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A case of γ 3 heavy chain disease with vacuolated plasma cells: A clinical, immunological, and ultrastructural study

par Feremans, Walter ;Caudron, M.;Bieva, Cl
Référence Journal of Clinical Pathology, 32, 4, page (334-343)
Publication Publié, 1979

Article révisé par les pairs

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Résumé :

A patient with lambda Bence-Jones proteinuria, Waldenstrom's macroglobulinaemia, and Franklin's disease (γ HCD), but without clinical evidence of a lymphoproliferative disorder, is presented. The serum contained two distinct immunoglobulin abnormalities: a monoclonal immunoglobulin M (IgM) of lambda type, and a protein fragment which was immunologically related to immunoglobulin G (IgG) and devoid of light chain activity. This γ HCD protein belongs to the γ 3 subclass with a molecular weight of approximately 60 000 daltons. The urine contained a Bence-Jones lambda protein as well as the γ HCD fragment. The two paraproteins were probably secreted by two different malignant clones. Ultrastructural study revealed pathological vacuolated plasma cells of a sort that has hitherto been principally described in association with μ HCD. The mechanism of the intracellular storage of pathological immunoglobulins is discussed in the light of the ultrastructural study.