Clinical Characteristics And Outcome Of Biphenotypic Acute Leukemia: 10 Case Reports And Literature Review

Figures & data

Table 1 Clinical Manifestations And Cytogenetic Abnormalities Of BAL Patients

UPID	Gender/Age	WBC (×10*9/L)	Hb(g/L)	Plt(×10*9/L)	BM Blast (%)	Cytogenetics	Fusion Genes	NGS	Diagnosis	Induction Chemo	Remission After Initial Chemo	Relapse	Status At End Follow-Up	OS (Month)
1	M/67	5.60	144.0	240	65.8	46, XY	Normal	ND	BAL, T/Myeloid lineage, with extra infusion	VDLP	PR	No	Alive	4
2	M/9	19.50	123.0	94	74.8	46, XY	Normal	ND	BAL, T/Monocytic lineage,	Unknown	Unknown	Unknown	Unknown	Unknown
3	F/24	10.80	74.0	72	27.5	46, XX	Bcr/Abl	ND	BAL, B/Myeloid lineage,	MEA	PR	Yes	Death	8
4	F/58	74.00	87.0	47	25.2	46, XX, t(9;22)(q34;q11)	Normal	Runx1 mutation	BAL, T/Myeloid lineage,	No	No CR	No	Death	2
5	M/22	82.00	75.0	26	80.8	46, XY	Normal	Runx1 mutation	BAL, T/Monocytic lineage, HSCT	IA	CR	No	Alive	33
6	M/3	14.20	76.0	35	24.2	45, XY, -7/46, idem, +8	Normal	ND	BAL, B/Monocytic lineage	VDLP	CR	Yes	Death	6
7	M/6	27.70	77.0	58	88.8	45, X,-Y,del(7)q32, t(8.21) (q22;q22)	Normal	ND	BAL, B/Myeloid lineage	VDLP	CR	Yes	Death	6
8	M/34	2.80	102.0	160	82.4	46, XY	Normal	ND	BAL, B/Myeloid lineage	VDLP	CR	Yes	Death	42
9	M/16	150.50	74.9	29	39.2	46, XY	Normal	JAK1, JAK3, FBXW7 mutation	BAL, T/B lineage	VDLP	CR	No	Alive	11
10	M/16	5.10	113.0	17	85.2	46, XY	Normal	ND	BAL, T/B lineage	VDLP	No CR	No	Death	4

Table 2 Clinical Manifestations Of BAL Patients Stratified By Immunophenotypes

	Total (n =10)	B+M type (n = 4)	T+M type (n = 4)	p value
Age, years (median, range)	25.5 (3–67)	17 (3–34)	39 (9–67)	N/A
Male (n, %)	8 (80.0)	3 (75.0)	3 (75.0)	1
WBC (×10*9/L) (median, range)	39.2±48.3(2.8–150.0)	13.9±10.4 (2.8–27.7)	45.3±38.4(5.6–82.0)	0.165
Hb (g/L) (Mean, range)	94.6±24.8(74.0–144.0)	82.3±13.2(74.0–102.0)	107.3±31.9(75.0–144.0)	0.198
Plate (×10*9/L) (Mean, range)	77.8±70.9 (17.0–240.0)	81.3±54.7(35.0–160.0)	101.8±96.5(26.0–240.0)	0.727
Blast cells in BM (%, range)	59.4±27.1(24.2–88.8))	55.7±34.6(24.2–88.8)	61.7±25.1(25.2–80.8)	0.791

Table 3 EGIL Or WHO Diagnosis Based On Cytochemical And Immunophenotype Data

UPID#	MPO Stain	Myeloid	T-Lineage	B-Lineage	Lymphoid	Hematopoietic	EGIL Diagnosis	WHO Diagnosis
1	Pos	CD43, CD117	CD3		CD10, TdT	CD34/HLA-DR	BAL, T/Myeloid lineage, with extra infusion	MPAL
2	Pos	CD38, CD58, CD64	CD2, cCD3, CD4, CD7, CD8				BAL, T/Monocytic lineage,	MPAL
3	Unknown	CD33, CD38, CD58,		CD22	CD79a	CD34/HLA-DR	BAL, B/Myeloid lineage,	MPAL
4	Pos	CD13, CD58, CD117	CD2, cCD3, CD7			CD34/HLA-DR	BAL, T/Myeloid lineage,	MPAL
5	Pos	CD13, CD33, CD38, CD117	CD2, cCD3, CD7		TdT	CD34/HLA-DR	BAL, T/Monocytic lineage, HSCT	MPAL
6	Pos	CD33, CD38, CD117		CD19, CD22	CD10, cCD79a, TdT	CD34/HLA-DR	BAL, B/Monocytic lineage	MPAL
7	Pos	CD13, CD33, CD38, CD58, CD71, CD123		CD19	CD79a	CD34/HLA-DR	BAL, B/Myeloid lineage	MPAL
8	Neg	CD13, CD33, CD38		CD19	CD10, CD79a	CD34/HLA-DR	BAL, B/Myeloid lineage	MPAL
9	Neg		CD2, cCD3, CD7	CD19	CD10, cCD79a		BAL, T/B lineage	MPAL
10	Neg		CD2, cCD3, CD7	CD19	cCD79a	CD34/HLA-DR	BAL, T/B lineage	MPAL

Table 4 Expression Of Cytochemical And Immunological Markers In BAL

		Total (%)	B+My (%)	T+My (%)	B+T (%)
Myeloid	MPO	7/10 (70)	3/4 (100)	2/4 (50)	1/2 (50)
	CD13	4/10 (40)	2/4 (50)	2/4 (50)	0/2 (0)
	CD33	6/10 (60)	4/4 (100)	1/4 (25)	1/2 (50)
	CD64	1/10 (10)	0/4 (0)	1/4 (25)	0/2 (0)
CD117	5/10 (50)	2/4 (50)	3/4 (75)	1/2 (50)
Lymphoid	CD10	4/10 (40)	2/4 (50)	2/4 (50)	1/2(100)
	CD79a	6/10 (60)	4/4 (100)	0/4 (0%)	2/2(100)
TdT	4/10 (40)	2/4 (50)	2/4 (50)	0/2 (0)
B-lineage	CD19	6/10 (60)	4/4 (100)	0/4 (0)	2/2(100)
CD22	2/10 (20)	2/4 (50)	0/4 (0)	0/2 (0)
T-lineage	CD2	4/10 (40)	0/4 (0)	3/4 (75)	2/2(100)
	cCD3/CD3	6/10 (60)	1/4 (25)	4/4 (100)	2/2(100)
	CD4	1/10 (10)	0/4 (0)	1/4 (25)	0/2 (0)
	CD7	4/10 (40)	0/4 (0)	2/4 (50)	2/2(100)
CD8	1/10 (10)	0/4 (0)	1/4 (25)	0/2 (0)
Hematopoietic	HLA-DR	8/10 (80)	4/4 (100)	3/4 (75)	1/2 (50)
	CD34	8/10 (80)	4/4 (100)	4/4 (100)	1/2 (50)
	CD43	1/10 (10)	1/4 (25)	0/4 (0)	0/2 (0)
	CD58	4/10 (40)	2/4 (50)	2/4 (50)	0/2 (0)
CD71	1/10 (10)	1/4 (25)	0/4 (0)	0/2 (0)

Figure 1 Kaplan–Meier plot of all patients’ overall survival.

Figure 2 Kaplan–Meier plot of patients’ overall survival: ALL-directed vs AML-directed chemotherapy.