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Objective: to consider clinical practice problems in the differential diagnosis of different types of plasma cell dyscrasias (PCD). Subjects and methods. Fourteen patients (8 men and 6 women) aged 52±12 years, in whom rheumatic diseases (RD) were ruled out and who were diagnosed as having primary PCD: different types of myeloma in 7 patients, myeloma + AL-amyloidosis in 2, AL-amyloidosis in 3, and Waldenstrom’s macroglobulinemia in 2, were examined. Results and discussion. The most common maldiagnosed RDs in patients with PCD were seronegative rheumatoid arthritis (RA), systemic lupus erythematosus, Sjogren’s disease, and different forms of vasculitis. The most frequent masks of RD were kidney (78%) and osteoarticular system (64%) lesions, vascular disorders (36%), peripheral polyneuropathies (36%), and enlarged salivary glands with xerostomia (28.5%). Serum and urine immunochemical study should be performed in all patients who have clinical manifestations of seropositive RA, spondyloarthritis, intensive bone pain syndrome, ulceronecrotic vasculitis, enlarged submandibular salivary glands with macroglossia in the absence of markers of autoimmune disease for the timely diagnosis of PCD and the exclusion of RD. The paper estimates the sensitivity and specificity of main methods used to diagnose different types of PCD.

The paper describes two patients (a 50-year woman and a 72-year man) in whom the clinical manifestations of ulceronecrotic vasculitis had long preceded before the diagnosis of multiple myeloma was made. In the former, monoclonal cryoglobulinemia type I induced ulcerative lesions with the development of dry toe gangrene and paraproteinemic renal lesion. In the latter, ulceronecrotic vasculitis with the development of dry toe gangrene was a manifestation of monoclonal paraproteinemia without signs of cryoglobulinemia. Both patients were found to have monoclonal blood secretion (РIgGλ и РIgGκ) and urine Bence Jones protein (BJλ+BJκ, BJκ) in the absence of immunological markers of vasculitis developing in patients with rheumatic diseases. Immunochemical study of serum/urine and, when monoclonal secretion of PIg+BJ is detected, further examination for plasma cell dyscrasia should be performed in all cases of vascular disorders (cold allergy, Raynaud's syndrome, purpura, ulcers of cruses, and gangrene of distal phalanxes of the hands/feet) in patients with atypical vasculitis. The timely immunochemical study of blood and urine will make it possible to diagnose plasma cell dyscrasia (different types of myelomic disease, Waldenstrцm macroglobulinemia, primary amyloidosis) at the early stage of the disease and to rule out unjustifiably diagnosed vasculitis in patients with atypical vascular lesions.