Summary
A review of the less frequent organ damage seen in the so-called essential mixed cryoglobulinemia, and distinguishing between cryodependent and cryofavouring organ involvement, is presented in this paper. In the first group, along with the well-known renal, cutaneous and articular lesions, the peripheral neuropathy and respiratory involvement, to date too little considered, are worthy of more accurate study. Gastroenteric and cardiac participations are rare. Among the cryofavouring manifestations of the disease, we have pointed out those affecting the liver. In our experience, however, as the immunohistochemical findings suggest, the histological patterns usually considered to be chronic persistent hepatitis are frequently an expression of a lymphoproliferative lymphoma-like process. A noteworthy percentage of lymphoid cells in the bone marrow has been found in 22 type II cryoglobulinemias after either needle puncture or osteomedullary biopsy. The histological pictures often resemble a lymphoproliferative disease. Clinical and laboratory (computerized tomographic scan) researches frequently show a moderate splenomegaly, but in none of the patients was superficial or deep lymph node enlargement found. The authors conclude by considering type II essential cryoimmunoglobulin to be a sign and a marker of a low-grade proliferative disease with rare evolution into overt lymphoma. At the same time, however, cryoimmunoglobulins themselves cause important injuries that affect the prognosis of the syndrome.
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Monteverde, A., Bordin, G., Zigrossi, P. et al. Cryodependent and cryoproducting involvement of organs in type II essential mixed cryoglobulinemia. La Ricerca Clin. Lab. 16, 357–366 (1986). https://doi.org/10.1007/BF02909362
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DOI: https://doi.org/10.1007/BF02909362