[Plasma proteomic analysis in children with infectious mononucleosis].

OBJECTIVE: To explore the abnormal expression of plasma proteins by analysis of proteomic expression profile in children with infectious mononucleosis (IM). METHODS: Two dimensional gel electrophoresis (2-DE) followed by the mass spectrometry was used to examine important protein spots with different expression levels between children with IM and normal controls. RESULTS: Seven differential proteins were obtained: hemopexin, vitamin D binding protein, fetuin A, C-reactive protein, apolipoprotein A, haptoglobin and transthyretin. Compared with the control group, haptoglobin showed a higher expression level in children with IM, and the expression levels of the other proteins were obviously down-regulated. CONCLUSIONS: The expression changes of differential proteins identified in this study are all related with the liver acute injury, suggesting that children with IM are associated with acute liver injury. Further studies on the characteristics of above proteins will contribute to the diagnosis and treatment of pediatric IM.

CARD11-BCL10-MALT1 complex-dependent MALT1 activation facilitates myocardial oxidative stress in doxorubicin-treated mice via enhancing k48-linked ubiquitination of Nrf2.

AIMS: Down-regulation of nuclear factor erythroid 2-related factor 2 (Nrf2) contributes to doxorubicin (DOX)-induced myocardial oxidative stress, and inhibition of mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1) increased Nrf2 protein level in rat heart suffered ischemia/reperfusion, indicating a connection between MALT1 and Nrf2. This study aims to explore the role of MALT1 in DOX-induced myocardial oxidative stress and the underlying mechanisms. RESULTS: The mice received a single injection of DOX (15 mg/kg, i.p.) to induce myocardial oxidative stress, evidenced by increases in the levels of reactive oxidative species while decreases in the activities of anti-oxidative enzymes, concomitant with a down-regulation of Nrf2; these phenomena were reversed by MALT1 inhibitor. Similar phenomena were observed in DOX-induced oxidative stress in cardiomyocytes. Mechanistically, knockdown or inhibition of MALT1 notably attenuated the interaction between Nrf2 and MALT1, and decreased the k48-linked ubiquitination of Nrf2. Furthermore, inhibition or knockdown of calcium/calmodulin-dependent protein kinase II (CaMKII-δ) reduced the phosphorylation of caspase recruitment domain-containing protein 11 (CARD11), and subsequently disrupted the assembly of CARD11, B-cell lymphoma 10 (BCL10) and MALT1 (CBM) complex, and reduced the MALT1-dependent k48-linked ubiquitination of Nrf2 in DOX-treated mice or cardiomyocytes. INNOVATION AND CONCLUSION: The E3 ubiquitin ligase function of MALT1 accounts for the down-regulation of Nrf2 and aggravation of myocardial oxidative stress in DOX-treated mice, and CaMKII-δ-dependent phosphorylation of CARD11 triggered the assembly of CBM complex and subsequent activation of MALT1.

[Clinical characteristic analysis of 96 cases of hypersensitivity pneumonitis].

OBJECTIVE: To improve understanding of the clinical characteristics and diagnosis of hypersensitivity pneumonitis (HP). METHODS: We retrospectively analyzed the clinical data, including clinical symptoms, laboratory tests, exposure, pulmonary function tests, chest CT imaging and cytological classification of bronchoalveolar lavage (BAL) of 96 patients with HP from Jan 2001 to Jun 2011 in Peking Union Medical College Hospital. We divided the patients into 2 groups: a pathologically-confirmed group and a clinically-suspected group. RESULTS: There were 58 females and 41 males. The median age at the diagnosis was 53 years. The most common exposures were low-molecular-weight chemicals (42.7%) and animal proteins (37.5%). Common clinical symptoms included dyspnea on exertion (90.6%) and cough (76.0%). Pulmonary function test showed diffusion abnormality (73.5%) and restrictive ventilatory impairment (59.7%). Chest CT scan revealed patchy or diffuse bilateral ground-glass opacities (64.6%), centrilobular nodules (21.9%), and air trapping (15.6%). Reticulation (45.8%), traction bronchiectasis (21.9%) and honeycombing(9.4%) were present in chronic HP. BAL lymphocyte counts > 0.2 and CD4/CD8 < 0.9 were more commonly seen in patients with a disease course of less than 1 year. The pathologically-confirmed group and the clinically-suspected group shared many similar characteristics including age at diagnosis, gender, clinical manifestation, pulmonary function impairments and imaging findings, but significant differences existed in certain parameters. In the pathologically- confirmed group, the duration of disease was longer (24 months vs 6 months, Z = -2.492, P = 0.013) and clubbed fingers were more common (23.4% vs 8.2%, χ(2) = 4.227, P = 0.040). Diffusion abnormality was present in more patients of this group (90.7% vs 44.0%, χ(2) = 35.219, P < 0.01). By CT scan, reticulation, traction bronchiectasis and honeycombing (57.5% vs 26.5%, χ(2) = 9.434, P < 0.01) were more evident as compared to the clinically-suspected group. The value of transbronchial lung biopsy for diagnosing HP was limited, with a positive result of only 8.2%. Surgical lung biopsy was needed in uncertain cases. CONCLUSION: The diagnosis of HP was difficult. In some cases a clinical diagnosis can be made by combination of history of exposure, CT manifestations and cell classification of BAL. For atypical cases a multi-disciplinary approach including pathologists, radiologists and pulmonologists is needed.

Cochinchina momordica seed extract induces apoptosis and cell cycle arrest in human gastric cancer cells via PARP and p53 signal pathways.

Cochinchina momordica seed is the dried ripe seed of Momordica cochinchinensis (Lour.) Spreng, which is a kind of fruit and consumed for dietary as well as medicinal uses. In this study, using the human SGC7901 and MKN-28 gastric cancer cell lines, we explored the anticancer activity of the extract from cochinchina momordica seed (ECMS). ECMS inhibited significantly the survival rates of SGC7901 and MKN-28 cells in concentration- and time-dependent manners by MTT assay. The typical apoptotic morphological changes were observed by Hoechst 33258 dye assay after SGC7901 and MKN-28 cells were treated with ECMS for 48 h. Flow cytometry analysis revealed that ECMS-treatment blocked the cells at the S phase of cell cycle. Furthermore, the protein expression levels of poly (ADP-ribose) polymerase (PARP) and Bcl-2 were downregulated notably by ECMS-treatment, whereas those of Fas/Fas-associated death domain, p53, and Bax were upregulated in SGC7901 cells. ECMS dramatically enhanced the enzymatic activities of caspase-3 and caspase-9 whilst slightly increased caspase-8 activity. Taken together, this study demonstrated that ECMS exerted cytotoxic activities via PARP and p53 signal pathways in the human gastric cancer cells.

A case of diffuse panbronchiolitis complicated by peripheral T cell lymphoma not otherwise specified.

We report a case of diffuse panbronchiolitis (DPB) complicated by peripheral T cell lymphoma not otherwise specified. A 40-year-old Chinese man presented with intermittent fever, cough and significant white sputum production for more than 9 years, in addition to dyspnea and chest congestion that worsened after exercise. A chest CT scan indicated diffuse centrilobular fine nodular opacities with a 'tree-in-bud' appearance in both lungs. An open-lung biopsy was performed, and DPB was diagnosed by histopathological analysis. Three months later, the patient's pulmonary symptoms worsened. A chest CT of both lungs revealed multiple patchy opacities as well as enlargement of the hilar, mediastinal and multiple superficial lymph nodes. A whole-body bone scan revealed multiple osteolytic lesions located in the thoracic, lumbar and sacral spine. A biopsy of the right supraclavicular lymph node was performed, and peripheral T cell lymphoma not otherwise specified was diagnosed histopathologically. Cases of DPB complicated by non-Hodgkin's lymphoma are a rare occurrence. To our knowledge, there is only one earlier report of such a case in the literature (in Japanese). However, the prevalence of DPB complicated by T cell tumors is relatively high, indicating a possible association in pathogenesis of T cell disorders and DPB.

[Pulmonary neuroendocrine cell hyperplasia and tumorlets in bronchiectasis: a clinicopathologic study of 22 cases with review of literature].

OBJECTIVE: To study the clinical and pathological features of pulmonary neuroendocrine cell hyperplasia and tumorlets with bronchiectasis. METHODS: Both the clinicopathologic changes and immunohistochemical findings were examined with microscopy and EnVision method in 22 cases of pulmonary neuroendocrine cell hyperplasia and tumorlets. RESULTS: The average age of the 22 patients was 53 years, with a male to female ratio of 9:13. On macroscopic examination the lungs showed bronchiectasis; one case was accompanied by gray-white, soft nodules (diameter < 5 mm). Microscopy of the HE sections showed the basic pathologic change was bronchiectasis, accompanied by neuroendocrine cell hyperplasia and tumorlet formation in the pulmonary parenchyma surrounding the bronchioles, presenting as single nodule (10 patients), or multifocal nodules (12 patients), with average size of 1.6 mm in diameter. No tumor cells were identified in the lymph nodes. Sixteen of 22 patients were disease-free after an average follow-up period of 58 months (17 - 117 months); one patient died suddenly after surgery; and five were loss of follow up. Immunohistologically, the tumor cells were positive for CgA (18/18), Syn (16/16), AE1/AE3 (16/16) , TTF-1 (14/15), and CD56 (14/14), and Ki-67 index was < 2% in 12 cases. CONCLUSIONS: Immunohistological staining for CgA, Syn, CD56, TTF-1 and AE1/AE3 can confirm the diagnosis. Early detection, pulmonary resection and follow-up help prevent the progression of these diseases.

[The clinicopathological analysis of 4 cases of IgG4-related nonspecific interstitial pneumonia].

OBJECTIVES: To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP. METHODS: Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010. The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin. Sections were cut for HE and immunohistochemical stain. According to the diagnostic criteria for IgG4-related disease, 4 cases were confirmed to be IgG4-related NSIP. The clinicopathological features including clinical history, laboratory examination, and pathologic evaluation were studied. RESULTS: The 4 patients with IgG4-related NSIP included 1 man and 3 women, with a median age of 48 years (range, 44 - 56 years). The presenting symptoms were dry cough or shortness of breath. One patient (1/4, 25.0%) was found to have a positive autoantibody but no cases showed positive RF in serum. The histological finding of the 4 cases was characterized by inflammatory cell infiltration in interstitium with fibrosis, and 1 case showed obliterative arteritis. The numbers of IgG4-positive plasma cells in the 4 cases were 42/hpf, 22/hpf, 11/hpf, and 33/hpf respectively, while the percentages of IgG4-positive to IgG-positive plasma cells were 70%, 71%, 57%, 43% respectively. CONCLUSIONS: IgG4-related interstitial pulmonary disease can be characterized as the NSIP pattern. The pathological features of IgG4-related NSIP include infiltration of lympho-plasmacytes and eosinophils in interstitium with fibrosis, and lymphoid follicles are frequently identified in the area of lymphocyte aggregation, but obliterative arteritis is infrequently identified in the lesion. Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease.

[The diagnostic value of CT-guided percutaneous needle lung biopsy in diffuse parenchymal lung diseases].

OBJECTIVE: This study was to evaluate the efficacy and limitation of CT-guided percutaneous cutting needle lung biopsy in the diagnosis of diffuse parenchymal lung diseases (DPLD). METHODS: A total of 481 patients admitted in Peking Union Medical College Hospital from January 2000 to December 2008 underwent CT-guided percutaneous cutting needle lung biopsy. The patients were evaluated by clinical history, physical examination and lung HRCT. Those with localized opacity or lesions in a single lung in the CT scan were excluded. Finally, 248 patients with DPLD in HRCT were enrolled for this study. RESULTS: The study patients included 114 males and 134 females, and the mean (± SD) age at diagnosis was 50 ± 16 (range from 13 - 78) years. Confirmed diagnosis by percutaneous needle lung biopsy was obtained in 130 patients (52.4%), including pulmonary infection (35.4%, 46/130), pulmonary malignant diseases (25.4%, 33/130), bronchiolitis obliterans organizing pneumonia/organizing pneumonia (22.3%, 29/130), pulmonary vasculitis (6.2%, 8/130), granulomatous lesions (4.6%, 6/130), pulmonary sarcoidosis (2.3%, 3/130), acute interstitial pneumonia (1.5%, 2/130), pulmonary amyloidosis (1.5%, 2/130), and pulmonary alveolar proteinosis (0.8%, 1/130). Open lung biopsy/video-assisted thoracoscopic surgery was performed in 37 out of 118 cases for which the diagnosis was undetermined by percutaneous lung biopsy. Confirmed diagnosis was obtained in 36 patients, including non-specific interstitial pneumonia (NSIP, 33.3%, 12/36), usual interstitial pneumonia (UIP, 8.3%, 3/36), pulmonary infection (16.7%, 6/36), neoplasm (8.3%, 3/36), lymphoid interstitial pneumonia, pulmonary vasculitis (5.6% 2/36), hypersensitivity pneumonitis (5.6%, 2/36), and pulmonary sarcoidosis, allergic bronchopulmonary aspergillosis, pulmonary hyalinizing granuloma, pneumoconiosis, Castleman's disease, and lymphoproliferative disorder (1 case respectively). CONCLUSION: CT-guided percutaneous cutting needle lung biopsy can provide confirmed diagnosis in half of patients with DPLD, and has a high diagnostic yield in patients with infectious or neoplastic diseases, but it is not a good method for diagnosis of interstitial lung diseases such as NSIP and UIP.

Antidiarrheal activity of the extracts of Valeriana jatamansi Jones on castor oil-induced diarrhea mouse by regulating multiple signal pathways.

ETHNOPHARMACOLOGICAL RELEVANCE: Valeriana jatamansi Jones, a traditional medicine, is used for various medicinal purposes worldwide. This species is popular for its gastro-protective properties and has been verified to exert antidiarrheal effects. Qiuxieling mixture, an oral liquid preparation used to treat diarrhea in children in clinical practice, was extracted from V. jatamansi Jones. AIM OF THE STUDY: Although Qiuxieling mixture has a good preventive effect on diarrhea children, the disgusting smell makes it intolerable. Therefore, we extracted odorless products from V. jatamansi Jones and Qiuxieling mixture. The present study is aimed to investigate the protective effects of two ethanolic extracts of V. jatamansi Jones and Qiuxieling mixture against castor oil-induced diarrhea and their possible mechanisms in mice. MATERIALS AND METHODS: The two extracts of V. jatamansi Jones and Qiuxieling mixture were detected by HPLC. A castor oil-induced diarrheal model was used to evaluate the antidiarrheal effects. The expression of Occludin in the small intestine was measured by IHC. Western blotting and immunofluorescence were used to detect the expression of proteins related to the oxidative stress and GSDMD-mediated pyroptosis signaling pathways. ELISA was used to detect the expression of IL-6 and IL-1ß in the small intestine of mice with diarrhea. RESULTS: The two extracts of V. jatamansi Jones and Qiuxieling mixture dose-dependently reduced the diarrhea index and the diarrhea rate, delayed the onset of diarrhea, and decreased the weight of the intestinal content. Meanwhile, they reversed the decreased expression of Occludin and restored the activity of Na+-K+-ATPase in the intestines of diarrheal mice. In addition, they reversed the depletion of GSH, attenuated the activation of the ERK/JNK pathway, promoted the Nrf2/SOD1 signaling pathways, and decreased the release of ROS in the intestines of diarrheal mice. Moreover, they suppressed GSDMD-mediated pyroptosis by downregulating the NLRP3/caspase-1/GSDMD signaling pathway. CONCLUSIONS: The two extracts of V. jatamansi Jones and Qiuxieling mixture exerted protective effects on castor oil-induced diarrhea in mice through a variety of mechanisms, including antioxidant stress, restoration of tight junctions between intestinal mucosal cells and regulation of the GSDMD-mediated pyroptosis pathway.

Pathological and high resolution CT findings in Churg-Strauss syndrome.

OBJECTIVE: To investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis. METHODS: We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well. RESULTS: The patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months). CONCLUSIONS: Asthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.

[Pulmonary lymphomatoid granulomatosis: an immunohistochemical and gene rearrangement study].

OBJECTIVE: To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis. METHODS: Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies. RESULTS: The age of patients ranged from 3 to 59 years. The male-to-female ratio was 3: 6. Histologically, all cases showed lymphocytic infiltration surrounding the blood vessels and in the perivascular areas. Most of these lymphoid cells expressed T-cell marker CD3. There were also variable numbers of CD20-positive B cells. The staining for CD56 was negative. According to the WHO classification, there were 4 cases of grade I , 1 case of grade II and 4 cases of grade III lesions. Six cases had gene rearrangement studies performed and 3 of them demonstrated clonal immunoglobulin gene rearrangement (including 1 of the grade II and 2 of the grade III lesions). No T-cell receptor gene rearrangement was detected. CONCLUSIONS: Pulmonary lymphomatoid granulomatosis may represent a heterogeneous group of lymphoproliferative disorders. Some of the cases show B-cell immunophenotype and clonal immunoglobulin gene rearrangement, especially the grade II and grade lesions. They are likely of lymphomatous nature.

[Non-Hodgkin's lymphoma with diffuse ground-glass opacity on chest CT: a report of 6 cases].

OBJECTIVE: To investigate the clinical, pathological and imaging characteristics, misdiagnosis and treatment of pulmonary non-Hodgkin's lymphoma with diffuse ground-glass opacities (GGO). METHODS: Six cases of pulmonary non-Hodgkin's lymphoma with diffuse GGO on chest CT diagnosed from January 2008 to March 2010 were retrospectively analyzed. RESULTS: There were 5 males and 1 female with average age of 52 years old (range: 30-59). The course had a range of 2-36 months. Most patients presented with dyspnea (n=5) and loss of weight (n=5). Enlargement of superficial lymph nodes (n=2) and hepatosplenomegaly (n=2) were also found. Laboratory tests showed that average hemoglobin decreased to 25 g/L and average serum LDH was 755 U/L. Chest CT showed diffuse GGO (n=2), diffuse GGO with consolidations (n=3), with wide lung septum (n=3), with multiple nodules (n=2), with enlargement of mediastinal lymph nodes (n=2). Diagnosis of the 6 cases were made by lung biopsy. Histological findings including intravascular lymphoma (n=2), diffuse large B cell lymphoma (n=2) and T cell lymphoma (n=2). The average follow-up period was 4 months (range: 2-6). Chemotherapy was administered in 4 patients with B cell lymphoma and all of them improved or remained stable. One patient with T cells lymphoma was lost to follow-up and another patient with T cell lymphoma died due to lung infection. CONCLUSIONS: Non-Hodgkin's lymphoma with diffuse GGO on chest CT scan is rare. And its misdiagnosis is common due to a lack of specific clinical manifestations. And lung biopsy is necessary for an early diagnosis.

S-propargyl-cysteine protects both adult rat hearts and neonatal cardiomyocytes from ischemia/hypoxia injury: the contribution of the hydrogen sulfide-mediated pathway.

In this study, we determined the cardioprotective effects of S-propargyl-cysteine (SPRC), a structural analog of S-allylcysteine (SAC), using in vivo models of acute myocardial infarction (MI) and in vitro hypoxic cardiomyocytes models. MI was created in rats by ligating the left anterior descending coronary artery. Plasma enzymes levels and cystathionine-gamma-lyase (CSE) activities were determined. Primary cultures of newborn rats' cardiomyocytes were injured by hypoxia for 6 h. Cell viabilities were measured with the thiazolyl blue assay. RT-PCR and western blot analysis revealed the expression of CSE in both models. The protective effects of SPRC were associated with an observed reduction in infarct size (20.8 +/- 2.4% vs. 36.0 +/- 1.3%), decreased plasma enzymes levels and reduced malondialdehyde levels when compared to the MI vehicle group (P < 0.05); cardiac function was also improved. SPRC increased CSE activity and plasma H2S concentration by 1.6-fold and 1.3-fold, respectively, in MI rats. Decreased cell viability (64.5 +/- 5.4%) in hypoxic cardiomyocytes could be rescued with use of SPRC (81.0 +/- 3.1%). Similarly, mRNA and protein expression of CSE were upregulated in the SPRC group. Treatment with the CSE inhibitor propargylglycine abolished the protective effects of SPRC. Our study provides novel evidence that SPRC is protective in myocardial infarctions via a H2S-related pathway.

Pulmonary manifestations of Sjögren's syndrome.

BACKGROUND: Primary Sjögren's syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. OBJECTIVES: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. METHODS: Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. RESULTS: The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. CONCLUSIONS: The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.

[Pathologic study of diffuse pulmonary interstitial fibrosis caused by chronic hypersensitivity pneumonitis].

OBJECTIVE: To study the pathologic characteristics of chronic hypersensitivity pneumonitis, especially the pattern of pulmonary interstitial fibrosis; and to compare the histologic features with those of idiopathic interstitial pneumonitis. METHODS: The HE-stained paraffin sections of 10 cases of chronic hypersensitivity pneumonitis encountered during the period from 2000 to 2008 were retrospectively analyzed. RESULTS: There were altogether 6 males and 4 females, with age of patients ranging from 23 to 59 years (mean=47.2 years). Clinically, the patients presented with chronic cough and shortness of breath for 4 months to 6 years. Histologically, 7 cases showed usual interstitial pneumonitis (UIP)-like fibrosis. Patchy fibrosis was observed under the pleura, adjacent to interlobular septa and around bronchioles. In all of the 7 cases, foci of fibroblastic proliferation, as well as bronchiolar metaplasia of peribronchiolar alveoli and mild bronchiolitis, were noted. Three cases presented with mild honeycomb changes of lung and 3 cases showed non-specific interstitial pneumonitis (NSIP)-like fibrosis, in which the alveolar septa were expanded by fibrous tissue and collagen, with relative preservation of alveolar architecture. Bronchiolitis and lymphocytic infiltrates in alveolar septa were seen. Schaumann bodies were identified in 1 case. In general, patients with chronic hypersensitivity pneumonitis were younger than patients with idiopathic UIP. Computed tomography often showed upper and middle lobar involvement and mosaic attenuation. Compared with idiopathic UIP, the UIP-like fibrosis of chronic hypersensitivity pneumonitis often occurred not only under the pleura and adjacent to interlobular septa, but also around bronchioles and was accompanied by bronchiolar metaplasia. CONCLUSIONS: Chronic hypersensitivity pneumonitis can mimic other types of lung conditions with interstitial fibrosis, especially UIP and NSIP. As a result, some cases of chronic hypersensitivity pneumonitis may be misdiagnosed as such.

[Intravascular lymphomatosis presenting in the lung].

OBJECTIVE: To investigate the clinical, radiographic and pathological characteristics of intravascular lymphomatosis primarily manifested in the lung, without skin and central nervous system involvements. METHODS: A case of T cell intravascular lymphomatosis presenting with fever and multiple pulmonary shadows on chest radiograph was described and 14 similar cases reported in the English literature were reviewed. RESULTS: We described a case of T cell intravascular lymphomatosis, who was a 36 year old man, complained of fever and multiple pulmonary shadows on chest radiograph and admitted to Peking Union Medical College Hospital in march, 2008. Open lung biopsy showed features characteristic of intravascular lymphomatosis. He received CHOP chemotherapy, but died 20 days after diagnosis. Most cases of intravascular lymphomatosis primarily manifested in the lung occurred in older patients, ranging from 36 to 79 years of age (mean 59 years), with a male predominance (M : F = 11 : 4). The chief complaints were dyspnoea (10/15), cough (9/15), fever (5/15) and weight loss (5/15). Pulmonary function tests usually revealed some degree of decreased diffusing capacity. Eight cases had high serum lactate dehydrogenase levels. Chest CT scan showed multiple reticular or/and nodular density. Immunophenotypically, 10 cases were B cell lineage, 3 cases were T cell lineage. Six cases of B cell intravascular lymphomatosis were followed, of whom 4 and were alive, and 2 died of respiratory failure. Three cases of T cell intravascular lymphomatosis showed poor prognosis, both of whom died of respiratory failure. CONCLUSIONS: Intravascular lymphomatosis primarily manifested in the lung is a rare malignant disease. Its pulmonary symptoms were nonspecific, and pulmonary function tests and chest CT scan manifested as those of interstitial pneumonia or pulmonary infection. It can be pathologically diagnosed through transbronchial lung biopsy or surgical lung biopsy.

[Clinical and radiological features of pulmonary tuberculosis manifested as interstitial lung diseases.].

OBJECTIVE: The purpose of this paper was to investigate the clinical and radiological features of pulmonary tuberculosis presenting as interstitial lung diseases (ILD). METHODS: We analyzed the data of cases suspected of diffuse parenchyma lung diseases at this hospital between October 2003 and October 2007. The diagnosis of active pulmonary tuberculosis was based on epithelioid granuloma or positive acid-fast bacilli in lung biopsy and changes on serial radiographs obtained during treatment. RESULTS: The data of a series of 230 consecutive patients with suspected ILD were retrospectively analyzed. The diagnosis was confirmed by lung biopsy. Twelve patients were confirmed to have pulmonary tuberculosis. There were 5 males and 7 females with a mean age of 38 +/- 11 years (range, 17 - 68). The median course of disease in these patients was 3 months (range, 0.5 - 18 months). Patients with pulmonary tuberculosis presented with fever (11/12), cough (9/12), weight loss (7/12), dyspnea (7/12), lymphadenopathy (4/12), and splenohepatomegaly (2/12). On chest CT scan, ground-glass attenuation was identified in 4, bilateral patchy infiltration in 5, tree-in-bud appearance 1, and centrilobular lesions in 2 of the 12 patients. During the follow-up period (median, 9 month, range from 3 to 12 month), 11 patients improved, but 1 died of diabetic ketoacidosis. CONCLUSION: The diagnosis of pulmonary tuberculosis should be considered in suspected ILD patients presenting with fever, splenohepatomegaly and lymphadenopathy.

[Clinicopathologic study of Churg-Strauss syndrome].

OBJECTIVE: To study the clinical and pathologic features of Churg-Strauss syndrome (CCS). METHODS: Three cases of Churg-Strauss syndrome, including 1 autopsy case and 2 cases with open thoracoscopic lung biopsy, were retrospectively reviewed. All the tissue samples were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. RESULTS: The first patient was a 68-year-old man who had history of asthma for 4 years, with recent exacerbation and chest pain for 2 weeks. Patient died 1 day after admission due to myocarditis and myocardial infarction. He did not have peripheral eosinophilia, skin or paranasal sinus pathology. CSS represented an incidental autopsy finding and he had never been treated with corticosteroid before. The other 2 patients were a 58-year-old male and a 12-year-old female, respectively. Both had history of asthma, peripheral eosinophilia and lung consolidations on computed tomographic examination. Pathologically, all cases showed vasculitis, perivascular allergic-type granulomas, eosinophilic pneumonia and asthmatic bronchitis. CONCLUSIONS: Thorough understanding of the clinical and pathologic criteria is essential for arriving at a correct diagnosis of CSS. Although some patients may present with atypical symptoms, lung biopsies often reveal the classic histologic findings which include vasculitis and perivascular allergic granuloma formation.

[Clinicopathologic study of primary marginal zone B-cell lymphoma of MALT type and lymphoid hyperplasia of lung].

OBJECTIVE: To study the clinicopathologic features, immunohistochemical findings and immunoglobulin heavy chain (IgH) gene rearrangement results of primary pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) and reactive lymphoid hyperplasia. METHODS: Twenty cases, included 13 cases of pulmonary MALToma and 7 cases of pulmonary lymphoid hyperplasia, encountered during the period from 1989 to 2007, were retrospectively analyzed. The samples were paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study and semi-nested polymerase chain reaction for IgH gene rearrangement were performed. RESULTS: The 13 cases of primary pulmonary MALToma were composed of a spectrum of lymphoid cells, including lymphocyte-like cells, centrocyte-like cells and mononuclear B cells with plasmacytoid differentiation. They often had diffuse or marginal zone growth patterns. Lymphoid follicles with neoplastic colonization were apparent. The lymphoma cells spread along alveolar septa and bronchovascular bundles. Vascular invasion was noted in 9 cases, pleura involvement in 6 cases and nodal involvement in 2 cases. Lymphoepithelial lesions (LEL) were identified in 9 cases of pulmonary MALToma. Immunohistochemically, the lymphocytes in LEL were CD20-positive and CD3-negative. On the other hand, LEL was also present in 2 of the 7 cases of lymphoid hyperplasia studied, with a mixture of CD20-positive B cells and CD3-negative T cells. Eight of the 9 cases of primary pulmonary MALToma were positive for IgH gene rearrangement, while all of the 7 cases of lymphoid hyperplasia were negative. CONCLUSIONS: Histologically, the cell population of primary pulmonary MALToma is similar to that of extranodal MALToma occurring in other organs. LEL, though commonly observed in pulmonary MALToma, are not specific and can also be seen in cases of reactive lymphoid hyperplasia. The immunophenotype of intraepithelial lymphocytes in pulmonary MALToma and reactive lymphoid hyperplasia is different. The presence of a monotonous population of CD20-positive intraepithelial lymphocytes supports a diagnosis of MALToma. IgH gene rearrangement study is also useful in differentiating both entities.

[Primary pulmonary lymphoma: analysis of 18 cases].

OBJECTIVE: To study the clinical characteristics, pathology, diagnosis and treatment of primary pulmonary lymphoma. METHODS: Eighteen cases of primary pulmonary lymphoma diagnosed from Jan 1989 to Feb 2007 were retrospectively analyzed. RESULTS: There were 6 males and 12 females, with a median age of 47.5 years (17-71 years). Fifteen cases were diagnosed by surgical lung biopsy; 1 by percutaneous needle lung biopsy (1/6), 1 by percutaneous needle lung biopsy and bronchoscopic examination at the same time, the other 1 by bronchoscopic examination (1/10). Histological diagnosis showed that 2 cases were Hodgkin lymphoma, 9 mucosa-associated lymphoid tissue lymphoma, 1 follicular lymphoma, 2 diffuse large B cell lymphoma 2 anaplastic large cell lymphoma, 2 non-Hodgkin lymphoma whichcould not be classified because the slides were from other hospitals. The most common symptoms were cough (9/18) and fever (6/18). ESR elevation was common (10/12). CT features included solitary or multiple nodules (14/18), patchy opacities (11/18), consolidations (5/18), pleural effusions (5/18), atelectasis (5/18), and cavities (1/18). Misdiagnosis was found in 11 patients. Treatment modalities included surgical resection, radiotherapy and chemotherapy. Median follow-up time was 11 months (10 d to 205 mon). Thirteen patients were still alive, 4 patients were lost, and 1 patient died. The prognosis was associated with the level of [25.1 x 10(9)/L(18.1 - 39. -1) x 10(9)/L in poor prognosis group, 6.7 x 10(9)/L (5.48 - 8.41) x 10(9)/L in good prognosis group, u = 0.000, P <0.05] leukocytosis (3/3 vs 1/10, P <0.05). CONCLUSIONS: The clinical manifestations of primary pulmonary lymphoma are nonspecific. Misdiagnosis is common. Surgical lung biopsy is necessary for early diagnosis.