Blue rubber bleb nevus syndrome: a case report and literature review.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multiple venous malformations and hemangiomas in the skin and visceral organs. The lesions often involve the cutaneous and gastrointestinal systems. Other organs can also be involved, such as the central nervous system, liver, and muscles. The most common symptoms are gastrointestinal bleeding and secondary iron deficiency anemia. The syndrome may also present with severe complications such as rupture, intestinal torsion, and intussusception, and can even cause death. Cutaneous malformations are usually asymptomatic and do not require treatment. The treatment of gastrointestinal lesions is determined by the extent of intestinal involvement and severity of the disease. Most patients respond to supportive therapy, such as iron supplementation and blood transfusion. For more significant hemorrhages or severe complications, surgical resection, endoscopic sclerosis, and laser photocoagulation have been proposed. Here we present a case of BRBNS in a 45-year-old woman involving 16 sites including the scalp, eyelid, orbit, lip, tongue, face, back, upper and lower limbs, buttocks, root of neck, clavicle area, superior mediastinum, glottis, esophagus, colon, and anus, with secondary severe anemia. In addition, we summarize the epidemiology, clinical manifestations, diagnosis, differential diagnosis and therapies of this disease by analyzing all previously reported cases to enhance the awareness of this syndrome.

Successful esophagectomy in a patient with combined esophageal cancer and hemophilia B.

Patients with esophageal cancer often require esophagectomy with esophagogastrostomy. However, the incidence of complications, such as hemorrhage, during operations for esophageal cancer is high, even with minimally invasive surgery. Without the appropriate interventions, the risk of major intraoperative and postoperative hemorrhage is very high in patients with esophageal cancer and hemophilia. We report the case of a 45-year-old man with esophageal cancer and hemophilia B who underwent a successful hybrid, minimally invasive Ivor-Lewis esophagectomy with appropriate perioperative management.

[Evaluation of biochemical metabolites by 31P MR spectroscopy in leukemic infiltration of liver].

OBJECTIVE: To evaluate the changes of phosphorus metabolites in leukemic infiltration of liver (LIL) with two-dimensional chemical shift imaging(2D CSI)(31)phosphorus MR spectroscopy ((31)P MRS). METHODS: Fifteen patients with LIL and 12 healthy subjects (control group) were scanned with liver 2D CSI(31)P MRS by a 1.5T MR Scanner(Sonata, Siemens Corporation). Relative quantification of phosphorus metabolites including phosphomonoesters (PME), inorganic phosphate (Pi), phosphodiesters (PDE) and beta-adenosine- triphosphate (beta-ATP) were detected and after calibrated with model factor, the ratios of PME/PDE, PME/(PME+PDE), PME/ATP, PDE/ATP and Pi/ATP were analyzed. RESULTS: Compared with control group, the PME value, PME/PDE ratio, PME/(PME+PDE) ratio and PME/ATP ratio were increased in LIL group (1.992 +/-0.876 Compared with 1.167 +/-0.427, P <0.05), (0.551 +/-0.339 Compared with 0.254 +/-0.059,P <0.01), (0.326 +/-0.13 Compared with 0.199 +/-0.049, P <0.01)and (1.402 +/-0.654 Compared with 0.792 +/-0.232, P <0.01) respectively. CONCLUSION: (31)P MRS examination can be used as a non-invasive procedure to evaluate the changes of phosphorus metabolites of leukemic infiltration of liver. The increase of PME value and its ratios to PDE, ATP and PME+PDE may indicate leukemic infiltration of liver.

[Following-up of nosocomial lower respiratory infection in patients with hematological malignancy after chemotherapy].

OBJECTIVE: To observe and investigate the risk factors and pathogen diversification of nosocomial lower respiratory infections in patients with hematological malignancy after chemotherapy. METHODS: Respiratory tract microbial population of fifty patients with different kinds of hematological malignancy and para-prepared to chemotherapy was quantitatively analyzed before and after chemotherapy at an arranged time from April, 2004 to December, 2005. Susceptibility test was determined for bacterium of nosocomial infection, and the homology of the same species of the bacteria was analyzed by a pulsed field gel electrophoresis (PFGE). RESULTS: Incidence rate of lower respiratory infections in patients with the hematological malignant after chemotherapy was 16%. The major nosocomial infectious pathogens were Acinetobacter spp; Escherichia coil and Fungus. Among them, Acinetobacter spp, were highly resistant to cephalosporins, quinolones, aminoglycosides, carbapenems and antibiotic with enzyme inhibitor, respectively but susceptible to Cefoperazone/Sulbactam belonging to antibiotic with enzyme inhibitor. And it was shown that there were two clones by the pulsed field gel electrophoresis (PFGE). CONCLUSION: Following-up of nosocomial lower respiratory infection in patients with hematological malignancy after chemotherapy might offer theoretical evidence for the rational use of antibiotics and the control of nosocomial infections.

[Application of bone marrow biopsy imprint in evaluating cellularity].

OBJECTIVE: To study the value of bone marrow biopsy imprint in evaluating cellularity. METHODS: The bone marrow tissues were obtained by trephine biopsy from 272 patients, and then put on the slides to make the imprints. The imprints was stained by Wright-Giemsa method, and the bone marrow smears and imprints were examined simultaneously according to the bone marrow cellularity criteria. RESULT: In bone marrow cellularity, four grades (distinct decrease, extreme decrease, distinct increase, and extreme increase) were significantly higher in bone marrow imprints than those in bone marrow smears (P <0.05), but there was no significantly differences between bone marrow imprints and sections (P >0.05). Using bone marrow sections as standard, in cellularily decreasing samples, the consistent rate of bone marrow imprints and smears were both high (84.4% and 97.9%), in the group of the normal and increased cellularity, the consistent rate of the bone marrow imprints (84.4% and 97.7%) was significantly higher than that in smears (60% and 64%, P<0.01). The sensitivity, specificity, Youden index, positive predictive value and positive likelihood rate of bone marrow imprints were all higher than those of the smears. Using the bone marrow sections as gold standard, in 124 cases with decreased cellularity in smears, the positive diagnosis rate for aplastic anemia and dyshaematopoiesis based on bone marrow imprints was 37.1% with a false positive rate of 7.3% which was lower than that of the bone marrow smears (false positive rate of 29.8%, P<0.01). CONCLUSION: To evaluate bone marrow cellularity, bone marrow imprint is better than bone marrow smear. The combination of the two examinations can make the diagnosis more convenient and quicker.

[The clinical significance of hematological damage in systemic lupus erythematosus and related antibodies].

OBJECTIVE: To investigate the incidence of hematological damage in systemic lupus erythematosus (SLE) and its relationship with disease state and immunological parameters of SLE. METHODS: 155 inpatients with SLE were enrolled in this retrospective study in our hospital. All patients fulfilled the criteria of SLE drawn up by American Rheumatism Association (ARA) in 1982. Fasting blood samples were obtained and blood routine, liver and renal function, immunological parameters were determined. According to SLE Disease Activity Index (SLEDAI), disease state was evaluated in 155 patients and their correlation parameters were analyzed, with hematological damage and immunological parameters analyzed. RESULTS: 67.7% of the patients had hematological disorder. Single cell line damage was present in 32.9% of the patients, while 34.8% of the patients were involved in damage of several cell lines. The detectable rate of Anti-Sm (43.8%) and Anti-RNP (64.8%) in patients with hematological disorder were higher than those in patients without hematological disorder (Anti-Sm 26.0%, Anti-RNP 44.0%). The positive rate of Anti-RNP were 69.0% in patients with red cell line damage while only 44.0% in patients without hematological disorder (P < 0.05). There was no significant difference in Anti-RNP between patients without hematological disorder and patients with leukocytopenia or thrombocytopenia. Among the 155 patients 49 were in progressive stage and 106 in stationary stage. Hematological damage occurred approximately in 75.5% of the patients in progressive stage and in 64.2% of the patients in stationary stage. The prevalences of Anti-dsDNA, Anti-RNP, Anti-Sm and several cell lines damage were significantly higher in patients with progressive stage than in patients with stationary stage. CONCLUSIONS: Hematological system is easily damaged in patients with SLE and its involvement is related with the disease state. Anti-RNP could reflect red cell line damage.