Polyclonal B‐cell lymphocytosis and hypergammaglobulinemia in patients with gaucher disease

Abstract

Sera from 23 individuals with Gaucher disease (GD) were analyzed for hypergammaglobulinemia and oligoclonal and monoclonal gammopathies. Serum IgG level was elevated in 15/23 (65%) patients, and a diffuse hypergammaglobulinemia was present in 10/23 (43%) patients. An oligoclonal gammopathy was noted in six patients, and a monoclonal gammopathy in two. Lymphocyte subset analysis was also carried out in eight individuals with GD. Four of five individuals showed increased surface Ig-positive lymphocytes, while 7/7 were positive for either increased CD19- and/or CD20-positive lymphocytes. An eighth patient was found to have a B-cell leukemia. Statistical analysis of kappa and lambda histograms were suggestive of a monoclonal excess. However, restriction enzyme analysis of four individuals with GD and increased B cells failed to show any evidence of Ig gene rearrangements. Serum Ig abnormalities and perhaps B-cell lymphocytosis appear to be common in the GD patient population and are not associated with circulating monoclonal lymphocytes.

Gaucher disease and cancer incidence: a study from the Gaucher Registry

“…The association of Gaucher disease with polyclonal and monoclonal gammopathies has previously been reported. 16,19,34,36,38,42,47 It has been theorized that persistent and progressive glucocerebroside storage may cause chronic stimulation of the immune system and consequent lymphoproliferation. 4 However, the inflammatory response in Gaucher disease may in fact be independent of glucocerebroside deposition, as systemic inflammation is reported in glucocerebrosidase-deficient mice who demonstrate minimal storage of glucosyl ceramide.…”

Intravascular bone marrow accumulation in persistent polyclonal lymphocytosis: a misleading feature for B-cell neoplasm

“…1 By contrast, polyclonal persistent lymphocytosis is unusual and observed in such conditions as hyposplenic states, 2 rheumatoid arthritis, 3 Gaucher's disease 4 or idiopathic persistent polyclonal B-cell lymphocytosis (PPBL). This entity, also called B-cell lymphocytosis with binucleated lymphocytes, was initially reported by Gordon et al 5 in young to middle-aged women, usually smokers.…”

Manifestaciones oculares de la enfermedad de Gaucher: presentación de un caso y revisión del tema

“…Con base en el estudio posmortem de 20 pacientes mayores con EG tipo 1, se ha propuesto una posible asociación entre la EG y malignidades hematológicas (47,48). El mieloma múltiple ha sido la neoplasia más frecuente (aumento del riesgo en 5,9 veces).…”