Table 4.
MCS; the 3 workshop cases
Case 230 | Case 241 | Case 326 | |
---|---|---|---|
Gender, age | Male, 61 years | Male, 65 years | Male, 38 years |
History | AML with trisomy 8 and mutations FLT3 S451F, IDH2 R140Q, and SRSF2 P95H, for which clinical and cytogenetic remission achieved after HSCT | Urothelial carcinoma of the renal pelvis treated by nephrotomy | Refractory T-cell lympoblastic leukemia/lymphoma (TCR-gamma delta+) treated with post chemotherapy and allogeneic HSCT |
Organs involved | Left inferior pubic ramus | Multiple (lytic) bone lesions | Small bowel, ascites, liver, and omental nodules |
IHC |
Positive: CD117 Weakly positive: tryptase Negative: CD2, CD3, CD25, CD34 |
Positive: CD30, tryptase, CD117, CD25 +/– Negative: CD20, CD79a, CD3, CD5, ALK, CD34, MPO, Glycophorin, FVIII |
Positive: tryptase, CD68 (KP-1), CD123, CD117, CD2, and CD25 Negative: TdT |
Flow |
Positive: CD45, CD33, CD117, CD13 Variable: CD38, CD64, Dim: CD7, CD4 (negative to partially dim) Negative: CD2, CD14, CD15, CD16, CD25, CD34, CD36, CD56, CD123, HLA-DR, CD11b (predominantly negative) |
Not available |
Positive: CD117, CD123, CD25, CD2, CD33 Dim: CD45, CD13 Negative: CD34, MPO, TdT |
Mutations | Imatinib-sensitive mutation KIT Y503_ F504insAY in exon 9, along with IDH2 R140Q and SRSF2 P95H mutations identical to the initial AML | No KIT D816V mutation | Complex karyotype: 78-89, XXXX, del(3) (p12p26), add(9)(p23), del(9)(p12-24), add(21) (p11.2)inc[cp 18] No KIT mutation |
Outcome | Treated with irradiation and daily imatinib for 5.5 months, on which he achieved complete remission. | Treated with midostaurine without effect, then dasatinib with 5-azacytidine; survival: 6 months | Palliative therapy Survival: 1 month |