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1.
We report a case of a 62-year old woman admitted to our hospital for multiple nodular metastatic liver lesions found by ultrasonography in a regular medical examination. Routine laboratory tests were normal. PET-CT showed multiple bone lesions and nodular liver lesions. Liver biopsy revealed nodular infiltration of multiple myeloma with positive staining of kappa light chain. Further investigation of bone marrow aspiration, immunofixation and immunoelectrophoresis of serum protein, urine test for Bence-Jones protein, 132-microglobulin in serum and urine confirmed the diagnosis. The patient also coinfected with hepatitis C virus (HCV). With six cycles of chemotherapy with VAD schedule, she achieved complete remission. In this report, a literature review of liver lesions involving multiple myeloma is also provided.  相似文献   

2.
Acute pancreatitis as an initial symptom of systemic lupus erythematosus (SLE) is rare. We present a report of a 46-year-old female patient who had fever, abdominal pain and vomiting, elevated pancreatic enzyme levels, hypocalcemia, hypoxemia, and various other laboratory abnormalities. She was first diagnosed with acute severe pancreatitis and then with SLE after further investigations. After a 2-mo treatment with somatostatin, the patient recovered.  相似文献   

3.
An association between chronic hepatitis C virus (HCV) infection and essential mixed cryoglobulinaemia and non-Hodgkin lymphoma (NHL) has been suggested. However, a causative role of HCV in these conditions has not been established. The authors report a case of a 50 year-old woman with chronic hepatitis C (CHC) who has been followed up since 1998 due to a high viral load, genotype 1b and moderately elevated liver function tests (LFTs). Laboratory data and liver biopsy revealed moderate activity (grade: 5/18, stage: 1/6). In April 1999, one-year interferon therapy was started. HCV-RNA became negative with normalization of LFTs. However, the patient relapsed during treatment. In September 2002, the patient was admitted for chronic back pain. A CT examination demonstrated degenerative changes. In March 2003, multiple myeloma was diagnosed (IgG-kappa, bone marrow biopsy: 50% plasma cell infiltration). MRI revealed a compression fracture of the 5th lumbar vertebral body and an abdominal mass in the right lower quadrant, infiltrating the canalis spinalis. Treatment with vincristine, adriamycin and dexamethasone (VAD) was started and bisphosphonate was administered regularly. In January 2004, after six cycles of VAD therapy, the multiple myeloma regressed. Thalidomide, as a second line treatment of refractory multiple myeloma (MM) was initiated, and followed by peginterferon-a2b and ribavirin against the HCV infection in June. In June 2005, LFTs returned to normal, while HCV-RNA was negative, demonstrating an end of treatment response. Although a pathogenic role of HCV infection in malignant lymphoproliferative disorders has not been established, NHL and possibly MM may develop in CHC patients, supporting a role of a complex follow-up in these patients.  相似文献   

4.
Abdominal pain observed in Henoch-Sch?nlein purpura (HSP) is usually attributed to edema and hemorrhage in the small bowel wall, secondary to a small-vessel vasculitis. Pancreatitis secondary to HSP is extremely rare. Here we report a 53-year-old man presented with acute pancreatitis that developed into characteristic rashes seen during HSP at the second day of the clinical onset, together with arthritis and glomerulonephritis. HSP is a rare and benign cause of acute pancreatitis. This complication can occur as an initial manifestation of HSP. Elevated serum amylase level can be considered as the early diagnostic tool for HSP pancreatitis. The patients with HSP who have abdominal pain as their chief complaint should be evaluated for pancreatitis, by routine serum amylase and abdominal computed tomography scan, to plan the specific treatment and avoid unnecessary surgery.  相似文献   

5.
Adult-onset Still’s disease is a multisystem inflammatory disorder of unknown etiology and is characterized by high, spiking fever, arthritis, evanescent maculopapular rash, myalgia, serositis, leukocytosis, and involvement of various organs including the eyes. The ocular manifestations have been described including orbital pseudotumor, ptosis, and diplopia with orbital pain but never Purtscher’s-like retinopathy. We describe a 21-year-old male patient with adult-onset Still’s disease who developed the Purtscher’s-like retinopathy. To our knowledge, this is the first reported adult-onset Still’s disease patient with Purtscher’s-like retinopathy as the initial presentation.  相似文献   

6.
Zhang  Bingqing  Sun  Yang  Xu  Na  Wang  Wei  Huang  Xiaoming  Chen  Jialin  Shen  Min  Wang  Rongrong  Zeng  Xuejun  Zhang  Xue 《Clinical rheumatology》2021,40(10):4325-4339
Clinical Rheumatology - Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive disease caused by ADA2 gene mutation that is characterized by three phenotype domains: vasculopathy and...  相似文献   

7.
Autoimmune disorder is one of the important side effects of interferon-α therapy. Some polymyositis cases as complication of interferon-α therapy were reported, but dermatomyositis were rarely. We report a case of dermatomyositis as a complication of interferon-α therapy for hepatitis C. A 52-year-old Japanese man was treated by combination therapy with pegylated interferon-α-2b and ribavirin for hepatitis C. Three months after the initiation of therapy, he showed erythema in the posterior cervical to dorsal and anterior cervical to thoracic regions, weight loss, general malaise, muscle pain, and severe increase in levels of muscle enzymes. We made a diagnosis of dermatomyositis according to these clinical features, proximal muscle-predominant myogenic change on electromyography, and infiltration of monocytes and CD4+-dominant lymphocytes on skin biopsy, although myositis-associated antibodies were absent. He was successfully treated with intravenous immunoglobulin and tacrolimus in addition to glucocorticoid. This is a very rare case of dermatomyositis associated with interferon-α therapy. We reviewed several similar published cases and the association of dermatomyositis and type I interferon.  相似文献   

8.
Obstructive jaundice secondary to tuberculosis (TB) is extremely rare. It can be caused by TB enlargement of the head of the pancreas, TB lymphadenitis, TB stricture of the biliary tree, or a TB mass of the retroperitoneum. A 29-year-old man with no previous history of TB presented with abdominal pain, obstructive jaundice, malaise and weight loss. Ultrasonography (US), computer tomography (CT) scan and endoscopic retrograde cholangiopancreatography (ERCP) were suggestive of a stenosis of the distal common bile duct (CBD) caused by a mass in the posterior head of the pancreas. Tumor markers, CEA and CA19-9 were within normal limits. At operation, an enlarged, centrally caseous lymph node of the posterior head of the pancreas was found, causing inflammatory stenosis and a fistula with the distal CBD. The lymph node was removed and the bile duct resected and anastomosed with the Roux-en Y jejunal limb. Histology and PCR based-assay confirmed tuberculous lymphadenitis. After an uneventful postoperative recovery, the patient was treated with anti-tuberculous medication and remained well 2.5 years later. Though obstructive jaundice secondary to tuberculous lymphadenitis is rare, abdominal TB should be considered as a differential diagnosis in immunocompromised patients and in TB endemic areas. Any stenosis or fistulation into the CBD should also be taken into consideration, and biliary bypass surgery be performed to both relieve jaundice and prevent further stricture.  相似文献   

9.
We describe a new type of cardiomyopathy caused by a mutation in the glycogenin-1 gene (GYG1). Three unrelated male patients aged 34 to 52 years with cardiomyopathy and abnormal glycogen storage on endomyocardial biopsy were homozygous for the missense mutation p.Asp102His in GYG1. The mutated glycogenin-1 protein was expressed in cardiac tissue but had lost its ability to autoglucosylate as demonstrated by an in vitro assay and western blot analysis. It was therefore unable to form the primer for normal glycogen synthesis. Two of the patients showed similar patterns of heart dilatation, reduced ejection fraction and extensive late gadolinium enhancement on cardiac magnetic resonance imaging. These two patients were severely affected, necessitating cardiac transplantation. The cardiomyocyte storage material was characterized by large inclusions of periodic acid and Schiff positive material that was partly resistant to alpha-amylase treatment consistent with polyglucosan. The storage material had, unlike normal glycogen, a partly fibrillar structure by electron microscopy. None of the patients showed signs or symptoms of muscle weakness but a skeletal muscle biopsy in one case revealed muscle fibres with abnormal glycogen storage. Glycogenin-1 deficiency is known as a rare cause of skeletal muscle glycogen storage disease, usually without cardiomyopathy. We demonstrate that it may also be the cause of severe cardiomyopathy and cardiac failure without skeletal muscle weakness. GYG1 should be included in cardiomyopathy gene panels.  相似文献   

10.
Wegener's granulomatosis (WG) is a disease of unknown etiology characterized by necrotizing granulomatous vascularitis. The upper and lower respiratory tract and kidney involvements are very common; however, its presentation as bilateral renal masses is unusual. We report a case of a 59-year-old female patient who presented with multiple bilateral renal masses. The patient presented with sinusal and ocular symptoms suggestive of WG, and positive antineutrophil cytoplasmic antibodies (c-ANCA) with an anti-PR3 pattern. Histopathologic examination of the renal biopsy specimen revealed granulomatous inflammation with vasculitis and fibrinoid necrosis. The patient management, including prednisone and cyclophosphamid, induced a marked improvement of the renal masses. This case illustrates that WG should be considered in the differential diagnosis of renal masses.  相似文献   

11.
Wang  Yukai  Zhao  Shucan  Du  Guangzhou  Ma  Songkun  Lin  Qisheng  Lin  Jianqun  Zheng  Kedi  Zhang  Guohong  Matucci-Cerinic  Marco 《Clinical rheumatology》2018,37(7):2001-2005
Clinical Rheumatology - Acute fibrinous and organizing pneumonia (AFOP) is a new histopathological pattern of acute lung injury first described by Beasley et al. in 2002. Hallmarks of pathological...  相似文献   

12.
Fibrous dysplasia (FD) is a benign skeletal disorder which may affect one or multiple bones. Lesions often involve long bones, ribs, and craniofacial bones and cause pain, fractures, and disfigurement. We describe an adolescent girl with mandibular FD who was successfully treated with bisphosphonates. She presented with a tumor-like lesion of the mandible. During the 2-year follow-up, the lesion expanded and caused significant disfigurement and pain necessitating psychiatric support. Treatment with bisphosphonate administered intravenously resulted in rapid pain relief, normalization of bone turnover, and cosmetic improvement. Management of FD has previously consisted of either conservative follow-up or surgery depending on disease activity and localization. Based on published reports and our experience, bisphosphonates should be considered in the treatment of symptomatic cases. This line of treatment may be suitable especially for craniofacial FD in which surgical treatment is particularly challenging.  相似文献   

13.
Presacral ganglioneuromas are so rare benign tumors that only 17 cases have been reported in the literature. They are abdominal masses growing slowly and differential diagnoses have to be considered. Surgical resection is important for definitive diagnosis because it represents the only therapeutic choice. Because of the benign nature of ganglioneuroma, adjuvant chemoor radiotherapy is not indicated but regular follow-up is necessary for an early diagnosis of potential local recurrence. We report a case of a 64-year-old man with a presacral ganglioneuroma.  相似文献   

14.
A 60-years old male was admitted to our department for investigation of constipation and hypogastric discomfort intensified during defecation of a few weeks duration. The cause proved to be a rectal carcinosarcoma that was treated by abdominoperineal resection and postoperative chemo-radiotherapy. The patient died 6 months later due to hepatic failure, showing evidence of disseminated disease. In general colonic carcinosarcomas constitute a rare category of malignant neoplasms whose nature is still incompletely understood. No specific treatment guidelines exist. Surgery is the mainstay of treatment and regardless of the addition of adjuvant therapy the prognosis is very poor. Systematic genetic analysis may be the clue for understanding the pathogenesis of these mysterious tumors.  相似文献   

15.
Presacral ganglioneuromas are so rare benign tumors that only 17 cases have been reported in the literature. They are abdominal masses growing slowly and differential diagnoses have to be considered. Surgical resection is important for definitive diagnosis because it represents the only therapeutic choice. Because of the benign nature of ganglioneuroma, adjuvant chemo-or radiotherapy is not indicated but regular follow-up is necessary for an early diagnosis of potential local recurrence. We report a case of a 64-year-old man with a presacral ganglioneuroma.  相似文献   

16.
A 60-years old male was admitted to our department for investigation of constipation and hypogastric discomfort intensified during defecation of a few weeks duration. The cause proved to be a rectal carcinosarcoma that was treated by abdominoperineal resection and postoperative chemo-radiotherapy. The patient died 6 months later due to hepatic failure, showing evidence of disseminated disease. In general colonic carcinosarcomas constitute a rare category of malignant neoplasms whose nature is still incompletely understood. No specific treatment guidelines exist. Surgery is the mainstay of treatment and regardless of the addition of adjuvant therapy the prognosis is very poor. Systematic genetic analysis may be the clue for understanding the pathogenesis of these mysterious tumors.  相似文献   

17.
Angiolipoma is a rare vascular variant of the benign lipomatous tumors and is generally seen in subcutaneous tissues. We report a 70-year-old female with abdominal distension not related to rectal small polypoid mass with peduncule described as angiolipoma by histologically, and review the literature.  相似文献   

18.
INTRODUCTION Bezoar is adapted from Arabic “bazahr” or “badzehr” which means an antidote or counter-poison due to the fact that till the 19th century, bezoars obtained from sacrificed animals were widely used as antidote[1]. Foreign bodies and bezoars…  相似文献   

19.
Lymphoepitelioma is a particular form of undifferentiat-ed carcinoma, characterized by a prominent lymphoid stroma, originally described in the nasopharynx. Lym-phoid stroma-rich carcinomas arising in other organs have been termed lymphoepithelioma-like carcinoma (LELC). In the liver, primary LELCs are very rare, and the majority has been identified as cholangiocarcino-mas. Here a rare case of lymphoepithelioma-like hepa-tocellular carcinoma (HCC) is described. A 47-year old woman presented with abdominal pain. Ultrasonogra-phy revealed a liver nodule, 2.2 cm in diameter, local-ized in the right lobe, adjacent to the gallbladder. Viral markers for hepatic B virus (HBV), hepatic C virus (HCV) and Epstein-Barr virus (EBV) were negative. The nod-ule was hypoechogenic. The patient underwent sur-gery, with resection of the nodule. Histology showed hepatocellular carcinoma, characterized by a promi-nent lymphoid infiltrate. At immunocytochemistry, tumor cells were reactive for Hep Par1 and glypican 3. Immunophenotyping of tumor infiltrating lymphocytes evidenced the predominance of CD8+ cytotoxic sup-pressor T cells. The postoperative clinical outcome was favorable and the patient was recurrence-free 15 mo after resection. This case, to the best of our knowl-edge, is the first reported non EBV and non cirrhosis-associated lymphoepithelioma-like hepatocellular carci-noma. The association between the lack of EBV infec-tion, the absence of cirrhosis, a "cytotoxic profile" of the inflammatory infiltrate and a good prognosis could identify a variant of lymphoepithelioma-like HCC with a favorable clinical outcome.  相似文献   

20.
Sigmoid volvulus is a rare presentation of Hirschsprung’s disease. A 38-year-old male presented with acute intestinal obstruction and a history of chronic constipation since childhood. Abdominal radiographs showed megarectum and megacolon with issipated feces. Sigmoidoscopy revealed gangrenous bowel mucosa affecting the sigmoid colon. Emergency laparotomy revealed a grossly dilated bowel with concurrent gangrenous sigmoid volvulus. He was treated successfully with proctocolectomy with J-pouch-anal anastomosis and a defunctioning ileostomy. Histological analysis was consistent with short segment Hirschsprung’s disease. Although uncommon, adult Hirschsprung’s disease is a cause of chronic constipation and can present acutely with a sigmoid volvulus. Mortality in cases with sigmoid volvulus is greater than in cases without (15.4% vs. 0%). A better awareness of this condition will facilitate management.  相似文献   

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