Table 1.
Comparison of demographic and clinical characteristics between clinical subgroups in the combined prospective and retrospective cohorts
| Group* | FP (n = 16) | MHES (n = 13) | SR (n = 16) |
|---|---|---|---|
| Demographics | |||
| Median age, years (range) | 39 (17–61) | 43 (3–84) | 43 (11–65) |
| Sex, M/F (%) | 15/1 (94/6) | 6/7 (41/59) | 6/11 (54/46) |
| Race | |||
| African American | 3 | 4 | 4 |
| Asian | 1 | 1 | 1 |
| White | 11 | 7 | 10 |
| Hispanic | 1 | 1 | 1 |
| Clinical manifestations | |||
| Cardiac | 4 | 4 | 3 |
| Splenomegaly | 7/14 | 4/13 | 1/15 |
| Laboratory parameters | |||
| Median AEC x 109/l* (n; range) | 7.707 (16; 4.342–27.555) | 6.42 (13; 1.57–150.48) | 4.747 (16; 1.29–70.29) |
| Median peak AEC × 109/l (n; range) | 12.117 (16; 4.830–120.0) | 13.226 (13; 3.76–151.0) | 8.95 (16; 2.856–82.0) |
| Serum tryptase, ng/mL (n; range) | 19.6 (12; 3.5–47.5) | 6.22 (12; 0.99–27) | 4.63 (16; 0.99–19.1) |
| Serum B12, pg/mL (n; range) | 2966 (14; 313–27529) | 1.28 (12; 372–5055) | 711 (16; 368–1316) |
| Serum IgE, pg/mL (n; range) | 12 (11; 1.5–5027) | 78 (11; 8–1854) | 223 (16; 3.9–42874) |
| Serum LDH, U/l (n; range) | 279 (11; 123–663) | 290 (11; 100–587) | 235 (15; 126–600) |
| Clonal TCR rearrangement | 2/16 | 1/13 | 6/16 |
| Aberrant T lymphocyte population by flow | 0/16 | 1/13** | 4/16*** |
| Cytogenetic abnormality | 1/14 | 3/13 | 1/14 |
*
FP—FIP1L1-PDGFRA-associated myeloid neoplasm, MHES—FIP1L1-PDGFRA-negative, myeloid HES, SR—FIP1L1-PDGFRA-negative, nonmyeloid, corticosteroid nonresponder. Abbreviations: AEC, absolute eosinophil count; TCR, T-cell receptor; LDH, lactate dehydrogenase; IgE, immunoglobulin E.
**
Large granular lymphocytes.
***
CD3dimCD4+.