard immunohistochemical method on formalin-fixed and routinely processed paraffin-embedded lymph node specimens and evaluated semiquantitatively as a percentage of tumor cells. Survivin immunoexpression (>45 % positive tumor cells) was found in 22 (39.28 %) and observed as cytoplasmic staining in 15 patients, or mixed (cytoplasmic and nuclear) staining in 7 patients. A significant difference in survivin immunoexpression was noticed between the GCB and the non-GCB subtypes of DLBCL (p = 0.031). However, survivin immunoexpression had no significant association with IPI, "bulky" disease, extranodal localization, hemoglobin, Ki-67 immunoexpression or other clinicopathological parameters. A univariate analysis showed that survivin positivity was an unfavorable factor for therapy response and a predictor of shorter survival in patients with DLBCL (p = 0.048 and p = 0.034, respectively). Patients with survivin overexpression experienced a relapse more often than patients without expression of this apoptotic protein (27.3 vs. 11.8 %), but this difference did not reach statistical significance (p = 0.131). The results of this study showed that disregulation of survivin expression had an important role in the determination of the course of the disease in patients with nodal DLBCL treated with R-CHOP. Therefore, survivin represents a potential target for therapeutic intervention in DLBCL.

Pulmonary events in antiphospholipid syndrome: influence of antiphospholipid antibody type and levels.

OBJECTIVE: This prospective clinical study examined the association between subclasses of antiphospholipid antibodies (aPL) and pulmonary manifestations in antiphospholipid syndrome (APS). METHODS: The cohort involved 329 patients: 214 patients with primary APS (PAPS) and 115 patients with secondary APS (SAPS). aPL analysis included detection of serum anticardiolipin antibodies [aCL (IgG/IgM)], ß2 glycoprotein I [ß2GPI (IgG/IgM)], and lupus anticoagulant (LA). RESULTS: In SAPS, high aCL IgG levels (> 100 PLU/mL) were more common in major pulmonary arterial thrombosis (p = 0.006) and medium aCL IgG levels (41-99 PLU/mL) in adult respiratory distress syndrome (ARDS; p = 0.047) and fibrosing alveolitis (p = 0.002). aCL IgG antibodies were more common in SAPS (p = 0.037). In PAPS, fibrosing alveolitis was more common in patients with medium ß2GPI IgM levels (p = 0.0001). LA correlated with pulmonary embolism (p = 0.03) and microthrombosis (p = 0.03) in SAPS, and with pulmonary microthrombosis (p = 0.03) in PAPS. Males were more likely to develop secondary pulmonary hypertension when diagnosed with PAPS (p = 0.019). CONCLUSION: Certain classes of aPL are associated with distinct pulmonary manifestation, indicating their predictive role and importance in diagnosis and treatment of APS.

Influence of antiphospholipid antibody levels and type on thrombotic manifestations: results from the Serbian National Cohort Study.

Repeated thromboses are the most frequent clinical manifestation of antiphospholipid syndrome (APS) in the presence of antiphospholipid antibodies (aPL). The objective of this study was to observe the prevalence and localization of thrombosis, and to investigate the importance of aPL type and level for thrombosis-related events in patients diagnosed with APS. These are the first results of patients enrolled in Serbian National Cohort Study which comprises 256 patients: 162 with primary antiphospholipid syndrome (PAPS) and 94 with APS associated with systemic lupus erythematosus (SLE). aPL analysis included detection of aCL (IgG/IgM), ß(2)GPI, and lupus anticoagulant. Thrombosis was diagnosed in 119 (46.5%) patients, with higher prevalence in PAPS compared with SLE patients (51.2% and 38.3%, respectively, p = 0.045). There was similar prevalence of arterial thrombosis in PAPS and SLE groups (34.6% and 34%, respectively, p = 0.932) although venous thrombosis was more frequent in PAPS (25.9% and 8.5%, respectively, p = 0.001). Thrombosis was observed in 92 (55.8%) patients who had more than one type of antibody (category I), in 13 (41.9%) patients with category IIa, in 19 (46.3%) patients with category IIb, and in 73 (44.2%) patients with category IIc (p = 0.10). The patients with thrombosis were older than those without thrombosis (49.8 and 39.8 years, respectively, p = 0.001). Overall, older age was a risk factor for thrombosis. The prevalence of venous thrombosis was higher in the PAPS group, but with lower frequency than in literature data. Any aPL type and level is a risk factor for thrombosis.

Biological and clinical features of non-Hodgkin's lymphoma in the elderly.

PURPOSE: The incidence of non-Hodgkin's lymphomas (NHLs) in elderly people has increased in recent years because the world population is getting older. The aim of this study was to compare the biological and clinical features in patients diagnosed with NHLs younger and older than 65 years, and the possible influence of age on the choice of optimal therapeutic approach. METHODS: We retrospectively evaluated 193 patients with NHLs: 111 (68%) were <65 years and 82 (42%) ≥65 years. The following parameters were analysed: age, gender, clinical stage, International Prognostic Index (IPI), histological type, presence of B symptoms, disease localization, presence of bulky mass, Karnofsky performance status (PS), comorbidities, blood counts, liver and renal function and serum LDH. RESULTS: Elderly patients had statistically more frequent indolent NHLs (p=0.036), IPI 3 and 4 (p<0.0001), presence of comorbidities (p<0.001), and less frequent presence of bulky disease (p7equals;0.043). Response to therapy was different in the 2 age groups: 29% of patients ≥65 years achieved complete remission (CR) in contrast to 71% of patients <65 years (p<0.001). The most frequent cause of death was disease progression (PD) (86% of younger patients and 71% of elderly patients (p7equals;0.150). Older patients died more frequently because of comorbidities compared younger ones (21 and 107percnt;, respectively; p=0.250), and had more complications of therapy (8.1 and 47percnt;, respectively (p=0.320). Overall survival (OS) was shorter in older patients in all lymphoma types: indolent lymphoma (36 vs. 17 months), aggressive (22 vs. 20 months) and very aggressive (14 vs. 1 months). Multivariate analysis showed that parameters for shorter survival in the elderly were Karnofsky PS <60, increased serum LDH and treatment toxicity. CONCLUSION: In elderly NHLs patients, treatment response and survival are significantly poorer. Since older patients mostly died of PD, they should be treated with standard regimens and best supportive measures.

Disconnection of chamber and catheter as a complication of central venous catheter type port-a-cath.

The use of a central vein catheter (CVC) type port-a-cath (VPS), apart from the comfort it provides to the patient undergoing chemotherapy, also carries certain complications. In this study, our patient was subjected to chemotherapy after a radical breast cancer operation and was given a CVC type VPS. After further care, a rare complication was verified--disconnection of the chamber and catheter, which one was visually identified in the right heart chamber. As the patient was vitally endangered, she was immediately hospitalized and the catheter was removed by catheterization of the right femoral vein, with scopic imaging. Early diagnosis and localization of the problem prevented more severe complications and mortality.

Weight and body composition changes during R-CHOP chemotherapy in patients with non-Hodgkin's lymphoma and their impact on dose intensity and toxicity.

PURPOSE: To estimate weight and body composition changes during R-CHOP combination therapy in patients with non-Hodgkin's lymphoma (NHL) and their impact on dose intensity (DI) and toxicity. METHODS: We prospectively evaluated body composition in patients with NHL before starting chemotherapy (visit 1), before the 3rd cycle (visit 2) and before the 6th cycle (visit 3). Body composition was assessed by bioelectrical impedance analysis (BIA) and confirmed by anthropometric measurements. RESULTS: Thirty patients with NHL were studied. There was no weight change from visit 1-2, but weight increased from visit 2-3 (-1.36 - or + 1.89 kg) and from visit 1-3 (-1.93 + or - 3.21 kg). Patients with weight gain had significantly better overall response rate (p=0.013) and 5-year survival rate (p <0.01). Fat mass increased from visit 1-2 (-1.068 + or - 1.72 kg; p=0.002), from visit 2-3 (-1.32 + or - 1.89 kg; p=0.001) and from visit 1-3 (-2.502 + or - 3.23 kg; p=0.001). There was no statistically significant change in lean body mass (LBM) during chemotherapy. Total body water changed significantly from visit 1-2 (-0.08 + or -2.55l kg; p=0.097), from visit 2-3 (-1.036 + or - 1.10 kg; p=0.001) and from visit 1-3 (-1.89 + or - 3.2l kg; p=0.004). The average relative DI (ARDI) of the R-CHOP regimen was 90% and the rate of complete remission was 63.3%. Overall hematologic toxicity was evident in 14 (46.7%) patients. There was statistical significance between concentrations of cyclophosphamide and doxorubicin (mg/kg fat and mg/kg LBM) whether overall hematologic toxicity was present or not. CONCLUSION: Patients in the study gained weight during chemotherapy with unfavorable changes in body composition. Attempt has been made to identify clinical variables to predict patients at risk for hematologic toxicity, but an approach for individualizing drug dosing should be continued.

Granular cell tumor of the common hepatic duct presenting as cholangiocarcinoma and acute acalculous cholecystitis.

Granular cell tumors (GCT) are rare benign tumors. Less than 1% of GCTs involve the extrahepatic biliary tree. Most researches favour a Schwann cell origin. Patient, caucasion, female, 31-year-old presented with 4 month history of painless jaundice and pruritus. US and CT revealed dilatation of intrahepatic biliary tree and surgery was performed. Firm tumor mass was found above the conjunction of cystic duct and common hepatic duct (CHD) that caused obstruction and gallblader empyema. The patient underwent radical surgical procedure because Klatskin tumor was clinically suspected. Patohystology and immunohistochemistry confirmed granular cell tumor. Eight years after surgery the patient is wellbeing without symptoms. To our knowledge 69 cases of GCT of the extrahepatic biliary tree have been reported and none of the acute acalculous cholecystitis case acompanied by GCT of CHD. Granular cell tumors are rarely diagnosed preoperatively. Wide resection offers the best chance for cure.

Short-segment jejunoplasty: the option treatment in the management of benign esophageal stricture.

A non-dilatable benign stricture of the esophagus is a problem for the patient and always a challenge for the surgeon. The present study is intended to provide some details of surgical technique and the physiological concept that constitutes the basis for patient selection for short-segment jejunoplasty. In this study, out of 98 patients, 91 had complex stricture of the lower, and seven of the cervical esophagus. The main cause of stenosis in 60 patients (61.2%) was gastroesophageal reflux, in 34 (34.7%) post-corrosive damage, and in three (3.0%) it was other causes. All patients were treated by three methods of jejunoplasty: group I (n=54 patients) short-segment of the jejunum; group II (n=37 pts) Roux-en-Y double tract; and group III (n=7 patients) short jejunal segment on the long vascular pedicle. Intraoperative complications occurred in 17 (17.34%) of the patients. An anastomotic leak occurred only in three (3.29%) in group I and II, and 1/98 patients (1.02%) died from mediastinitis. Follow-up of functional results for up to 36 months (average 18 months) was available in 77/97 (79.3%) hospital survivors. Fifty-seven (74%) patients are satisfied with their ability to take food, postoperative reflux was completely denied by 72 (93.5%) patients. Postvagotomy diarrhea was transient and gradually subsided over the course of the first 6 months.