THE CASTLEMAN DISEASE, POEMS-SYNDROME AND PRODUCTIVE VASCULITIS IN MICROVASCULAR NETWORK OF DIFFERENT TISSUES

Objective: to study the condition of microvascular network in the tissues surrounding the biopsied lymph nodes: in the fat and connective tissue capsule, and to match these findings with the clinical picture of the disease.

 Material and methods. Our research included 24 patients with Castleman’s disease (CD), all negative for HIV and herpes virus-8 type, at the age of 36–70 years (men-14, women-10). 10 patients had hyaline-vascular type of CD, 14 patients had plasma-cell multicenter type. Of the 24 patients, 10 patients developed POEMS syndrome. The diagnosis of CD was confirmed by the histological examination of the lymph node and immunohistochemical study of the distribution of B- and T- lymphocytes in the node tissue. Vessels showed immunohistochemical positivity with antibodies to CD34, plasma cells with antibodies to CD138. Four patients with POEMS syndrome did not receive treatment, 6 patients received various types of therapy. Of these, 2 patients underwent therapy with autologous stem cell transplantation of bone marrow. Both patients were in remission of CD and POEMS for 10 years. Two patients received 5 cycles of therapy according to VCD scheme. They were alive for 5 years with the reduction of POEMS’s symptoms. 1 patient with POEMS and autoimmune hemolysis has received 10 courses of R-CHOP and has resulted in remission for 3 years. Two of the 6 deceased patients with POEMS syndrome underwent autopsy. 

Results and discussion. In all patients, regardless of the histological variant of CD, productive vasculitis in microvascular network was found and it had following characteristics: thickening of the walls due to lymph-histiocytic infiltration, fibrosis, swelling of endothelial cells, which narrowed or completely obstruct the vessel’s lumen. The most severe sclerosis of small vessels in the lymph node tissue was observed in patients with POEMS, in which perivascular infiltration included plasma cells. Patients with POEMS showed extensive involvement in the sclerotic process nearby vessels. The study of the microvascular network on the autopsy material revealed its systemic damage in all internal organs. 

Conclusion. Regardless of the histological variant of Castleman’s disease, in all 24 cases there was a lesion of microvascular network in the form of plasma cell productive vasculitis, with the most severe changes in group of patients with POEMS syndrome.

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