Summary of pathologic findings of patients with POEMS syndrome
| . | Total cases (n = 143) . | Pretreatment cases (n = 67) . | Posttreatment cases (n = 76) . |
|---|---|---|---|
| Peripheral blood smear | |||
| Erythrocytosis (Hgb > 17.5 g/dL male, > 15.5 g/dL female) | 5 | 5 | 0 |
| Leukocytosis (WBC > 10.5 × 109/L) | 19 | 14 | 5 |
| Thrombocytosis (> 450 × 109/L) | 45 | 31 | 14 |
| Circulating PCs | 0 | 0 | 0 |
| Dysplastic changes | 0 | 0 | 0 |
| BM aspirate and biopsy findings | |||
| RBC atypia | 0 | 0 | 0 |
| Granulocytic atypia | 0 | 0 | 0 |
| BM cellularity | |||
| Normocellular | 85 | 43 | 42 |
| Hypercellular | 22 | 16 | 6 |
| Hypocellular | 36 | 8 | 28 |
| Lymphoid aggregate | 59 | 33 | 26 |
| PC rimming | 54/59 | 32/33 | 22/26 |
| Megakaryocyte (meg) | |||
| Cytologic atypia | 40 | 29 | 11 |
| Clusters present | 112 | 62 | 50 |
| > 5 clusters | 34/112 | 25/62 | 9/50 |
| Megs/hpf, range | 0.2-20.7 (median, 4.8) | (2.8-20.7, 6.3) | (0.2-11.5, 3.1) |
| Meg hyperplasia | 50 | 36 | 14 |
| Plasma cells | |||
| % PCs, range | < 5-60 (median, < 5) | < 5-60 (median, < 5) | < 5-30 (median, < 5) |
| PC atypia (binucleated, nucleoli, Russell or Dutcher bodies, large size) | 41 | 27 | 14 |
| Interstitial PC and perivascular | 36 | 6 | 30 |
| Small clusters | 64 | 34 | 30 |
| Large clusters | 43 | 27 | 16 |
| Monotypic λ | 72 | 40 | 32 |
| Monotypic κ | 4* | 4† | 0 |
| Polytypic light chain | 67 | 23 | 44 |
| Amyloid | 0/49 | 0/33 | 0/16 |
| HHV8 | 0/32 | 0/20 | 0/12 |
| JAK2V617F | 0/43 | NA | NA |
| . | Total cases (n = 143) . | Pretreatment cases (n = 67) . | Posttreatment cases (n = 76) . |
|---|---|---|---|
| Peripheral blood smear | |||
| Erythrocytosis (Hgb > 17.5 g/dL male, > 15.5 g/dL female) | 5 | 5 | 0 |
| Leukocytosis (WBC > 10.5 × 109/L) | 19 | 14 | 5 |
| Thrombocytosis (> 450 × 109/L) | 45 | 31 | 14 |
| Circulating PCs | 0 | 0 | 0 |
| Dysplastic changes | 0 | 0 | 0 |
| BM aspirate and biopsy findings | |||
| RBC atypia | 0 | 0 | 0 |
| Granulocytic atypia | 0 | 0 | 0 |
| BM cellularity | |||
| Normocellular | 85 | 43 | 42 |
| Hypercellular | 22 | 16 | 6 |
| Hypocellular | 36 | 8 | 28 |
| Lymphoid aggregate | 59 | 33 | 26 |
| PC rimming | 54/59 | 32/33 | 22/26 |
| Megakaryocyte (meg) | |||
| Cytologic atypia | 40 | 29 | 11 |
| Clusters present | 112 | 62 | 50 |
| > 5 clusters | 34/112 | 25/62 | 9/50 |
| Megs/hpf, range | 0.2-20.7 (median, 4.8) | (2.8-20.7, 6.3) | (0.2-11.5, 3.1) |
| Meg hyperplasia | 50 | 36 | 14 |
| Plasma cells | |||
| % PCs, range | < 5-60 (median, < 5) | < 5-60 (median, < 5) | < 5-30 (median, < 5) |
| PC atypia (binucleated, nucleoli, Russell or Dutcher bodies, large size) | 41 | 27 | 14 |
| Interstitial PC and perivascular | 36 | 6 | 30 |
| Small clusters | 64 | 34 | 30 |
| Large clusters | 43 | 27 | 16 |
| Monotypic λ | 72 | 40 | 32 |
| Monotypic κ | 4* | 4† | 0 |
| Polytypic light chain | 67 | 23 | 44 |
| Amyloid | 0/49 | 0/33 | 0/16 |
| HHV8 | 0/32 | 0/20 | 0/12 |
| JAK2V617F | 0/43 | NA | NA |
Values are numbers unless otherwise indicated.
Hgb indicates hemoglobin; HHV-8, human herpes virus-8; hpf, high power field; NA, not available; PC, plasma cell; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes; RBC, red blood cell; and WBC, white blood cell.
One case with biclonal plasma cell populations: IgA λ-POEMS and IgG κ-multiple myeloma.