Amyloidosis Primary

Mucolytic Adjuvant therapy for abnormal, viscid, or inspissated mucus secretions in chronic bronchopulmonary disease (chronic emphysema, emphysema with bronchitis, chronic asthmatic bronchitis, tuberculosis, bronchiectasis, primary amyloidosis of lung) acute bronchopulmonary disease (pneumonia, bronchitis, tracheobronchitis) pulmonary complications of cystic fibrosis tracheostomy care pulmonary complications associated with surgery use during anesthesia posttraumatic chest conditions atelectasis due to mucus obstruction diagnostic bronchial studies (bronchograms, bronchospirometry, bronchial wedge catheterization). 

In this condition monoclonal immunoglobulins are produced due to a clonal disorder of either plasma cells or lymphocytes. Light chains produced are more frequently of the A than the k type (A7, S17). The monoclonal immunoglobulins produced could be either IgG, IgA, IgM, or IgD. The deposition of a fibrillar protein, most often k light chains, in many tissues is referred to as amyloidosis. The deposition of A. light chains alone is referred to as primary amyloidosis. 

Neuritic plaques are lesions found in brain and cerebral vasculature. Whether genetic variations promote a primary amyloidosis in the majority of AD patients is unresolved. 

Rnbinow, A., Koff, R.S., Cohen, A.S. Severe intrahepatic cholestasis in primary amyloidosis. A report of four cases and a review of the literature. Amer. J. Med. 1978 64 937-946... 

Peters, R.A., Koukoulis, G., Glimson, A., Portamnn, B., Westaby, D., William, R. Primary amyloidosis and severe intrahepatic cholestatic jaundice. Gut 1994 35 1322-1325... 

Sandberg-Gertzen, H., Ericzon, B.G., Blomberg, B. Primary amyloidosis with spontaneous splenic rupture, cholestasis, and liver failure treated with emergency liver transplantation. Amer. J. Gastroenterol. 1998 93 2254-2256... 

Clinically, amyloidosis is classified (omitting some rare forms) into five main groups (1) primary amyloidosis, (2) amyloidosis associated with multiple myeloma, (3) secondary amyloidosis associated with inflammatory or infectious diseases, (4) a form associated with aging, and (5) familial amyloidosis. Increased knowledge of the chemical structure of the deposits has led to a revised classification that takes into consideration the type of amyloid protein present. Tins classification is used in Table 20-14. 

AL amyloidosis is the most common form of these disorders. Bone marrow plasmacytosis and excess plasma cell production of antigenicaHy identical monoclonal light chains are common to primary amyloidosis and multiple myeloma. Thus a clear distinction between these two conditions is not possible chemically. They appear to differ only in the presence or absence of osteolytic lesions. AL deposits may occur in the tongue, heart, lymph nodes, spleen, joints, peripheral nerves, and skin. 

Kyle RA, Gertz MA, Greipp PR, Witzig TE, Lust JA, Lacy MQ, et al. A trial of three regimens for primary amyloidosis colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med 1997 336 1202-7. 

Skinner M, Anderson J, Simms R, Falk R, Wang M, Libbey C, et al. Treatment of 100 patients with primary amyloidosis a randomized trial of melpha-lan, prednisone, and colchicine versus colchicine only. Am J Med 1996 100 290-8. 

Acetylcysteine decreases thickness of mucous secretions in lung. As a mucolytic agent, acetylcysteine splits disulfide linkages between mucoprotein molecular complexes, decreasing their viscosity. It is used as an adjunct therapy in emphysema with bronchitis, chronic asthmatic bronchitis, mberculosis, bronchiectasis, primary amyloidosis of the lung, pneumonia, and tracheobronchitis. In addition, it is used in pulmonary complications of cystic fibrosis and those associated with anesthetics, surgery, or care following tracheostomy. 

Monoclonal gammopathy is related to the presence in the blood of a monoclonal Ig (Sahota et al, 1999). Serum protein analysis should be done when multiple myeloma, Waldenstrom s macroglobulinemia, primary amyloidosis or a related B cell disorder is suspected (Attaelmannan and Levinson, 2000). The strategies to detect as well as to identify monoclonal gammopathies in the clinical laboratory have been reviewed (Keren et al, 1999 Kyle, 1999b). Other, but not routinely used electrophoretic techniques, have been employed to further characterize monoclonal Igs, including isoelectric focusing and 2-DE. 

Chapman AD, Brown PA, Kerr KM. Right heart failure as the dominant clinical picture in a case of primary amyloidosis affecting the pulmonary vasculature. Scott Med J 1999 44(4) 116-117. 

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