Hyperviscosity syndrome

Unless M protein concentrations exceed 60 g/L, patients with multiple myeloma do not develop hyperviscosity syndrome. Despite the presence of plasma cells in bone marrow exceeding 10% and the presence of M protein exceeding 25 g/L, nearly 15% of patients with multiple myeloma are asymptomatic. However, asymptomatic patients presenting with IgA myeloma protein and M protein concentrations exceeding 30 g/L and Bence Jones-protein excretion in excess of 50 mg/day in presence of a lytic bone lesion can progress to multiple myeloma earlier than other, asymptomatic patients (Wl). 

The concentration of monoclonal IgM in serum approximates 30 g/L or greater (K37). A significant number of patients excrete monoclonal light chains in urine, with a predominance of k over A. light chains (K37). An increase in monoclonal IgM contributes to an increase in serum viscosity. In one study 15 patients with WM whose M protein concentrations ranged from 43 to 80 g/L (median 55 g/L), had serum viscosity greater than 4.0 cP (K37). Rouleaux formation in hyperviscosity syndrome results in an increase in erythrocyte sedimentation rate (ESR). 

Pll. Pope, R. M., Fletcher, M. A., Mamby, A., and Shapiro, C. M., Rheumatoid arthritis associated with hyperviscosity syndrome and intermediate complex formation. Arch. Intern. Med. 135, 281-285 (1975). 

Intensive plasma exchange has been studied in the treatment of numerous diseases, above all in Waldenstrom s macroglobulinemia, hypercholesterolemia, hyperviscosity syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, myasthenia... 

D-penicillamine is so named because it was first isolated as an amine, from the degradation products of penicillin by Abraham et al [87]. Later studies showed the characteristic chemical behavior of D-penicillamine which involve three types of reactions, formation of disulphide links, formation of thiazolidine rings, and formation of metal complexes and chelates [67]. It was first used in 1956 in the treatment of Wilson s disease [88]. D-penicillamine has since been used in the treatment of many diseases, such as cystinuria [89], rheumatoid arthritis [90-92], systemic sclerosis [93], primary bdiary cirrhosis [94], heavy metal poisoning due to lead [95], cadmium [%], and mercury [97], and hyperviscosity syndrome [99]. In rheumatoid arthritis, D-peni-cdlamine has been widely accepted as an effective second line treatment. Despite of its effectiveness, it causes many adverse effects, such as skin rashes [99,100], taste abnormalities [100,101], hepatic dysfunction [102-104], gastrointestinal toxiciiy [99,105], proteinuria [100,106], hematuria [107, 108], thrombocytopenia [92, 109], aplastic anemia [110], lupus-like syndrome [111, 112], Goodpasture s-tike pulmonary renal syndrome [113-115], vasculitis [116,117], myasthenia gravis [118-122], polymyositis [123, 124], and dermatomyositis [125]. 

A condition in which there are excess macroglobulins in the blood. The high levels of large molecular weight proteins lead to sluggish blood flow and this, in turn, can result in thrombosis in the small blood vessels. Retinal vein thrombosis, cerebral thrombosis and peripheral gangrene (hyperviscosity syndrome) are features. 

Common risk factors for developing branch retinal vein thrombosis (BRVT) and central retinal vein thrombosis (CRVT) include increased plasma fibrinogen, diabetes, decreased exercise, hypertension, and hyperviscosity (205). Sickle cell anemia, polycythemia vera, and other proliferative disorders may also lead to this syndrome. 

Sokol, R.J., Martier, S. and Emhart, C.B. (1985). Identification of alcohol abuse in the prenatal clinic. In Early Identification of Alcohol Abuse. NIAAA Research Monograph-17, NIAAA Wesenberg, R.L. (1978). Neonatal thick blood" syndrome. Hosp. Pract., May, 137-145 Wibberley, D.G., Khera, A.K., Edwards, J.H. and Rushton, D.I. (1977). Lead levels in human placentae from normal and malformed births. ]. Med. Genet., 14, 339-345 Wirth, F.H., Goldberg, K.E. and Lubchenco, L.O. (1979). Neonatal hyperviscosity I. Incidence. Pediatrics, 63, 833-836... 

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