Autoimmunity in Waldenstrom's Macroglobulinaemia

Abstract

Fifty-seven consecutive patients with Waldenström's Macroglobuliemia were studied retrospectively for autoimmune manifestations. 28 patients or 51% (16 women and 13 men) had clinical and/or serological autoimmune manifestations, two or more of these being concomitant in 20 (12 women and 8 men). The predominant findings were Coombs' positive autoimmune hemolytic anemia (16%), seropositive rheumatoid arthritis (16%), inflammatory gastric ulcer with parietal cell autoantibodies (12%), and IgM-cardiolipin syndrome (11%). 40% of the autoimmune manifestations were present at the time of diagnosis of the Waldenström's Macroglobulinaemia and 60% were observed over a mean period of 4.7 years. All patients had an IgM M-component. There was no correlation between autoimmunity and the size of the M-component or the degree of hypo-IgG and hypo-IgA gammaglobulinemia. The only correlation between autoimmunity and infection was found in patients with gastric ulcer and parietal cell autoantibodies, in whom the infection was caused by Helicobacter pylori.

Autoimmune thrombocytopenia in non-Hodgkin's lymphomas

“…The prevalence of ITP in lymphoma studies was 0/92 in myeloma, 8 4% in MZL (54), 1.2% in follicular lymphomas (our department), 3.8-10% in makroglobulinemia, 10,28,55 and 9% in mantle cell lymphoma. 56 In T-cell lymphomas a prevalence of 11.1% 57 and in AILD prevalence of 24% 58,59 of thrombocytopenias was reported, but pathogenesis was not determined (Table 2 and 3).…”

“…Among the hematologic autoimmune disorders, AIHA is more common than ITP while in CLL, AIHA was 3.3 times more common than ITP (8)(9)(10)(11). Both are less frequent in non-CLL-NHL and AIHA was only approximately twice as frequent as ITP (AIHA 1.57%, ITP 0.76%).…”

“…The frequency of ITP in chronic lymphocytic leukemia (CLL) and NHL has been determined in several studies . [8][9][10][11][12][13][14][15] We analyzed data of individual non-CLL NHL patients with ITP from published case reports and original series of our institute with regard to the temporal relationship between ITP and lymphoma, the frequency in NHL subtypes, laboratory data and treatment outcome.…”

Autoimmune hemolytic anemias and IgG antierythrocyte autoantibodies in Waldenström's macroglobulinemia: association with FcγRIIa polymorphism

“…In the absence of a molecular marker and due to the rarity of chromosomal abnormalities in ET, assays based on X-linked polymorphic markers in female patients were developed to investigate clonality. [1][2][3] We have previously shown that the simultaneous study of several marker genes using methylation and expressionbased assays associated to the study of both granulocytes and platelets provides the finest evaluation of clonality status of hematopoiesis. 1,2 Using X-chromosome inactivation patterns (XCIPs) analysis, several groups reported that circulating granulocytes and platelets derived from ET patients displayed a monoclonal pattern.…”

“…1,2 Immunoglobulin G fragment C (Fcg)-mediated erythrophagocytosis has been recognized as the major pathogenic mechanism responsible for AIHA. 3,4 The clearance of immune complexes and the efficacy of their phagocytosis by splenic macrophages depend on the interactions between Fcg receptors (FcgR) and immunoglobulin G (IgG).…”