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Introduction: Monoclonal light and heavy chain deposition disease is a rare syndrome distinct from light chain amyloid, which is defined by the presence of monoclonal deposits of immunoglobulins in various tissues., Case-Report: A 65-year-old man presented with renal symptoms due to membranoproliferative glomerulonephritis, associated with urticarial papules located on the arms and back. Histological examination of a skin biopsy specimen showed lymphocytic vasculitis. Direct immunofluorescence examination of kidney and skin lesions using anti-gamma 2 and anti-Kappa monoclonal antibodies, showed a similar staining on the basement membrane zone and vessel walls., Comments: As far as we know, this is the first documentation of monoclonal light and heavy chain deposition disease associated with a lymphocytic skin vasculitis and renal involvement caused by similar monoclonal deposits of immunoglobulins in the kidney and skin.

The authors report the case of a 72 year old woman affected by gamma heavy chain disease in whom the heavy chain was detected in the serum and the urine as well. Further study of the paraprotein showed that it belonged to the gamma 1 subclass. This observation was unusual because of the presence of a 15 year-long peripheral neuropathy and the existence of various auto-antibodies, especially intrinsic factor antibody.

Description of a case presenting as an inflammatory polyarthritis. A uterine sarcoma was detected one year later and led to the death of the patient. The abnormal protein belonged to the gammal subclass. Biosynthesis experiments, performed on the marrow plasma cells, showed that light chains were not produced and that the molecular weight of the monomeric unit of the serum protein was slightly smaller than that of the protein secreted in vitro, suggesting the occurrence of a secondary and limited extracellular proteolysis.

Specific bone lesions are not usually found in heavy chain alpha disease we report a case in which images identical to those seen in Parker and Jackson's sarcoma (bone lymphoma) were found. The malignant lymphomatous nature of the lesion could not be confirmed as a bone biopsy was not made, but its regression under chemotherapy makes the diagnosis almost certain. Indirectly, arguments in favour of this hypothesis can be drawn from the common cellular origin of bone lymphomas and monoclonal gammopathies. Furthermore, it is logical to assume that heavy chain alpha disease, as with most other immuno-proliferative syndromes, can be associated with malignant lymphomatous changes in the skeleton.

The authors report on a new case of Franklin's disease; the originality of which lies in the fact that it was associated with hypothyroïdism and mainly with B virus chronic active hepatitis. It seems likely that chronic antigenic stimulation during active hepatitis played role in the pathogenesis of the monoclonal gammapathy.