1/30. Intravascular haemolysis: a potential missed diagnosis.Raised serum bilirubin is a common finding as part of a routine "liver function test" profile. This case describes a patient with a raised serum bilirubin that was eventually found to be caused by intravascular haemolysis. The tests that confirmed the cause were instigated by clinical biochemistry staff but not before the patient had undergone unnecessary invasive procedures and had the diagnosis delayed by some time. The case is a reminder to biochemistry and haematology laboratories and clinical staff to investigate the possibility of haemolysis as well as hepatitis and Gilbert's disease as a cause of an isolated raised bilirubin.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
2/30. Nimesulide-induced severe hemolytic anemia and acute liver failure leading to liver transplantation.We present the case of a 63-year-old woman who had undergone 7 months of treatment with Nimesulide (100 mg/b.i.d.) for symptomatic osteoarthritis. The patient was admitted to our unit with a clinical picture of progressive jaundice over 3 weeks. Clinical and analytical studies revealed acute liver failure, this being confirmed by liver biopsy, which showed submassive necrosis. Serological tests for different viral agents causing hepatitis were all negative. In addition, she presented a picture of severe haemolytic anaemia resistant to several treatments and needed multiple transfusions. Twenty-three days after admission, the patient presented hepatic encephalopathy and received an orthotopic liver transplant on day 25. The evolution after transplantation was good and the patient continues in good health with no evidence of haemolysis almost 2 years later. Liver toxicity due to Nimesulide is well known, but to our knowledge the occurrence of haemolytic anaemia has not been related to this drug previously. For these reasons, Nimesulide has been restricted or removed from the market in several countries in recent months.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
3/30. Mycophenolate mofetil in the treatment of autoimmune HCV-associated haematological disorders showing steroid resistance or dependence.We report two cases of hepatitis c virus (HCV) associated autoimmune haematological disorders successfully treated with an unusual protocol (mycophenolate mofetil: MMF). The first case was a male patient with chronic HCV infection who developed, during interferon (IFN)/ribavirin therapy, severe autoimmune thrombocytopenia unresponsive to steroids. MMF was then administered and, simultaneously, the steroid dose was gradually reduced until withdrawal. Following this strategy, a progressive increase in platelet count and complete negativity of anti-PLT antibodies were achieved without changes in HCV-rna quantitative determination. The second case was a woman with HCV liver cirrhosis with severe anaemia and coombs test positivity partially responsive to continuous administration of steroid high doses. However, this treatment unmasked a severely painful vertebral osteoporosis. For this reason we introduced MMF and simultaneously steroid therapy was progressively reduced until withdrawal. Haemoglobin reached a normal value and the coombs test became negative within 60 days. These case reports suggest that MMF may represent an interesting therapeutic approach for autoimmune HCV associated haematological disorders.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
4/30. asialoglycoprotein receptor facilitates hemolysis in patients with alcoholic liver cirrhosis.hemolysis in patients with advanced alcoholic liver disease is a common clinical problem and indicates an unfavorable prognosis. In many cases, the etiology of the hemolysis remains unknown. We observed three patients with alcoholic liver disease, suffering from severe hemolytic anemia, requiring multiple blood transfusions. Steroid therapy was ineffective and two of the patients died. All patients had a soluble variant of the human asialoglycoprotein receptor (s-ASGP-R) in their serum, as well as high titers of autoantibodies against this receptor (anti-ASGP-R). Consecutively, examination of 60 patients with alcoholic liver disease revealed a high incidence for s-ASGP-R (36%) and anti-ASGP-R (27%) in patients with alcoholic liver cirrhosis (ALC) compared to patients with cirrhosis due to viral hepatitis. The potential etiology of hemolysis was studied in vitro on erythrocytes from patients with ALC and from healthy donors. Isolated ASGP-R but not anti-ASGP-R bound to the surface of erythrocytes preferentially of blood group A1 and caused dose-dependent agglutination and hemolysis, while this phenomenon was much lower using erythrocytes of the blood group B and almost absent with blood group O-erythrocytes. Furthermore, agglutination and hemolysis only occurred in erythrocytes from ALC-patients or after the pre-treatment of cells with neuraminidase. ASGP-R induced agglutination and hemolysis was blocked by the competitive ASGP-R inhibitor asialofetuin. In conclusion, our results indicate a new, non-immunological mechanism for hemolysis in patients with alcoholic liver disease, mediated through agglutination by a soluble variant of the human asialoglycoprotein receptor and mechanical shear stress.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
5/30. Chronic hepatitis c associated with Coombs-positive hemolytic anemia.hepatitis c virus (HCV) is a recognized cause of significant extrahepatic disease. Induction of autoimmune hemolytic anemia (AIHA) has been reported, either during or after interferon (IFN) treatment of HCV infection. We herein report a 56-year-old patient with HCV infection who developed severe Coombs-positive AIHA in the absence of treatment with IFN. prednisone therapy was initiated, but intravenous immunoglobulins were added because of persistent hemolysis. Clinical course was complicated by rapid deterioration and the development of Creutzfeldt-Jakob disease. Having discarded other possible causes of AIHA, we suggest a possible association between AIHA and infection by HCV.- - - - - - - - - - ranking = 4keywords = hepatitis (Clic here for more details about this article) |
6/30. Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients.A deficiency of citrin, which is encoded by the SLC25A13 gene, causes both adult-onset type II citrullinemia (CTLN2) and neonatal intrahepatic cholestasis (NICCD). We analyzed 16 patients with NICCD to clarify the clinical features of the disease. Severe intrahepatic cholestasis with fatty liver was the most common symptom, but the accompanying clinical features were variable, namely; suspected cases of neonatal hepatitis or biliary atresia, positive results from newborn screening, tyrosinemia, failure to thrive, hemolytic anemia, bleeding tendencies and ketotic hypoglycemia. Laboratory data showed elevated serum bile acid levels, hypoproteinemia, low levels of vitamin k-dependent coagulation factors, and hypergalactosemia. Hypercitrullinemia was detected in 11 out of 15 patients examined. Most of the patients were given a lactose-free and/or medium chain triglycerides-enriched formula and lipid-soluble vitamins. The prognosis of the 16 patients is going fairy well at present, but we should observe these patients carefully to see if they manifest any symptom of CTLN2 in the future.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
7/30. Severe systemic autoimmune disease associated with Epstein-Barr virus infection.infection with Epstein-Barr virus (EBV) has been associated with different autoimmune manifestations. The authors describe a girl who developed a severe systemic autoimmune disease with severe autoimmune hemolytic anemia, mild autoimmune thrombopenia, antineutrophil antibodies, and fatal autoimmune hepatitis after EBV infection. Despite immunosuppressive treatment and ultimately liver transplantation, this patient could not overcome her clinical condition and died. The etiopathogenesis of this complex disease and the association with EBV infection is discussed.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
8/30. ceftriaxone-induced hemolytic anemia and hepatitis in an adolescent with hemoglobin sc disease.OBJECTIVES: To describe a case of a ceftriaxone-induced hemolytic anemia and hepatitis leading to multiple organ failure and death in an adolescent with hemoglobin sc disease and to review the previous cases of this rare and potentially fatal disorder in children. DESIGN: Case report and literature review. SETTING: intensive care unit. Patient: adolescent with hemoglobin SC. INTERVENTIONS: emergency treatment. MEASUREMENTS AND MAIN RESULTS: After 4 days of ceftriaxone therapy, the adolescent experienced an acute hemolytic reaction (hemoglobin decreased to 5 g/dL with hemoglobinuria) and severe hepatitis (all enzymes increasing dramatically including aminoaspartate transferase >20,000 IU/L). Renal failure and ultimately multiple organ failure ensued, and the patient died on hospital day 19. Direct antiglobulin tests on red cells obtained from the patient on hospital day 2 showed microscopic agglutination with polyspecific and anticomplement (C3) antiglobulin reagents. plasma samples showed macroscopic agglutination reactions when incubated in the presence of ceftriaxone, many days after cessation of ceftriaxone, indicating the continued presence of ceftriaxone-dependent antibodies. CONCLUSIONS: Drug reactions leading to hemolysis are relatively uncommon, and a total of ten cases of ceftriaxone-induced hemolytic anemia have been reported in children. The present case describes an adolescent who ultimately died on hospital day 19 from multiple organ failure, although the presentation of this case seems atypical in several respects. Children with clinical syndromes that place them at risk for hemolysis and children who frequently require broad spectrum antibiotics present unique diagnostic challenges, and the possibility that hemolytic syndromes may be due to ceftriaxone must be considered.- - - - - - - - - - ranking = 6keywords = hepatitis (Clic here for more details about this article) |
9/30. The sulfone syndrome secondary to dapsone prophylaxis in a patient undergoing unrelated hematopoietic stem cell transplantation.dapsone is commonly used for pneumocystis carinii pneumonia (PCP) prophylaxis in immunocompromised patients. It has been used as an alternative therapy in the hematopoietic stem cell transplant (HSCT) setting in patients who can't tolerate trimethoprim-sulfamethoxazole. The Sulfone syndrome is not a well-known sequela of dapsone therapy and occurs at various doses, ranging from 50-300 mg/d. In all cases the syndrome occurs within 2 months of initiating therapy. Its clinical manifestations include: fever, methemoglobinemia, hemolytic anemia, exfoliative dermatitis and transaminits. A 51-year old female underwent a matched unrelated hematopoiectic stem cell transplant for acute mylogenous leukemia. dapsone therapy was initiated on day 28 at a dose of 100 mg/day for PCP prophylaxis secondary to the patient's history of a sulfonamide allergy. On day 59, one month after initiation of therapy she developed hepatitis, hemolytic anemia, fever and methemoglobinemia of 8%. She was transferred to the intensive care unit and subsequently developed an exfoliative dermatitis. We conclude that the clinical presentation of this patient after HSCT on dapsone therapy coincide with the sulfone syndrome not previously described in a patients after HSCT.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
10/30. A case of stevens-johnson syndrome, cholestatic hepatitis and haemolytic anaemia associated with use of mefenamic acid.A woman with a history of drug allergy, renal impairment and carcinoma of the breast with pulmonary micrometastases developed haemolytic anaemia and stevens-johnson syndrome following the use of mefenamic acid, paracetamol (acetaminophen) and furosemide (frusemide). In addition there was severe cholestatic hepatitis in the absence of clinical evidence of sepsis, biliary obstruction or recurrent metastases. The rash resolved on steroid therapy but the patient eventually died from both renal and liver failure. Acute tubular necrosis with a background of chronic tubulointerstitial nephritis was also found at autopsy. Although in the presence of multiple drug therapy the causative agent cannot be identified with absolute certainty, the association of these severe idiosyncratic hepatic and dermatological reactions with haemolytic anaemia strongly suggests mefenamic acid as the most likely culprit.- - - - - - - - - - ranking = 5keywords = hepatitis (Clic here for more details about this article) |
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