Table 1.
Selected clinical and biologic presenting features of St. Jude patients with ETP-ALL
| Pt | Age (yr) |
Sex | WBC (× 103/ mm3) |
Mediastinal mass |
FAB typea |
EGIL scoreb | TCR gene rearrangementc | Karyotype | |||
|---|---|---|---|---|---|---|---|---|---|---|---|
| T-cell | Myeloid | TCRB | TCRG | TCRD | |||||||
| 1 | 11.9 | M | 76.7 | No | L2 | 4 | 3 | - | - | - | 46,XY,del(11)(q23)[2]/46,XY[29].nuc ish[MLLx2] |
| 2 | 17.3 | M | 9.1 | No | L1 | 5 | 1 | - | - | - | 46,XY[11] |
| 3 | 15.4 | M | 3.8 | No | L2 | 4.5 | 1 | - | - | - | 54,XY,+Y,+4,add(5)(p15),+8,+9,+19,+21,+21, +22[14]/46,XY[14] |
| 4 | 16.0 | M | 41.9 | No | L1 | 4 | 1 | - | - | - | 47,XY,t(3;11)(q12;p15),+19[15]/46,XY[1] |
| 5 | 17.0 | M | 4.5 | No | L2 | 4 | 1 | - | - | - | 46,XY[25] |
| 6 | 13.8 | F | 2.8 | No | L1 | 5.5 | 1 | - | - | - | 47,XX,+4,t(10;12)(p11.2;q15)[3]/47,XX,idem, del(5)(q22q35)[3]/46,XX[14] |
| 7 | 10.4 | M | 460.5 | Yes | L2 | 4.5 | 1 | - | - | - | 46,XY,del(5)(q22q33),+10,del(13)(q12q14), add(15)(p11.1),del(17)(p11.2),−19[12]/46,XY[3] |
| 8 | 16.9 | M | 273 | Yes | L2 | 6 | 2 | - | - | - | 46,XY,del(5)(q13q22),inv(12)(p11.2q24.1), del(13)(q12q22)[8]/46,idem,del(1)(q21q42)[10]/46,XY[2] |
| 9 | 10.1 | M | 2.2 | No | L1 | 4.5 | 2 | Yes | Yes | Yes | 47,XY,der(1)add(1)(p36.3)add(1)(q32),t(4;7) (q21;p22),del(9)(p13),der(11)add(11)(p13)add(11)(q21),add(12)(p11.2),add(18)(q23),+mar[12]/47,idem,-der(1),-der(11), add(19)(p13.3)[7]/ 46,XY[6] |
| 10 | 7.9 | M | 44.8 | No | L1 | 6 | 3 | Yes | No | No | 46,XY,del(1)(p22p32),inv(2)(p11.2q13)c,der(4)t(4;10)(q21;q26),der(5)t(5;10) (p13;p11.2),der(10) t(4;10;4;10;5)(4qter->4q?::10p11.2->10q11.2::4q?::10q::5p13->5pter)[18]/ 46,XY,inv(2)(p11.2q13)c[2] |
| 11 | 3.4 | M | 31.8 | No | L2 | 3.5 | 1 | Yes | No | No | 47,XY,+9,der(11)del(11)(p11.2p15)inv(11)(p11.2q22)[19]/46,XY[1] |
| 12 | 12.8 | F | 16.6 | No | L1 | 6 | 1 | No | No | Yes | 46,XX,t(4;11)(q21;p15),del(5)(q22q35),del(12) (p12)[20] |
| 13 | 6.2 | F | 63.8 | No | L1 | 3.5 | 3 | Yes | No | No | 47,X,-X, ins(12;9)(p12;q13q34),+16,+19[9]/48,X,del(X) (q24),idem[9] |
| 14 | 10.6 | M | 53.8 | No | L1 | 4.5 | 2.5 | No | No | No | 46,XY[20] |
| 15 | 12.1 | M | 181.6 | No | L1 | 3.5 | 2 | Yes | Yes | Yes | 46,XY,add(7)(q35),del(11)(q21q23.1),del(13) (q12q22)[20].nuc ish [MLLx2] |
| 16 | 18.9 | M | 13.1 | No | L1 | 6 | 1 | Yes | No | No | 46,XY,+6,inv(10)(p13q22),add(12)(p13),der(12) t(12;14)(p13;q11.2),del(13)(q22q24),−14[17]/46,XY[3] |
| 17 | 9.9 | M | 3.9 | No | L1 | 4 | 3 | Yes | No | Yes | 48,XY,+19,+22[20] |
Morphology according to the French-American-British (FAB) classification of ALL. Unusual morphologic features (not seen in typical ALL) included prominent surface blebs (n = 5), associated myeloid dysplasia with dysplastic eosinophils (n = 2), phagocytosis by blasts of platelets and nucleated elements (n = 2), and cytochemical myeloperoxidase expression in 1% to 2 % of putative leukemic blasts (n = 4).
Scoring for T-lymphoid or myeloid cell differentiation follows criteria of the European Group for the Immunological Characterization of Leukemias (EGIL) and World Health Organization.35 Findings indicative of T-ALL include CD3 expression, score of 2; CD2, CD5, CD8 or CD10 expression, score of 1, and TdT, CD7 or CD1a expression, score of 0.5. Those indicative of AML included myeloperoxidase expression, score of 2, CD117, CD13, CD33 or CD65 expression, score of 1, and CD14, CD15 or CD64 expression, score of 0.5. Leukemias with scores greater than 2 for myeloid and 1 for lymphoid are termed biphenotypic by EGIL criteria.
Dashes indicate not done.