Number of plasma cells per high-power field immunoreactive with free κ and λ immunoglobulin light chains in sections of spleen from patients with AL amyloidosis
| Case . | Site† . | Immunophenotype . | Splenic plasma cells . | Serum* . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| AL‡ . | Marrow plasma cells . | Spleen plasma cells . | Free κ . | Free λ . | P . | Free κ, μg/mL§ . | Free λ, μg/mL§ . | Free κ/λ ratio . | ||
| 1 | H | λ6 | λ6 | λ6 | 0.6 ± 0.2 | 87.1 ± 7.2 | <.001 | 1.57 | 396.2 | 0.004 |
| 2 | H | λ1 | λ1 | λ1 | 7.4 ± 1.1 | 15.2 ± 1.1 | <.001 | 8.96 | 525.8 | 0.017 |
| 3 | L | κ1 | κ1 | κ1 | 102.1 ± 3.7 | 9.0 ± 1.7 | <.001 | n.d. | n.d. | n.d. |
| 4 | N, LU | λ6 | λ6 | λ6 | 12.8 ± 1.6 | 28.3 ± 1.5 | <.001 | 12.6 | 277.8 | 0.045 |
| 5 | K | λ2 | λ2 | λ2 | 7.7 ± 0.7 | 15.3 ± 1.0 | <.001 | n.d. | n.d. | n.d. |
| 6 | I | κ1 | n.d. | κ1 | 4.9 ± 0.8 | 0.5 ± 0.3 | <.001 | 634.4 | 14.5 | 43.8 |
| 7 | K, TH | λ1 | λ1 | λ1 | 1.1 ± 0.3 | 16.1 ± 1.6 | <.001 | 22.6 | 71.7 | 0.32 |
| 8 | L | κ1 | κ1 | κ1 | 15.4 ± 1.4 | 1.7 ± 0.4 | <.001 | 332.8 | 43.7 | 7.62 |
| 9 | H, T | λ1 | λ1 | λ1 | 6.3 ± 0.8 | 11.1 ± 1.0 | <.001 | 46.1 | 258.3 | 0.18 |
| 10 | L, N | κ1 | κ1 | κ1 | 5.7 ± 1.7 | 0.3 ± 0.2 | <.01 | 738.3 | 50.1 | 14.7 |
| 11 | H | λ8 | λ8 | λ8 | 5.5 ± 0.8 | 9.5 ± 0.9 | <.01 | 17.5 | 471.7 | 0.04 |
| 12 | L, S, A | λ2 | λ2 | λ2 | 0.7 ± 0.3 | 2.2 ± 0.3 | <.01 | n.d. | n.d. | n.d. |
| 13 | K, H, S | λ3 | λ3 | λ3 | 1.9 ± 0.4 | 2.4 ± 0.5 | n.s. | 11.6 | 120.2 | 0.10 |
| 14 | H, K | λ2 | λ2 | —¶ | 0.3 ± 0.2 | 0.1 ± 0.1 | n.s. | 36.4 | 329.0 | 0.11 |
| 15 | L | κ4 | κ4 | — | 0.2 ± 0.1 | 0.3 ± 0.2 | n.s. | 103.4 | 123.9 | 0.83 |
| 16 | S, L | λ1 | n.d. | — | 0.3 ± 0.2 | 1.1 ± 0.6 | n.s. | n.d. | n.d. | n.d. |
| 17 | K | λ1 | λ1 | λ1 | 18.1 ± 1.6 | 14.5 ± 1.2 | n.s. | 15.0 | 844.3 | 0.02 |
| 18 | K | λ2 | λ2 | — | 0.1 ± 0.1 | 0.3 ± 0.1 | n.s. | 21.9 | 320.8 | 0.07 |
| 19 | K | λ3 | λ3 | λ3 | 2.1 ± 0.4 | 1.6 ± 0.3 | n.s. | 36.2 | 221.8 | 0.16 |
| 20 | K, H | λ3 | λ3 | — | 0.2 ± 0.1 | 0.0 | n.s. | 8.9 | 106.9 | 0.08 |
| 21 | L | λ2 | λ2 | λ2 | 1.7 ± 0.7 | 1.6 ± 0.5 | n.s. | 48.8 | 279.4 | 0.17 |
| 22 | L, S | κ3 | n.d. | κ3 | 3.6 ± 0.5 | 4.5 ± 0.9 | n.s. | n.d. | n.d. | n.d. |
| 23 | S | λ3 | n.d. | — | 0.1 ± 0.1 | 1.3 ± 0.3 | n.s. | n.d. | n.d. | n.d. |
| 24 | K, S, LN | κ1 | n.d. | κ1 | 2.9 ± 0.7 | 2.5 ± 0.6 | n.s. | n.d. | n.d. | n.d. |
| 25 | K, L, S | λ2 | n.d. | — | 0.3 ± 0.2 | 0.6 ± 0.2 | n.s. | n.d. | n.d. | n.d. |
| 26 | I | κ3 | n.d. | κ3 | 8.7 ± 0.8 | 9.9 ± 1.4 | n.s. | n.d. | n.d. | n.d. |
| Normal | 5.0 ± 0.6 | 3.5 ± 0.9 | n.s. | 4.2-13.0 | 16.4-127.3 | 0.07-0.28 | ||||
| Case . | Site† . | Immunophenotype . | Splenic plasma cells . | Serum* . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| AL‡ . | Marrow plasma cells . | Spleen plasma cells . | Free κ . | Free λ . | P . | Free κ, μg/mL§ . | Free λ, μg/mL§ . | Free κ/λ ratio . | ||
| 1 | H | λ6 | λ6 | λ6 | 0.6 ± 0.2 | 87.1 ± 7.2 | <.001 | 1.57 | 396.2 | 0.004 |
| 2 | H | λ1 | λ1 | λ1 | 7.4 ± 1.1 | 15.2 ± 1.1 | <.001 | 8.96 | 525.8 | 0.017 |
| 3 | L | κ1 | κ1 | κ1 | 102.1 ± 3.7 | 9.0 ± 1.7 | <.001 | n.d. | n.d. | n.d. |
| 4 | N, LU | λ6 | λ6 | λ6 | 12.8 ± 1.6 | 28.3 ± 1.5 | <.001 | 12.6 | 277.8 | 0.045 |
| 5 | K | λ2 | λ2 | λ2 | 7.7 ± 0.7 | 15.3 ± 1.0 | <.001 | n.d. | n.d. | n.d. |
| 6 | I | κ1 | n.d. | κ1 | 4.9 ± 0.8 | 0.5 ± 0.3 | <.001 | 634.4 | 14.5 | 43.8 |
| 7 | K, TH | λ1 | λ1 | λ1 | 1.1 ± 0.3 | 16.1 ± 1.6 | <.001 | 22.6 | 71.7 | 0.32 |
| 8 | L | κ1 | κ1 | κ1 | 15.4 ± 1.4 | 1.7 ± 0.4 | <.001 | 332.8 | 43.7 | 7.62 |
| 9 | H, T | λ1 | λ1 | λ1 | 6.3 ± 0.8 | 11.1 ± 1.0 | <.001 | 46.1 | 258.3 | 0.18 |
| 10 | L, N | κ1 | κ1 | κ1 | 5.7 ± 1.7 | 0.3 ± 0.2 | <.01 | 738.3 | 50.1 | 14.7 |
| 11 | H | λ8 | λ8 | λ8 | 5.5 ± 0.8 | 9.5 ± 0.9 | <.01 | 17.5 | 471.7 | 0.04 |
| 12 | L, S, A | λ2 | λ2 | λ2 | 0.7 ± 0.3 | 2.2 ± 0.3 | <.01 | n.d. | n.d. | n.d. |
| 13 | K, H, S | λ3 | λ3 | λ3 | 1.9 ± 0.4 | 2.4 ± 0.5 | n.s. | 11.6 | 120.2 | 0.10 |
| 14 | H, K | λ2 | λ2 | —¶ | 0.3 ± 0.2 | 0.1 ± 0.1 | n.s. | 36.4 | 329.0 | 0.11 |
| 15 | L | κ4 | κ4 | — | 0.2 ± 0.1 | 0.3 ± 0.2 | n.s. | 103.4 | 123.9 | 0.83 |
| 16 | S, L | λ1 | n.d. | — | 0.3 ± 0.2 | 1.1 ± 0.6 | n.s. | n.d. | n.d. | n.d. |
| 17 | K | λ1 | λ1 | λ1 | 18.1 ± 1.6 | 14.5 ± 1.2 | n.s. | 15.0 | 844.3 | 0.02 |
| 18 | K | λ2 | λ2 | — | 0.1 ± 0.1 | 0.3 ± 0.1 | n.s. | 21.9 | 320.8 | 0.07 |
| 19 | K | λ3 | λ3 | λ3 | 2.1 ± 0.4 | 1.6 ± 0.3 | n.s. | 36.2 | 221.8 | 0.16 |
| 20 | K, H | λ3 | λ3 | — | 0.2 ± 0.1 | 0.0 | n.s. | 8.9 | 106.9 | 0.08 |
| 21 | L | λ2 | λ2 | λ2 | 1.7 ± 0.7 | 1.6 ± 0.5 | n.s. | 48.8 | 279.4 | 0.17 |
| 22 | L, S | κ3 | n.d. | κ3 | 3.6 ± 0.5 | 4.5 ± 0.9 | n.s. | n.d. | n.d. | n.d. |
| 23 | S | λ3 | n.d. | — | 0.1 ± 0.1 | 1.3 ± 0.3 | n.s. | n.d. | n.d. | n.d. |
| 24 | K, S, LN | κ1 | n.d. | κ1 | 2.9 ± 0.7 | 2.5 ± 0.6 | n.s. | n.d. | n.d. | n.d. |
| 25 | K, L, S | λ2 | n.d. | — | 0.3 ± 0.2 | 0.6 ± 0.2 | n.s. | n.d. | n.d. | n.d. |
| 26 | I | κ3 | n.d. | κ3 | 8.7 ± 0.8 | 9.9 ± 1.4 | n.s. | n.d. | n.d. | n.d. |
| Normal | 5.0 ± 0.6 | 3.5 ± 0.9 | n.s. | 4.2-13.0 | 16.4-127.3 | 0.07-0.28 | ||||
H indicates heart; L, liver; LN, lymph node; LU, lung; K, kidney; I, intestine; TH, thyroid; T, tongue; A, adrenal; n.d., not determined; and n.s., not significant.
Specimens obtained less than 1 month before death, except in cases 9 and 21 (taken 3 and 4 months before death, respectively).
Principal site of amyloid deposition based on postmortem examination.
Determined by chemical analyses of amyloid extracts.
Values derived from a mAb-based enzyme-linked immunosorbent assay (ELISA).
No clonal plasma cells detected.