Immunoglobulins in Chronic Cold Haemagglutinin Disease

Abstract

Summary. Total serum IgM levels in 32 patients with chronic cold haemagglutinin disease ranged from 100 to 2400 mg/100 ml; follow‐up in nine patients showed slowly progressive increases. The excess IgM was mostly cold agglutinin and although there was considerable variation in serologic activity on a molecular basis, specific lytic activity against enzyme‐treated red cells was very similar. The cold agglutinin IgM seemed well produced and closely parallel to normal IgM; few 7S units and little Bence‐Jones proteinuria were detected. Non cold‐agglutinin IgM levels were mostly normal, but 31% of patients showed subnormal levels of IgA or IgG especially when the IgM level was greater than 1000 mg/100 ml.

Monomeric (7S) IgM found in the serum of rheumatoid arthritis patients share idiotypes with pentameric (19S) monoclonal rheumatoid factors.

“…Previous reports document the occurrence of polyclonal, low molecular weight IgM (7S, IgM) in various patients with autoimmune diseases (1)(2)(3)(4)(5)(6)(7)(8) and occasionally in apparently normal individuals (9,10). In rheumatoid arthritis (RA)' patients, seropositive for rheumatoid factor (RF+), the occurrence of 7S IgM is associated with disease severity and frequently with vasculitis, (11,12).…”

Sarcoidosis in a Patient with Cold Haemagglutinin Disease

“…The CHAD is a type of paraproteinemia characterized by the production of autoantibodies (anti-Ii). Our patient presented also, as has been earlier re ported in CHAD by Cooper and Hobbs [5], a deficit in IgA. The tuber culin skin test was always negative.…”

“…Our patient fulfills the diagnostic criteria of CHAD: Raynaud's phe nomenon, evidence of chronic haemolysis, a positive direct anti-globulin test of the complement type and the presence in the serum of a high-titred cold agglutinin [5], Although the formation of sarcoid-like granulomas has been reported in different entities [1] and the presence of granulomas may result from many diseases [11], the presence in our patient of bilateral hilar adenopa thy, the demonstration of non-caseating granulomas by bronchial biopsy [21] and the positive Kveim test are acceptable criteria for sarcoidosis [14]. Several possibilities may then be considered: (1) coincidental asso ciation; (2) the CHAD is an early manifestation of sarcoidosis; (3) the CHAD has favoured the appearance of sarcoidosis; (4) there is a common aetiologic agent or immunologic alteration that has favoured both diseas es.…”

Interleukin 6 dependence of spontaneous in vitro differentiation of B cells from patients with IgM gammapathy.

“…P its early discovery. This course has been observed, for instance, in patients with autoimmune hemolytic anemia and monoclonal IgM with cold agglutinin activity (21) and in patients with a peripheral neuropathy and serum monoclonal IgM directed to MAG (22). Whether these diverse clinicopathological patterns associated with monoclonal IgM correspond to two successive stages of the same lymphoproliferative process remains unsettled.…”