Disorders related to monoclonal protein tissue deposition
Disease . | M-protein . | Ultrastructure . | Target organs/systems . | Association with MM or WM . | Sources . |
|---|---|---|---|---|---|
| Amyloidosis | κ/λ ratio, 1:3 | Fibrils (8-12 nm in diameter) | Kidney, heart, liver, PNS, ANS | 10%-20% | Kyle and Gertz,16 Obici et al17 |
| LCDD | κ/λ ratio, 3:1 | Amorphous | Kidney≫heart, liver, PNS | 40%-65% | Buxbaum and Gallo,49 Pozzi et al,55 Lin et al,61 Heliman et al62 |
| Crystal-storing histiocytosis | κ/λ ratio, 9:1 | Crystals (intracellular) | Kidney (adult Fanconi syndrome), BM, LN, liver, spleen and diverse additional sites | Frequently associated with smoldering MM or lymphomas | Lebeau et al,66 Messiaen et al72 |
| Cryoglobulinemia type I | IgG > IgM, IgA | Granular often with substructure | Vessels, kidney | Frequently associated with MM or WM | Karras et al74 |
| Cryocrystalglobulinemia | IgG | Crystals | Vessels, kidney, synovium | Various B-cell disorders, including MM | Papo et al,75 Ball et al77 |
| GOMMID* | Mostly IgG | Microtubules (20-60 nm) | Kidney | Frequently associated with CLL | Bridoux et al76 |
Disease . | M-protein . | Ultrastructure . | Target organs/systems . | Association with MM or WM . | Sources . |
|---|---|---|---|---|---|
| Amyloidosis | κ/λ ratio, 1:3 | Fibrils (8-12 nm in diameter) | Kidney, heart, liver, PNS, ANS | 10%-20% | Kyle and Gertz,16 Obici et al17 |
| LCDD | κ/λ ratio, 3:1 | Amorphous | Kidney≫heart, liver, PNS | 40%-65% | Buxbaum and Gallo,49 Pozzi et al,55 Lin et al,61 Heliman et al62 |
| Crystal-storing histiocytosis | κ/λ ratio, 9:1 | Crystals (intracellular) | Kidney (adult Fanconi syndrome), BM, LN, liver, spleen and diverse additional sites | Frequently associated with smoldering MM or lymphomas | Lebeau et al,66 Messiaen et al72 |
| Cryoglobulinemia type I | IgG > IgM, IgA | Granular often with substructure | Vessels, kidney | Frequently associated with MM or WM | Karras et al74 |
| Cryocrystalglobulinemia | IgG | Crystals | Vessels, kidney, synovium | Various B-cell disorders, including MM | Papo et al,75 Ball et al77 |
| GOMMID* | Mostly IgG | Microtubules (20-60 nm) | Kidney | Frequently associated with CLL | Bridoux et al76 |
MM indicates multiple myeloma; WM, Waldenström macroglobulinemia; GOMMID, glomerulonephritis with organized microtubular monoclonal immunoglobulin deposits; PNS, peripheral nervous system; ANS, autonomic nervous system; ≫, much more frequent than; BM, bone marrow; LN, lymph nodes; >, more frequent than; CLL, chronic lymphocytic leukemia
Cryoglobulins are sometimes, but not always, detectable in patients with GOMMID