[Essential mixed cryoglobulinemia with liver involvement: a still open problem].
Essential mixed cryoglobulinemia (EMC) is a syndrome characterized by cryoglobulinemia and clinical features including purpura, arthralgia, asthenia (Meltzer-Franklin syndrome) without evidence of any systemic disease Liver involvement in the course of EMC is described in 50-84% of patients. It consists of mild silent hepatosplenomegaly and slightly rise of serum amino transferase. Eleven patients with clinical and laboratory findings suggestive for EMC (five type II and six type III) underwent percutaneous liver biopsy to evaluate the degree of liver involvement. Two liver cirrhosis, two chronic active hepatitis, one chronic persistent hepatitis and a case of hepatic steatosis were found. A type III cryoglobulinemia was present in four of the six patients with liver involvement. All the patients were Hbs Ag negative but three of them were Hbs Ab positive. The pathogenesis of liver involvement in the course of EMC is still now uncertain. The authors believe that a previous HBV infection plays no role in the pathogenesis of EMC syndrome. This syndrome must be considered different from mixed cryoglobulinemia secondary to chronic liver disease. They suggest that liver biopsy is mandatory during the course of EMC even when clinical and laboratory data are silent.
Grieco
A
A
Istituto di Clinica Medica Generale, Università Cattolica Sacro Cuore, Roma.
Manna
R
R
Pietrantoni
A
A
Cercato
M C
MC
Ria
F
F
Vecchio
F M
FM
Gambassi
G
G
D000368:Aged; D001247:Asthenia / Q000175:diagnosis; D001707:Biopsy, Needle; D003449:Cryoglobulinemia / Q000175:diagnosis*; D003450:Cryoglobulins / Q000032:analysis; D005260:Female; D006510:Hepatitis B Antibodies / Q000032:analysis; D006514:Hepatitis B Surface Antigens / Q000032:analysis; D006521:Hepatitis, Chronic / Q000175:diagnosis; D006801:Humans; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D011693:Purpura / Q000175:diagnosis; D011928:Raynaud Disease / Q000175:diagnosis; D013577:Syndrome
D003450:Cryoglobulins; D006510:Hepatitis B Antibodies; D006514:Hepatitis B Surface Antigens
Crioglobulinemia mista essenziale con coinvolgimento epatico: un problema ancora aperto.