Orthotopic liver transplantation (LTx) is currently the only available treatment that has been proven to halt the progress of familial amyloidotic polyneuropathy (FAP). The aim of this study was to assess mortality and symptomatic response to LTx for FAP. All 86 FAP patients transplanted at our hospital between April 1990 and November 2005 were included in the study. Five patients underwent retransplantation. The 1-, 3-and 5-year patient survival rates in patients transplanted during 1996-2005 were 94.6%, 92.3% and 92.3%, respectively, a significant difference from the rates of 76.7%, 66.7% and 66.7%, respectively, during 1990-1995 (p = 0.0003). Multivariate analysis revealed that the age at the time of LTx (≥40 years), duration of the disease (≥7 years) and modified body mass index (mBMI) (<600) were independent prognostic factors for patient survival. A halt in the progress of symptoms was noted in most patients, but only a minority experienced an improvement after LTx. To optimize the posttransplant prognosis, LTx should be performed in the early stages of the disease, and close post-LTx monitoring of heart function by echocardiography and of heart arrhythmia by Holter ECG is mandatory.

For the immunohistochemical detection of immunoglobulin (Ig) light chain amyloidosis on formalin-fixed, paraffin-embedded tissue sections, we prepared polyclonal antibodies against synthetic peptides corresponding to positions 118-134 of Ig lambda light chain and positions 116-133 of Ig kappa light chain. Nineteen cases of systemic Ig lambda light chain amyloidosis (Alambda amyloidosis), 10 cases of systemic Ig kappa light chain amyloidosis (Akappa amyloidosis), one case of immunohistochemically unclassified systemic amyloidosis and five cases of localized Alambda amyloidosis were tested with these antibodies. Anti-lambda (118-134) antiserum and the affinity-purified antibody both reacted with 18 of the 19 cases of systemic Alambda amyloidosis and all cases of localized Alambda amyloidosis, although the immunoexpression was somewhat variable in intensity in different areas within the same specimen in both systemic and localized amyloidosis. The signal intensities in plasma cells and serum reacted for anti-lambda (118-134) antiserum were weaker than signals obtained with commercially available anti-Ig lambda light chain antibodies. Anti-kappa (116-133) antiserum and the affinity-purified antibody reacted with nine of the 10 cases of systemic Akappa amyloidosis. We conclude that these antibodies against synthetic peptides corresponding to the Ig light chain constant region are useful for the classification of amyloidosis on formalin-fixed, paraffin-embedded tissue sections.

No abstract

No abstract

We assessed whether a panel of seven antibodies is useful in the differentiation of adenocarcinoma cells (ACCs) from reactive mesothelial cells (RMCs) in effusion samples and to determine optimal specimen preparation conditions for immunocytochemical analysis of effusion samples. Immunocytochemistry (ICC) was performed on three types of effusion preparations from the same effusion specimens: ethanol-fixed smears, ethanol-fixed cell-blocks, and formalin-fixed cell-blocks. Commercially available antibodies MOC-31, Ber-EP4, CA19-9, CEA, EMA, CA125, and HBME-1 were tested on RMCs from four samples of various etiology and 15 samples of adenocarcinoma from various primary sites. Ethanol-fixed smears showed strong immunoreactivity to all antibodies tested. The immunoreactivity of ethanol-fixed and formalin-fixed cell-blocks was significantly lower with all antibodies except CA19-9. Smear preparations are more sensitive than cell-blocks for immunocytochemical study. A panel of antibodies MOC-31, Ber-EP4, CA19-9, and CEA appears to be suitable to distinguish between ACCs and RMCs.