An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage,intestinal perforation,cardiac failure and peripheral neuropathy

Churg–Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.     

Acute massıve pulmonary embolism assocıated wıth olanzapıne

Treatment with low-potency anti-psychotic agents is an important risk factor in the development of pulmonary embolism (PE). We report a case of 74 years old female patient receiving olanzapine for psychotic depression admitted to the emergency service with the complaints of chest pain and shortness of breath. She had tachypnea, hypotension and tachycardia. Arterial blood gas analysis showed hypoxemia-hypocapnia and D-dimer level was high. Computed tomographic pulmonary angiography (CTPA) demonstrated pulmonary embolism in both main pulmonary arteries, through lobar and segmental branches. Tissue plasminogen activator (t-PA) was administered in intensive care unit. As the only possible risk factor for PE was olanzapine, olanzapine treatment was terminated with pyschiatry consultation. During the 12-month follow-up of the patient; malignancy was not observed. Diagnosis and prevention of PE are the important goals to reduce morbidity and mortality in subjects receiving olanzapine.     

Therapy‐related B‐lymphoblastic leukemia associated with Philadelphia chromosome and MLL rearrangement: Single institution experience and the review of the literature

Therapy related acute lymphoblastic leukemia (t‐ALL) of B cell origin is rare and constitutes approximately 2% of all ALL. Previously compiled data on the complete cytogenetic analysis of 48 t‐B‐ALL cases suggested that MLL rearrangement at 11q23 gene locus is the most common abnormality. Philadelphia chromosome (Ph) and a normal karyotype were reported as the second and third most common karyotypes, respectively. We investigated cytogenetic karyotypes of six t‐B‐ALL cases with a pre‐B cell immunophenotype. Ph + t‐B‐ALL was noted in four of six patients previously treated with radiation and/or chemotherapy. In addition, one case demonstrated MLL rearrangement at 11q23 locus while one case demonstrated normal cytogenetic karyotype. Five of the six t‐B‐ALL patients had persistent leukemia following initiation of chemotherapy for secondary leukemia with survival ranging from 10 to 21 months. To our knowledge, only fourteen patients with Ph + t‐B‐ALL have been described in the literature. In the current study, three of four cases with Ph + t‐B‐ALL were associated with treated breast carcinoma while one patient was treated for Hodgkin lymphoma. All four patients had undergone radiation therapy. The results may indicate a plausible association between Ph+t‐B‐ALL and prior radiation exposure.     

Noninvasive central venous pressure measurement by controlled compression sonography at the forearm.

OBJECTIVES: We sought to validate a new noninvasive technique to determine central venous pressure (CVP) using high-resolution compression sonography. BACKGROUND: Information concerning CVP is crucial in clinical situations, including cardiac failure, volume overload, and sepsis. The measurement of CVP, however, requires puncture of a vein with attendant risk of complication. METHODS: After a proof-of-concept study in healthy subjects, a prospective blinded evaluation was performed comparing CVP measurement using a central venous catheter with measurement using compression sonography in critically ill (intensive care unit) patients. RESULTS: In healthy subjects with experimentally induced venous hypertension with a wide range of pressure values, a strong correlation (r = 0.95; p < 0.001) between noninvasive and invasive peripheral venous pressure at the forearm was shown. High interobserver agreement with an intraclass correlation coefficient of 0.988 shows excellent reliability of the system. Noninvasive peripheral venous pressure measurement at the forearm showed a good correlation with CVP in 50 intensive care unit patients with the forearm positioned both below heart level (r = 0.84; p < 0.001) and at heart level (r = 0.85; p < 0.001). The mean difference between invasive and noninvasive measurement was negligible (-0.1 +/- 3.5 cm H2O and -0.7 +/- 3.4 cm H2O, respectively). CONCLUSIONS: Controlled-compression sonography is a valuable tool for measuring venous pressure in peripheral veins and allows reliable indirect assessment of CVP without intravenous catheterization.     

Reactive oxygen species and chemokines： Are they elevated in the esophageal mucosa of children with gastroesophageal reflux disease？

AIM： To determine the role of inflammatory cytokines and reactive oxygen species （ROS） in childhood reflux esophagitis. METHODS： A total of 59 subjects who had complaints suggesting GERD underwent esophagogastroduoden oscopy. Endoscopic and histopathologic diagnosis of reflux esophagitis was established by Savary-Miller and Vandenplas grading systems, respectively. Esophageal biopsy specimens were taken from the esophagus 20% proximal above the esophagogastric junction for conventional histopathological examination and the measurements of ROS and cytokine levels. ROS were measured by chemiluminescence, whereas IL-8 and MCP-1 levels were determined with quantitative immunometric ELISA on esophageal tissue. Esophageal tissue ROS, IL-8 and MCP-1 levels were compared among groups with and without endoscopic/histo- pathologic esophagitis. RESULTS： Of 59 patients 28 （47.5%） had normal esophagus whereas 31 （52.5%） had endoscopic esophagitis. In histopathological evaluation, almost 73% of the cases had mild and 6.8% had moderate degree of esophagitis. When ROS and chemokine levels were compared among groups with and without endoscopic esophagitis, statistical difference could not be found between patients with and without esophagitis. Although the levels of ROS, IL-8 and MCP-1 were found to be higher in the group with histopathological reflux esophagitis, this difference was not statistically significant. CONCLUSION： These results suggest that the grade of esophagitis is usually mild or moderate during childhood and factors apart from ROS, IL-8 and MCP-1 may be involved in the pathogenesis of reflux esophagitis in children.