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Monoclonal Gammopathies and the Kidney (Hardcover, 2003 ed.)
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Monoclonal Gammopathies and the Kidney (Hardcover, 2003 ed.)
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Table 1 Comparison of MIDD and immunoglobulinic amyloidosis MIDD
Amyloidosis Presentation Multivisceral or predominant involvement
of an organ (kidney) Underlying disease Monoclonal
immunoproliferative disorders, mostly myeloma. Monoclonal bone
marrow plasma cell populations without overt malignancy.
Possibility of 'non-secretory' forms. Deposits by EM (Granular)
non-organized Fibrillar (13 pleated sheets) Spectrum LCDD : L
chains, most K, either AL-amyloidosis : fragments (?)* of or
apparently enlarged, normal-sized normal-sized or apparently
enlarged or short. Frequent glycosylation L chains most A. Frequent
and polymeration. glycosylation.Abnormal susceptibility to
proteolysis. LHCDD : associated H chains Cases with associated
short H chains (short by biosynthesis by biosynthesis experiments.
experiments). HCDD: short H chains. AH-amyloidosis : short H
chains. L chain isotypy V K IV predominance. VAVI C A3predominance.
V region mutations Yes Yes *Found in necropsy material; whether
they exist in vivo is doubtful. 3 After Preud'homme et al.
AL-amyloidosis). Therefore, we believe' that the term MIDD should
be restricted to this pathological entity, whatever the Ig content
of the deposits, light 4 5 ) or both (LHCDD ). However, the chains
(LCDD), heavy chains (HCDD New York group used the denomination
MIDD for both Randall's disease and immunoglobulinic
amyloidosis'S". The two processes have many features in common
(Table I) and they can be associated in the same patients.
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