Demographic and clinical data of 10 patients with systemic mastocytosis
. | A . | B . | C . | D . | E . | F . | G . | H . | I . | J . |
|---|---|---|---|---|---|---|---|---|---|---|
| Sex, age, y | M, 61 | F, 57 | F, 48 | M, 54 | M, 51 | M, 64 | F, 32 | F, 44 | F, 75 | F, 46 |
| Year of diagnosis of systemic mastocytosis | 1999 | 1996 | 2002 | 1996 | 2001 | 2002 | 1999* | 2002 | 1998 | 2000 |
| Previous cytoreductive therapy† | No | No | No | No | No | No | IFN, PRD | Hydrea | IFN, PRD | No |
| Skin symptoms | + (P, D) | + (P, D) | + (P, D) | + (P, D) | — | + (P, D) | + (P, D) | — | + (P, D) | + (P, D) |
| Urticaria pigmentosa | + | + | + | + | + | — | +* | — | + | + |
| Flushing | + | + | + | + | + | — | + | — | + | + |
| Syncope/anaphylaxis | + | — | — | + | — | — | + | — | + | — |
| Gastric/abdominal complaints | + | + | + | — | + | — | + | + | + | + |
| Diarrhea | — | — | + | + | + | + | — | — | + | + |
| Bone symptoms/osteoporosis | + | + | + | — | + | + | — | — | — | — |
| Weight loss | + | — | — | + | + | — | — | + | — | + |
| Severe fatigue | + | + | + | — | + | + | + | + | + | + |
| Anemia | — | — | — | — | + (9.4 g/dL) | — | + (9.9 g/dL) | + (8.5 g/dL) | — | — |
| Thrombocytopenia | — | — | — | — | + (117) | — | — | — | — | — |
| Bone marrow infiltration | 15% | 10% | 10% | 20% | 75% | 15% | 60% | 1%-15% | >60% | 35% |
| Hepatosplenomegaly | — | — | — | — | + | + | + | + | — | — |
| Lymphadenopathy | — | — | — | — | + | — | — | — | — | + |
| WHO classification3 | ISM* | ISM | ISM | SM-AHNMD* | ASM* | SM-AHNMD | ASM | SM-AHNMD | SSM* | ASM |
| Tryptase, N < 13.5 μg/L) | 120 | 34 | 200 | 100 | 497 | 270 | > 200 | 170 | 92 | 165 |
| Urinary M-histamine, N < 150 μM/M creatine | 489 | 447 | 354 | 1478 | 4686 | 1220 | 4094 | 4652 | 6506 | 1928 |
| c-kit mutation | NT* | NT | NT | NT | + | Absent | + | + | NT | NT |
| Associated hematologic disorder | — | — | — | MDS, +8 | Atyp MKC | Atyp CML, +8 | — | Atyp CML | — | — |
. | A . | B . | C . | D . | E . | F . | G . | H . | I . | J . |
|---|---|---|---|---|---|---|---|---|---|---|
| Sex, age, y | M, 61 | F, 57 | F, 48 | M, 54 | M, 51 | M, 64 | F, 32 | F, 44 | F, 75 | F, 46 |
| Year of diagnosis of systemic mastocytosis | 1999 | 1996 | 2002 | 1996 | 2001 | 2002 | 1999* | 2002 | 1998 | 2000 |
| Previous cytoreductive therapy† | No | No | No | No | No | No | IFN, PRD | Hydrea | IFN, PRD | No |
| Skin symptoms | + (P, D) | + (P, D) | + (P, D) | + (P, D) | — | + (P, D) | + (P, D) | — | + (P, D) | + (P, D) |
| Urticaria pigmentosa | + | + | + | + | + | — | +* | — | + | + |
| Flushing | + | + | + | + | + | — | + | — | + | + |
| Syncope/anaphylaxis | + | — | — | + | — | — | + | — | + | — |
| Gastric/abdominal complaints | + | + | + | — | + | — | + | + | + | + |
| Diarrhea | — | — | + | + | + | + | — | — | + | + |
| Bone symptoms/osteoporosis | + | + | + | — | + | + | — | — | — | — |
| Weight loss | + | — | — | + | + | — | — | + | — | + |
| Severe fatigue | + | + | + | — | + | + | + | + | + | + |
| Anemia | — | — | — | — | + (9.4 g/dL) | — | + (9.9 g/dL) | + (8.5 g/dL) | — | — |
| Thrombocytopenia | — | — | — | — | + (117) | — | — | — | — | — |
| Bone marrow infiltration | 15% | 10% | 10% | 20% | 75% | 15% | 60% | 1%-15% | >60% | 35% |
| Hepatosplenomegaly | — | — | — | — | + | + | + | + | — | — |
| Lymphadenopathy | — | — | — | — | + | — | — | — | — | + |
| WHO classification3 | ISM* | ISM | ISM | SM-AHNMD* | ASM* | SM-AHNMD | ASM | SM-AHNMD | SSM* | ASM |
| Tryptase, N < 13.5 μg/L) | 120 | 34 | 200 | 100 | 497 | 270 | > 200 | 170 | 92 | 165 |
| Urinary M-histamine, N < 150 μM/M creatine | 489 | 447 | 354 | 1478 | 4686 | 1220 | 4094 | 4652 | 6506 | 1928 |
| c-kit mutation | NT* | NT | NT | NT | + | Absent | + | + | NT | NT |
| Associated hematologic disorder | — | — | — | MDS, +8 | Atyp MKC | Atyp CML, +8 | — | Atyp CML | — | — |
IFN indicates interferon-alfa; PRD, prednisone; Hydrea, hydroxyurea; P, pruritus; D, Darier sign; ISM, indolent systemic mastocytosis; SM-AHNMD, systemic mastocytosis with associated hematologic non-mast cell disease; ASM, aggressive systemic mastocytosis; SSM, smoldering systemic mastocytosis; NT, not tested; MDS, myelodysplastic syndrome; +8, trisomy 8; Atyp MKC, atypical megakaryocytes; Atyp CML, atypical chronic myeloid leukemia; and—, symptom or sign not present.
In 1989 extensive urticaria pigmentosa (UP) was present, which disappeared after IFN-alfa therapy.
H1 and H2 blocking and cromoglycate were excluded.