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A case of hepatic amyloidosis with a rapidly progressive course
AJG – September, Suppl., 2003
slight variations of viral load only detected with sensitive HBV DNA assays. The relative strength of this group is relatively unknown, but could be a very important to treatment with new and safe antivirals, given the well known risk of hepatocellular carcinoma and progression to cirrhosis, being generally excluded from regular trials because of normal transaminases (ALT). Methods: We prospectively evaluated 148 HBsAg positive patients during 54 months. HBV DNA was assessed every 6 –9 months by PCR. Histology and immunohistochemmistry was available in all patients. Results: Positive HBV DNA was detected in 115/148, with normal ALT in 26% (39). All positive DNA with normal ALT were AntiHBe⫹ (HBeAg-). Only 22% of HBsAg ⫹ patients had minimal lesions, with variable degrees of hepatitis and cirrhosis in all others. Only 23% are properly defined as inactive carriers, as 77% had DNA persistently or temporarily detected by PCR. Cirrhosis was diagnosed in 12% of inactive carriers, 8% of normal ALT patients with ⫹ DNA, and in 35% of abnormal ALT patients. Conclusions: We conclude that beyond the classical candidates for antiviral treatment (51%), in our population of patients, a significative group (26%) could be enrolled in therapeutic trials if the treatment goal is to erradicate HBV DNA. 266 THE NEED OF VACCINATION AGAINST HEPATITIS A IN PATIENTS WITH CHRONIC LIVER DISEASE : FACT OR FANCY? Guilherme Macedo, Ph.D.* Susana Lopes, M.D. Fernando Araujo, M.D. Tavarela Veloso, Ph.D. H S Joa˜ o, Porto, Portugal. Purpose: It has been suggested that there is an ongoing change in recent years, in the prevalence of anti HAV antibody among portuguese population, particularly related to overall improvement in sanitary conditions, both in urban and rural areas in the last 3 decades. This lower prevalence would be related with the lower infection rate in children, adolescents and young adults. Methods: Given the recommendations for anti HAV vaccination in chronic liver disease patients, we analysed the prevalence of anti HAV antibody in 320 patients from the Hepatology out-patient clinic with mean age of 48 years (18-75). Seroconversion was assessed at the 7th month (1 month after booster dose). Results: Only in 13 patients (4%) with chronic liver disease, the anti HAV was not detected. Their mean age is 25 (18 – 40) years old. Anti HAV prevalence was 87% in ⬍ 40 years old patients (85/98) and 100% in ⬎ 40 years old patients (222/222). Their clinical features are presented (5 with HCV chronic hepatitis, 6 with HBV chronic hepatitis, 1 with Alagille Syndrome and one post liver transplantation for fulminant hepatitis) and their seroconversion rate was 100% . Conclusions: We conclude that, even if among out-patient population there is a lower prevalence of anti HAV antibody, pre vaccination testing is mandatory, and its use is expected to be exceptional in chronic liver disease patients older than 40. 267 CORRELATION OF SERUM HEPATITIS C VIRUS RNA LOAD AND ITS ASSOCIATION WITH ALANINE AMINOTRANSFERASE IN PATIENTS REPORTING TO GASTROENTEROLOGY AND LIVER CLINIC OF HOLY FAMILY, RAWALPINDI, PAKISTAN Mohammad Umar, F.C.P.S., Faiz Anwar, F.C.P.S., Hamama Tul Bushra, F.C.P.S.*, Atifa Shoaib, F.C.P.S., Amir Chohan, M.B.B.S. Rawalpindi Medical College, Rawalpindi, Punjab, Pakistan. Purpose: Serum Hepatitis C virus RNA load is among the important factors in determining the response to anti-viral therapy in chronic Hepatitis C patients. This study was conducted to see pattern of viral load in patients
Abstracts
S91
with chronic hepatitis C in local population and its association with alanine Aminotransferase. Methods: We have studied viral load in 50 patients. These are divided into 4 categories according to age. We also calculated the relationship between viral load and alanine Aminotransferase (ALT). Patients were tested for ALT and viral load with Amplicor HCV RNA essay for qualitative detection and DNA essay for quantitative estimation. Results: Total patients are 50, with age ranging from 22 to 56 years. Mean age is 38.94 ⫾ 8.52 SD. Females are 21 (42%), Males are 29 (58%); age between 20 to 30 years are 8 (16%), between 31 to 40 years are 25 (50%), between 41 to 50 are 12 (24%), 5 (10%) have age greater than 50 years. Mean viral load is 20.44 MEq/ml. 19 (38%) patients have viral load less than 3 MEq/ml and 31 (62%) patients have greater than 3 MEq/ml. Mean ALT is 112 IU/L. Conclusions: Most patients have viral load ⱖ 3 MEq/ml (3 million copies/ ml). Mean viral load in patients more than 50 years was significantly (p ⬍ 0.01) higher than other patients. No positive correlation was found between ALT and HCV RNA viral load (p ⬍ 0.05).
268 A CASE OF HEPATIC AMYLOIDOSIS WITH A RAPIDLY PROGRESSIVE COURSE Georgios I. Papachristou, M.D.*, Shelly S. Lo, M.D., Toby O. Graham, M.D. University of Pittsburgh Medical Center, Pittsburgh, PA. Background: Amyloidosis is a rare disorder of protein metabolism that leads to extracellular tissue deposition of insoluble proteinaceous material. Although hepatic involvement is common in systemic amyloidosis, clinically significant liver failure is very rare. We report a case of primary amyloidosis, AL type, with massive hepatomegaly complicated by swiftly progressive liver failure. Case: A 58 year-old African American female visited her primary physician with a 10 day history of epigastric fullness and fatigue. She reported having dark urine, pale stools, and pruritus. On examination she had scleral icterus and a vertical liver span of 20 cm. Laboratory findings were significant for abnormal liver tests (AST 91 IU/liter , ALP 1887 IU/liter , total bilirubin 8.9 mg/dl with a direct of 5.2 mg/dl), hypoalbuminemia (1.8 g/dl), and mild coagulopathy (INR 1.4). CT scan showed hepatomegaly with diffusely homogenous low attenuated hepatic parenchyma suggestive of an infiltrative process. Liver biopsy revealed diffuse parenchymal infiltration by acellular material with hepatocyte compression and loss. The immunostain for amyloid protein P was positive. The M spike (IgG kappa) on electrophoresis confirmed a diagnosis of primary amyloidosis, AL type. One month following diagnosis, she developed liver failure, complicated by S. viridans sepsis, renal and respiratory failure, and expired. Discussion: Primary systemic amyloidosis (AL) is a plasma cell dyscrasia associated with a monoclonal protein derived from immunoglobulin light chain fragments. The liver is commonly involved in all forms of systemic amyloidosis, especially in AL. Hepatomegaly, modest elevations in ALP, and hypoalbuminemia are the most common features. The presence of hyperbilirubinemia suggests an ominous prognosis and most deaths are related to renal or cardiac complications. Treatment of AL focuses on suppressing the underlying B cell monoclonal proliferative disorder. Treatment options include chemotherapy alone, or chemotherapy followed by autologous stem-cell transplantation in patients with a high performance status. In individuals with isolated advanced hepatic amyloidosis, orthotropic liver transplantation followed by autologous stem-cell transplantation has been recommended. In the above case, a question remains whether initiation of chemotherapy or liver transplantation at the time of presentation, would have altered the patient’s rapid downhill course.
slight variations of viral load only detected with sensitive HBV DNA assays. The relative strength of this group is relatively unknown, but could be a very important to treatment with new and safe antivirals, given the well known risk of hepatocellular carcinoma and progression to cirrhosis, being generally excluded from regular trials because of normal transaminases (ALT). Methods: We prospectively evaluated 148 HBsAg positive patients during 54 months. HBV DNA was assessed every 6 –9 months by PCR. Histology and immunohistochemmistry was available in all patients. Results: Positive HBV DNA was detected in 115/148, with normal ALT in 26% (39). All positive DNA with normal ALT were AntiHBe⫹ (HBeAg-). Only 22% of HBsAg ⫹ patients had minimal lesions, with variable degrees of hepatitis and cirrhosis in all others. Only 23% are properly defined as inactive carriers, as 77% had DNA persistently or temporarily detected by PCR. Cirrhosis was diagnosed in 12% of inactive carriers, 8% of normal ALT patients with ⫹ DNA, and in 35% of abnormal ALT patients. Conclusions: We conclude that beyond the classical candidates for antiviral treatment (51%), in our population of patients, a significative group (26%) could be enrolled in therapeutic trials if the treatment goal is to erradicate HBV DNA. 266 THE NEED OF VACCINATION AGAINST HEPATITIS A IN PATIENTS WITH CHRONIC LIVER DISEASE : FACT OR FANCY? Guilherme Macedo, Ph.D.* Susana Lopes, M.D. Fernando Araujo, M.D. Tavarela Veloso, Ph.D. H S Joa˜ o, Porto, Portugal. Purpose: It has been suggested that there is an ongoing change in recent years, in the prevalence of anti HAV antibody among portuguese population, particularly related to overall improvement in sanitary conditions, both in urban and rural areas in the last 3 decades. This lower prevalence would be related with the lower infection rate in children, adolescents and young adults. Methods: Given the recommendations for anti HAV vaccination in chronic liver disease patients, we analysed the prevalence of anti HAV antibody in 320 patients from the Hepatology out-patient clinic with mean age of 48 years (18-75). Seroconversion was assessed at the 7th month (1 month after booster dose). Results: Only in 13 patients (4%) with chronic liver disease, the anti HAV was not detected. Their mean age is 25 (18 – 40) years old. Anti HAV prevalence was 87% in ⬍ 40 years old patients (85/98) and 100% in ⬎ 40 years old patients (222/222). Their clinical features are presented (5 with HCV chronic hepatitis, 6 with HBV chronic hepatitis, 1 with Alagille Syndrome and one post liver transplantation for fulminant hepatitis) and their seroconversion rate was 100% . Conclusions: We conclude that, even if among out-patient population there is a lower prevalence of anti HAV antibody, pre vaccination testing is mandatory, and its use is expected to be exceptional in chronic liver disease patients older than 40. 267 CORRELATION OF SERUM HEPATITIS C VIRUS RNA LOAD AND ITS ASSOCIATION WITH ALANINE AMINOTRANSFERASE IN PATIENTS REPORTING TO GASTROENTEROLOGY AND LIVER CLINIC OF HOLY FAMILY, RAWALPINDI, PAKISTAN Mohammad Umar, F.C.P.S., Faiz Anwar, F.C.P.S., Hamama Tul Bushra, F.C.P.S.*, Atifa Shoaib, F.C.P.S., Amir Chohan, M.B.B.S. Rawalpindi Medical College, Rawalpindi, Punjab, Pakistan. Purpose: Serum Hepatitis C virus RNA load is among the important factors in determining the response to anti-viral therapy in chronic Hepatitis C patients. This study was conducted to see pattern of viral load in patients
Abstracts
S91
with chronic hepatitis C in local population and its association with alanine Aminotransferase. Methods: We have studied viral load in 50 patients. These are divided into 4 categories according to age. We also calculated the relationship between viral load and alanine Aminotransferase (ALT). Patients were tested for ALT and viral load with Amplicor HCV RNA essay for qualitative detection and DNA essay for quantitative estimation. Results: Total patients are 50, with age ranging from 22 to 56 years. Mean age is 38.94 ⫾ 8.52 SD. Females are 21 (42%), Males are 29 (58%); age between 20 to 30 years are 8 (16%), between 31 to 40 years are 25 (50%), between 41 to 50 are 12 (24%), 5 (10%) have age greater than 50 years. Mean viral load is 20.44 MEq/ml. 19 (38%) patients have viral load less than 3 MEq/ml and 31 (62%) patients have greater than 3 MEq/ml. Mean ALT is 112 IU/L. Conclusions: Most patients have viral load ⱖ 3 MEq/ml (3 million copies/ ml). Mean viral load in patients more than 50 years was significantly (p ⬍ 0.01) higher than other patients. No positive correlation was found between ALT and HCV RNA viral load (p ⬍ 0.05).
268 A CASE OF HEPATIC AMYLOIDOSIS WITH A RAPIDLY PROGRESSIVE COURSE Georgios I. Papachristou, M.D.*, Shelly S. Lo, M.D., Toby O. Graham, M.D. University of Pittsburgh Medical Center, Pittsburgh, PA. Background: Amyloidosis is a rare disorder of protein metabolism that leads to extracellular tissue deposition of insoluble proteinaceous material. Although hepatic involvement is common in systemic amyloidosis, clinically significant liver failure is very rare. We report a case of primary amyloidosis, AL type, with massive hepatomegaly complicated by swiftly progressive liver failure. Case: A 58 year-old African American female visited her primary physician with a 10 day history of epigastric fullness and fatigue. She reported having dark urine, pale stools, and pruritus. On examination she had scleral icterus and a vertical liver span of 20 cm. Laboratory findings were significant for abnormal liver tests (AST 91 IU/liter , ALP 1887 IU/liter , total bilirubin 8.9 mg/dl with a direct of 5.2 mg/dl), hypoalbuminemia (1.8 g/dl), and mild coagulopathy (INR 1.4). CT scan showed hepatomegaly with diffusely homogenous low attenuated hepatic parenchyma suggestive of an infiltrative process. Liver biopsy revealed diffuse parenchymal infiltration by acellular material with hepatocyte compression and loss. The immunostain for amyloid protein P was positive. The M spike (IgG kappa) on electrophoresis confirmed a diagnosis of primary amyloidosis, AL type. One month following diagnosis, she developed liver failure, complicated by S. viridans sepsis, renal and respiratory failure, and expired. Discussion: Primary systemic amyloidosis (AL) is a plasma cell dyscrasia associated with a monoclonal protein derived from immunoglobulin light chain fragments. The liver is commonly involved in all forms of systemic amyloidosis, especially in AL. Hepatomegaly, modest elevations in ALP, and hypoalbuminemia are the most common features. The presence of hyperbilirubinemia suggests an ominous prognosis and most deaths are related to renal or cardiac complications. Treatment of AL focuses on suppressing the underlying B cell monoclonal proliferative disorder. Treatment options include chemotherapy alone, or chemotherapy followed by autologous stem-cell transplantation in patients with a high performance status. In individuals with isolated advanced hepatic amyloidosis, orthotropic liver transplantation followed by autologous stem-cell transplantation has been recommended. In the above case, a question remains whether initiation of chemotherapy or liver transplantation at the time of presentation, would have altered the patient’s rapid downhill course.