Thrombocytosis:A paraneoplastic syndrome in patients with hepatocellular carcinoma

AIM:Hepatocellular carcinoma(HCC)patients manifest avariety of paraneoplastic syndromes.Thrombocytosis wasreported in children with hepatoblastoma.The aims of thisstudy were to evaluate the prevalence and dinical significanceof thrombocytosis in HCC patients and its relationships withserum thrombopoietin (TPO).METHODS:We retrospectively reviewed clinical,biochemicaland image data of 1 154 HCC patients.In addition,wemeasured platelet count and serum TPO in HCC patientswith and without thrombocytosis,in patients with cirrhosis,chronic hepatitis and healthy subjects in a cross-sectional study.RESULTS:Thirty-one(2.7%)of 1 154 HCC patients hadthrombocytosis(platelet count≥400 K/mm~3).HCC patientswith thrombocytosis were significantly younger,had ahigher serum α-fetoprotein,higher rate of main portal veinthrombosis,larger tumor volume,shorter survival,andwere less likely to receive therapy than HCC patients withoutthrombocytosis.Multivariate logistic regression analysesshowed that tumor volumes≥30% and serum α-fetoprotein≥140 000 ng/mL could significantly predict thrombocytosis.HCC patients with thrombocytosis had a significantly highermean serum TPO than those without,as well as patientswith cirrhosis,chronic hepatitis and healthy subjects.Platelet count and serum TPO dropped significantly aftertumor resection in HCC patients with thrombocytosis andre-elevated after tumor recurred.Furthermore,the expression of TPO mRNA was found to be more in tumor tissues thanin non-tumor tissues of liver in an HCC patient withthrombocytosis.CONCLUSION:Thrombocytosis is a paraneoplastic syndromeof HCC patients due to the overproduction of TPO by HCC.It is frequently associated with a large tumor volume andhigh serum α-fetoprotein.     

Thrombocytosis： A paraneoplastic syndrome in patients with hepatocellular carcinoma

AIM: Hepatocellular carcinoma (HCC) patients manifest a variety of paraneoplastic syndromes. Thrombocytosis was reported in children with hepatoblastoma. The aims of this study were to evaluate the prevalence and dinical significance of thrombocytosis in HCC patients and its relationships with serum thrombopoietin (TPO).METHODS: We retrospectively reviewed clinical, biochemical and image data of 1 154 HCC patients. In addition, we measured platelet count and serum TPO in HCC patients with and without thrombocytosis, in patients with cirrhosis,chronic hepatitis and healthy subjects in a Eoss-sedJonal study.RESULTS: Thirty-one (2.7%) of 1 154 HCC patients had thrombocytosis (platelet count ≥400 K/mm^3). HCC patients with thrombocytosis were significantly younger, had a higher serum α-fetoprotein, higher rate of main portal vein thrombosis, larger tumor volume, shorter survival, and were less likely to receive therapy than HCC patients without thrombocytosis. Multivariate logistic regression analyses showed that tumor volumes ≥30% and serum α-fetoprotein≥ 140 000 ng/mL could significantly predict thrombocytosis.HCC patients with thrombocytosis had a significantly higher mean serum TPO than those without, as well as patients with cirrhosis, chronic hepatitis and healthy subjects.Platelet count and serum TPO dropped significantly after tumor resection in HCC patients with thrombocytosis and re-elevated after tumor recurred. Furthermore, the expression of TPO mRNA was found to be more in tumor tissues than in non-tumor tissues of liver in an HCC patient with thrombocytosis.CONCLUSION: Thrombocytosis is a paraneoplastic syndrome of HCC patients due to the overproduction of TPO by HCC.It is frequently associated with a large tumor volume and high serum α-fetoprotein.     

Bacillus Calmette-Guérin-induced granulomatous hepatitis in a patient with a superficial bladder carcinoma

A 71-year-old male patient with a superficial transitional cell carcinoma of the urinary bladder developed high fever and jaundice, accompanied by progressively increasing serum aminotransferase activities, 2 weeks after the fourth local instillation with an attenuated live strain of Mycobacterium bovis [bacillus Calmette-Guérin (BCG)]. A liver biopsy showed non-caseating granulomatous hepatitis. Cultures for mycobacteria were negative. Mycobacterial DNA was not detected in liver tissue using the polymerase chain reaction. Empirical treatment with rifampicin and isoniazid was started, resulting in partial recovery. After 6 months of therapy, however, serum aminotransferase activities were still twice the upper limit of normal. A second liver biopsy still demonstrated several granulomas. Only after addition of prednisolone, liver tests completely normalized. Also histologically the lesions improved dramatically. This suggests that the BCG hepatitis was at least partially caused by a hypersensitivity reaction. Our patient is the first reported case of BCG hepatitis with histological follow-up under therapy.     

Angiocentric T cell lymphoma of the central nervous system in a patient with Sjögren's syndrome

Patients with Sj?gren's syndrome (SS) have an increased risk of developing lymphoproliferative disorders, mainly non-Hodgkin's lymphoma and pseudolymphoma. We describe a young Caucasian woman with a 7 year history of SS, who presented with meningeal signs and symptoms and was found to have non-Hodgkin's lymphoma of the central nervous system.     

Functional asplenia in a patient with Rothmann-Makai syndrome: a pathogenetic relationship?

SIR, A 27-yr-old woman was referred for evaluation of mild anaemia,leucocytosis and thrombocytosis. History revealed a diagnosisof Rothmann–Makai syndrome (lipogranulomatosis subcutanea),a rare variant of Weber–Christian disease, at the ageof 13 yr. At that time the patient had presented with a minorfracture of the left ankle, and when the plaster was removedshe was found to     

Elevation of alkaline phosphatase in a pregnant patient with antiphospholipid syndrome: HELLP syndrome or not?

SIR, Pregnant patients with APS may develop HELLP syndrome (haemolysis;elevated liver enzymes; low platelet count) that can manifestfirst by elevation of ALP level. However, other causes of ALPelevation must be discussed and include elevation of ALP ofplacental origin. We report for the first time an APS pregnant     

Indeterminate colitis: a review of the concept--what's in a name?

The precise diagnosis of colitis cannot always be established with the available diagnostic tools. The subgroup of patients with an uncertain diagnosis has been classified as "indeterminate colitis" (IC). The definition of "indeterminate," however, has changed over the years. Originally, IC was proposed by pathologists for colectomy specimens, usually from patients operated on for severe colitis, showing overlapping features of ulcerative colitis (UC) and Crohn's disease (CD). Later, the same terminology was used for patients showing no clear clinical, endoscopic, histologic, and other features allowing a diagnosis of either UC or CD. Therefore, it is difficult to compare different studies. An International Organization of Inflammatory Bowel Diseases (IOIBD) working party confirmed 1) the ambiguous nature of the term, and 2) proposes an updated classification for the category of patients with an unclear diagnosis. According to this, the term IBD unclassified (IBDU) is confirmed, as suggested by the Montreal Working Party 2005 for patients with clinically chronic colitis, that clearly have IBD but when definitive features of CD or UC are absent. In resected specimens the term "colitis of uncertain type or etiology" (CUTE) is preferred. It is accepted that most of the time this may have a prefix, such as severe, chronic. The classification of IBD varies when based only on biopsies rather than on a colectomy specimen. The vast majority of these have severe colitis. For those that cannot bear to abandon the highly ambiguous term IC, if it is used at all, this is where it can be used parenthetically.     

Bowel-associated dermatosis − arthritis syndrome in a patient with ulcerative colitis: an extraintestinal manifestation of inflammatory bowel disease

Bowel-associated dermatosis ? arthritis syndrome (BADAS) is a neutrophilic dermatosis characterized by cutaneous lesions that begin as erythematous macules and progress to vesiculopustular eruptions. It has been described in patients with inflammatory bowel disease as well as those who have undergone various intestinal surgeries. Pathologically, the lesions show features of vasculitis without fibrinoid necrosis. In a patient with diagnosed inflammatory bowel disease, these neutrophilic dermatoses should be viewed as external signals of bowel inflammation. Management requires long-term treatment of the underlying disease. We report a case of BADAS in a patient with ulcerative colitis in which the skin lesions were associated with increased colonic inflammation.     

Complicated granulomatous colitis in a Japanese patient with Hermansky–Pudlak syndrome, successfully treated with infliximab

We report a case of refractory granulomatous colitis and perineal fistulas complicated by Hermansky–Pudlak syndrome (HPS) that was successfully treated by repeated infusions of infliximab. The patient was a mentally retarded 30-year-old Japanese man who was diagnosed with HPS based on albinism, nystagmus, and a history of bleeding diathesis in his childhood. Since he was 28 years old, he had suffered from severe granulomatous colitis with perineal fistulas, which had been resistant to medical treatment including sulfasalazine, mesalamine, and steroids. At 30 years of age, diverting colostomy was performed aiming at subsiding severe bleeding and a perianal abscess. Three months after surgery, the patient developed sudden onset of fever, chills, and bloody bowel movements. Then, infliximab was administered at a dose of 5 mg/kg, and most of symptoms were relieved within 48 h of administration. The patient obtained remission with a constant daily dosage of azathioprine and a monthly dosage of infliximab. The present case is the first report of complicated granulomatous colitis with HPS, which was successfully treated with infliximab in Japan.     

Molecular biomarkers in urothelial carcinoma of the bladder: are we there yet?

The unprecedented advances in cancer genetics and genomics are rapidly affecting the clinical management of solid tumors. Molecular diagnostics are now an integral part of routine clinical management for patients with lung, colon, and breast cancer. In sharp contrast, molecular biomarkers have been largely excluded from current management algorithms for urologic malignancies. The need for new treatment options that can improve upon the modest outcomes currently associated with muscle-invasive bladder cancer is evident, and validated prognostic molecular biomarkers that can help clinicians to identify patients in need of early, aggressive management are lacking. Robust predictive biomarkers that are able to forecast and stratify responses to emerging targeted therapies are also needed.