An Unusual Presentation of Plasma Cells - Castleman Disease: A Case Report.

We present the case of a 76 year old female patient admitted in the Department of Cardiology for physical asthenia, profuse sweating and dyspnea with orthopnea for about one month. Clinical and paraclinical assessments performed at admission confirmed the diagnosis of cardiac tamponade. Surgical intervention was performed and 400 mL of clear effusion were drained. Post-operative evolution was marked by recurrence of symptoms, requiring after 3 weeks a new drainage of 600 mL of clear effusion, and biopsy of the pericardium was performed. Pathological exam described serous pericarditis with chronic inflammatory infiltrate, xanthogranulomatous reaction intricated in the pericardium and mesothelial hyperplasia. The patient was subsequently transferred to the Department of Internal Medicine for further investigations. Physical examination showed a patient with altered general status, pallor, vesicular murmur absent in both bases, presenting cutaneous hyperpigmentation at the level of the right hemi-abdomen and hip with posterior extension, and a peripheral indurated erythematous plaque. The patient presented nodular masses of 3 cm in the right latero-cervical and bilateral axillary regions, non-adherent to the superficial structures, as well as adenopathic blocks in both inguinal regions. CT scan of the thorax and abdomen showed moderate bilateral pleuresia, minimal pericardial effusion (15 mm) and multiple adenopathies on both sides of the diaphragm. Skin biopsy was performed, as well as bone marrow aspirate and excision of a right axillary lymph node. Pathological exams and immunohistochemistry tests confirmed the diagnosis of Plasma Cells Castleman disease.

Noninvasive Markers of Improvement of Liver Steatosis Achieved by Weight Reduction in Patients with Nonalcoholic Fatty Liver Disease.

UNLABELLED: Nonalcoholic fatty liver disease (NAFLD) is strongly associated with insulin resistance and metabolic syndrome, which are linked to obesity. The aim of the study was to assess if weight reduction through 12 months of lifestyle intervention and exercise would lead to improvement of steatosis. METHODS: In a prospective observational study 86 overweight subjects (51 men, 35 women) with steatosis were recruited, after excluding other etiologies. Patients were assigned a caloric goal and a daily fat goal. Physical activity focused on moderate-intensity activities. Blood samples (biochemistry, HOMA-IR, cytokine levels, steatotest) were collected at entry and months 6 and 12. All subjects underwent abdominal CT scan before commencement and after 12 months to assess visceral and subcutaneous adipose tissue (VAT/SAT) area. RESULTS: After 12 months baseline descriptive characteristics (weight, BMI, waist circumference) decreased significantly. Biochemical parameters that decreased significantly were: GGT (40.0 ± 18.0 vs 31.1 ± 13; p = 0.01), ALT (58.5 ± 23.5 vs 32.7 ± 14.8; p = 0.001), cholesterol (236.4 ± 54.8 vs 204.8 ? 91; p = 0.05), LDL (160.1 ± 47.4 vs 125.3 ± 40; p = 0.05) and HOMA-R (4.86 ± 0.63 vs 3 ± 0.41; p = 0.018). Steatotest improved significantly (0.68 ± 0.16 vs 0.38 ± 0.14; p = 0.02). Modification of adipocytokines was significant for leptin (p = 0.018) and adiponectin (p = 0.003). Factors associated with regression of steatosis were weight, BMI, ALT, waist circumference, GGT, HOMA, leptin, VAT and steatotest. Multivariate logistic regression showed the following factors related to improved steatosis: BMI < 25 kg/m2, ALT < 42 U/L, leptin < 10.5 ng/ml and adiponectin > 8.4 µg/ml. CONCLUSIONS: Overweight persons who achieve significant reductions in body weight through 12 months of physical activity and low caloric diet can decrease liver fat, VAT and SAT. Even in those with minimal weight loss ALT levels, steatosis, adipokines and cardiovascular risk factors improved.

Diffuse polypoid lesions of the digestive tract in severe hypogammaglobulinemia.

We present a case report of diffuse polypoid lesions of the digestive tract in which multiple endoscopic techniques (upper endoscopy, colonoscopy, and capsule endoscopy) were performed in order to complete and refine the diagnosis in a patient with severe hypogammaglobulinemia with recurrent infections, bronchiectasies and diffuse nodular lymphoid hyperplasia of the digestive tract. In patients with hypogammaglobulinemia, the endoscopic evaluation of the digestive tract might be mandatory, even in asymptomatic patients. The complete assessment should include the upper endoscopy and colonoscopy with multiple biopsies and, if possible, capsule endoscopy.

Platelet histogram indices and cardiovascular disease in patients with rheumatoid arthritis.

UNLABELLED: BACKGROUND; Previous studies reported the increased prevalence of cardiovascular disease (CVD) in patients with rheumatoid arthritis (RA) compared to the general population. However, the predictors for the development of CVD in patients with RA were not clearly established, and the role of thrombosis mechanisms was inconsistently characterized in these patients. The aim of this study was to evaluate the platelet histogram indices, as markers of platelet activation, in patients with RA with or without CVD. MATERIAL AND METHODS: In 64 pts with RA (mean age: 58.0 +/- 12.7 yrs) we performed the standard clinical evaluation and biochemical workup with platelet histogram, including mean platelet volume (MPV) and platelet distribution width (PDW) as markers of platelet activation. We divided the study population into two groups: A - 41 patients with RA without CVD and B - 23 patients with RA and CVD (ischemic heart disease, peripheral artery disease or cerebrovascular disease). The values of MPV and PDW were also analyzed in an age- and sex-mached control group of 20 subjects without RA and CVD and in a group of 62 patients with CVD without RA (stable angina). RESULTS: The platelets number was similar in both groups, but the platelet histogram showed higher values for MPV (9.6 vs. 8.6 fL, p < 0.01) and PDW (16.1 vs. 14.0, p < 0.01) in patients with RA and CVD, reflecting greater platelet activation in these patients. MPV values were lower in patients with RA, but the values of PDW were higher in these patients comparing to control. Patients with RA with CVD have higher values of PDW than patients with CVD, but without RA, showing an increased platelet activation in RA. The PDW values correlate with fibrinogen (0.63; p = 0.003) but not with CRP or ESR, while the MPV was not correlated with the inflammatory markers in patients with RA. CONCLUSIONS: The pathogensis of CVD in patients with RA may be linked to an increased prothrombotic activity which might be evaluated by platelet histogram indices.

Endothelial dysfunction in inflammatory rheumatic diseases.

The importance of cardiovascular disease in inflammatory rheumatic diseases was recognized as one of the determinants of increased mortality in these patients. An increased cardiovascular disease was reported in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), but also in other rheumatic diseases. Several hypotheses were elaborated, but the chronic inflammatory status seems to be a primordial factor. Therefore, the diagnostic and treatment of cardiovascular disease, even in subclinical status, should be one of most important goals in the global management of these patients. The endothelial dysfunction is now regarded as an important and early step in the processes that promote atherosclerosis. In patients with inflammatory rheumatic diseases, the presence of the endothelial dysfunction was reported and linked with several clinical or biological features of each disease. Moreover, the potential benefits on the endothelial dysfunction of several therapies were assessed especially in patients with RA or SLE. The aim of this article is to review the impact of the endothelial dysfunction and the methods to improve it in patients with these conditions.

Young woman with polyserositis, ovarian cystic mass and increased level of CA-125. Case report of peritoneal and pleural tuberculosis.

A 21-year-old woman was addressed to our department for progressive abdominal swelling, fatigue and fever. The clinical examination, the ultrasound examination and the computed tomography showed the presence of polyserositis (ascites and pleural effusion) and revealed a cystic mass at the level of right ovary. The laboratory work-up showed an increased level of CA-125, suggesting a malignancy. The thoracoscopy with visualization of the pleura revealed disseminated small white spots. The laparoscopic exploration of the pelvis and of the peritoneum also showed the same disseminated lesions and a cystic-like mass at the level of the right ovary which was excised and diagnosed as a benign cyst. At the analysis of the frozen and paraffin sections, the diagnostic of pleural and peritoneal tuberculosis was made and the specific quadruple treatment was started with a good evolution at two months and with the normalization of the CA-125 level. This case report underlines the importance of tuberculosis in the differential diagnosis of patients with polyserositis and increased levels of CA-125.

Giant iliac artery aneurysm--a rare cause of hydronephrosis.

The presence of arterial aneurysm--an abnormal dilatation of an arterial segment due to various causes--might lead to compression effects and to various symptoms. Finding an etiology for unilateral hydronephrosis represents in some cases a real challenge for the clinician and targeted investigations must be ordered for the diagnostic approach. The abdominal ultrasound examination is one of the first imaging modalities but the abdominal computed tomography is helpful for definitive conclusions. We present a rare case of unilateral hydronephrosis due to a giant left iliac artery aneurysm in a 77-year-old male with history of peripheral artery disease.

Polycystic hepatic disease.

Polycystic hepatic disease is a very rare hereditary disease. Most of the patients have no symptoms but sometimes they present abdominal discomfort, fever, jaundice. Almost half of the patients associate renal cysts. Rarely, the cysts may appear in other organs such as the pancreas, the spleen or the lung. We present a case of polycystic hepatic disease. In our patient the cysts replace almost the entire hepatic structure. However, the hepatic functional tests were normal.

A rare case of pulmonary emphysema.

Alpha-1-antitrypsin deficiency (AAT) is one of the three most common lethal genetic diseases in the caucasian population (together with cystic fibrosis and Down syndrome). Its primary manifestation is early-onset panacinar emphysema. Slowly progressive dyspnea is the primary symptom, although some patients initially have symptoms of cough, sputum production, or wheezing. A minority of patients develops hepatic cirrhosis. We present a case of a 40 year-old male, light smoker, with chronic obstructive lung disease with predominance of panacinar emphysema, with AAT deficiency (72 mg/dl; normal values = 200-300 mg/dl) complicated with cor pulmonale and chronic respiratory failure. The main clinical consequence of AAT deficiency is the early onset of panacinar emphysema, typically more severe at the lung bases. Smoking plays an important part in the natural history of the disease, both increasing the severity and decreasing the age at onset of emphysema.