Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Abstract

Xanthomas are a common manifestation of lipid metabolism disorders. They include hyperlipemic xanthoma, normolipemic xanthoma, and a related condition, necrobiotic xanthogranuloma (NXG). All 3 forms can be associated with monoclonal immunoglobulin (MIg). In an attempt to improve diagnosis, understanding, and treatment of this association, we retrospectively analyzed a personal series of 24 patients (2 hyperlipemic xanthoma, 11 normolipemic xanthoma, and 11 NXG) and 230 well-documented reports from the literature. With the exception of the nodules and plaques featured in NXG, the clinical presentation of xanthomatous lesions usually resembled that seen in common hyperlipidemic forms and could not be used to suspect MIg-associated xanthomas. Extracutaneous sites were not rare. The MIg was an IgG in 80% of cases. Myeloma was diagnosed in 35%. Hypocomplementemia with low C4 fraction was present in 80% of studied patients. Low C1 inhibitor serum levels were found in 53%. Cryoglobulinemia was detected in

Xanthomas: Clinical and pathophysiological relations

“…Eruptive xanthomas call attention to severe hypertriglyceridemia, especially in patients with newly diagnosed or decompensated diabetes mellitus; an elevated risk of acute pancreatitis occurs within the framework of the chylo micronemic syndrome 35,36,41 . Diffuse plane xanthomas can be associated with paraproteinemia (multiple myeloma, monoclonal gammopathy of undetermined significance) and with lymphoproliferative diseases (skin lymphomas, chronic lymphatic leukaemia, chronic myeloid leukaemia 22,26 .…”

“…For the purpose of a general review, the following groups seem useful: normolipidemic xanthomas (NX), hyperlipidaemic xanthomas (HX), and necrobiotic xanthogranuloma (NXG) (ref. 21,22 ). Normolipidemic xanthomas mostly appear as diffuse flat skin lesions, while hyperlipidaemic types are polymorphous, often tuberous, and can affect either skin or tendons and joints.…”

Multiple Tuberous Xanthomas: A Diagnostic Dilemma on Cytology

“…[8] Xanthomas consist of cholesterol, cholesterol esters, triglycerides, phospholipids and numerous lipid-laden foamy macrophages. [9] case, the patient had lesions at similar sites. Hyperlipidemias may basically be classified as either familial (also called primary caused by specific genetic abnormalities, or acquired (also called secondary) when resulting from another underlying disorder that leads to alterations in plasma lipid and lipoprotein metabolism.…”

Paraneoplastik özellik gösteren metabolik hastalıklar

“…Ksantomlar, lipid metobalizmasındaki bozukluklar sonucunda, kolesterolden zengin köpüksü hücrelerin deri ve tendonlarda birikimi ile ortaya çıkan bir grup hastalıktır 14 . Bazı ksantom tiplerinde eşlik eden monoklonal gammopati bulunur (Tablo 2).…”

“…Buna göre paraprotein-lipoprotein komplekslerinin makrofajlar tarafından fagositozuyla dermal perivasküler alanlarda köpüksü histiyositler birikir [16][17][18] komplekslerin nadir de olsa damar duvarlarında çökmesi sonucunda ise peteşi ve purpuralar izlenebilir 17 . Ayrıca olguların %13'ünde deri dışı tutulum bildirilmiştir 14 . Diffuz normolipemik yassı ksantom, idiopatik olabileceği gibi başta multipl miyelom (MM) ve diğer monoklonal gammopatiler olmak üzere çeşitli hematolojik hastalıklar veya lösemi ve lenfoma gibi lenfoproliferatif hastalıklar ile lipoprotein metabolizma bozukluklarına eşlik edebilir [15][16][17]19 .…”