Table 1 Clinical data of 20 Mexican... | American Society of Hematology

Table 1

Clinical data of 20 Mexican children with HVLL

Case	Sex	Age	Clinical diagnosis	Symptoms at diagnosis						Treatment	Follow-up
Case	Sex	Age	Clinical diagnosis	Edema	Blisters	Ulcers	Crusts	Scars	FTTH + HS	Treatment	Follow-up
1a	M	10	ESVP							Thalidomide, topical steroids	9 y
1b	M	16	3 y history	−	+	−	+	+	+	Thalidomide, topical steroids	Remission and recurrences AwD
2	F	13	Lymphoma	−	+	+	−	−	−	—	LFU
3	M	4	EVSP							—	LFU
3	M	4	2 y history	−	+	−	+	+	+	—	LFU
4a	M	11	HVLL							Thalidomide, topical steroids	13 y
4b		20		−	+	−	+	+	+	Chemotherapy	Remission and recurrences
4c		24									AwD
5	M	1	Bullous urticaria	−	+	−	+	+	−	—	LFU
6	F	6	ESVP							Chloroquine	5 mo improvement
6	F	6	1.5 y history	+	+	+	+	+	+	Chloroquine	LFU
7	M	13	ESVP	−	+				+	—	LFU
8a	F	10	ESVP							Cyclophosphamide systemic steroids	3 y remission and recurrences
8b	F	13	Vasculitis, HMB	−	+	+	+	+	+	Cyclophosphamide systemic steroids	LFU
9	M	6	ESVP							Thalidomide, systemic steroids, cyclophosphamide	3 y improved
9	M	6	2 mo	+	+	+	+	+	+	Thalidomide, systemic steroids, cyclophosphamide	DwD*
10	M	8	Psoriasiform dermatitis/ESVP							Systemic steroids, IFN	13 mo improved
10	M	8	12 mo history	−	+	−	+	+	+	Systemic steroids, IFN	LFU
11	F	11	ESVP 2 y history							Thalidomide	4 y improved
11	F	15	PTCL, nos	+	+	+	+	+	−	—	LFU/DoD
12	M	15	ESVP							Thalidomide,	2 y Improved
12	M	15	12 mo history	+	−	+	+	+	−	Topical steroids, chloroquine	LFU
13	M	8	ESVP							Thalidomide, systemic steroids	1 mo improved
13	M	8	5 y history	+	+	+	+	+	−	Thalidomide, systemic steroids	LFU
14	M	12	ESVP							Thalidomide, systemic steroids, cyclophosphamide	2 mo improved
14	M	12	18 mo history	−	+	+	+	+	−	Thalidomide, systemic steroids, cyclophosphamide	LFU
15°	M	6	ESVP							Thalidomide, systemic steroids, methrotexate, cyclophosphamide	12 y
15b		7		+	+	+	+	+	+		AwD
15c		12
16°	M	8	ESVP							Thalidomide	4 y
16b		12	Nasal NK/T-cell lymphoma	+	+	+	+	+	+	Chemotherapy	DoD
16c		12
17	F	6	ESVP, HMB							—	LFU
17	F	6	4 y history	−	+	+	+	+	−	—	LFU
18	F	6	ESVP	+	+	+	+	+	−	—	LFU
19	M	8	ESVP, HMB							—	LFU
19	M	8	3 y history	+	+	+	+	+	−	—	LFU
20	M	15	HVLL, HMB							Chemotherapy	2 mo
20	M	15	2 y history	−	+	+	+	+	−	Chemotherapy	DoD

Case	Sex	Age	Clinical diagnosis	Symptoms at diagnosis						Treatment	Follow-up
Case	Sex	Age	Clinical diagnosis	Edema	Blisters	Ulcers	Crusts	Scars	FTTH + HS	Treatment	Follow-up
1a	M	10	ESVP							Thalidomide, topical steroids	9 y
1b	M	16	3 y history	−	+	−	+	+	+	Thalidomide, topical steroids	Remission and recurrences AwD
2	F	13	Lymphoma	−	+	+	−	−	−	—	LFU
3	M	4	EVSP							—	LFU
3	M	4	2 y history	−	+	−	+	+	+	—	LFU
4a	M	11	HVLL							Thalidomide, topical steroids	13 y
4b		20		−	+	−	+	+	+	Chemotherapy	Remission and recurrences
4c		24									AwD
5	M	1	Bullous urticaria	−	+	−	+	+	−	—	LFU
6	F	6	ESVP							Chloroquine	5 mo improvement
6	F	6	1.5 y history	+	+	+	+	+	+	Chloroquine	LFU
7	M	13	ESVP	−	+				+	—	LFU
8a	F	10	ESVP							Cyclophosphamide systemic steroids	3 y remission and recurrences
8b	F	13	Vasculitis, HMB	−	+	+	+	+	+	Cyclophosphamide systemic steroids	LFU
9	M	6	ESVP							Thalidomide, systemic steroids, cyclophosphamide	3 y improved
9	M	6	2 mo	+	+	+	+	+	+	Thalidomide, systemic steroids, cyclophosphamide	DwD*
10	M	8	Psoriasiform dermatitis/ESVP							Systemic steroids, IFN	13 mo improved
10	M	8	12 mo history	−	+	−	+	+	+	Systemic steroids, IFN	LFU
11	F	11	ESVP 2 y history							Thalidomide	4 y improved
11	F	15	PTCL, nos	+	+	+	+	+	−	—	LFU/DoD
12	M	15	ESVP							Thalidomide,	2 y Improved
12	M	15	12 mo history	+	−	+	+	+	−	Topical steroids, chloroquine	LFU
13	M	8	ESVP							Thalidomide, systemic steroids	1 mo improved
13	M	8	5 y history	+	+	+	+	+	−	Thalidomide, systemic steroids	LFU
14	M	12	ESVP							Thalidomide, systemic steroids, cyclophosphamide	2 mo improved
14	M	12	18 mo history	−	+	+	+	+	−	Thalidomide, systemic steroids, cyclophosphamide	LFU
15°	M	6	ESVP							Thalidomide, systemic steroids, methrotexate, cyclophosphamide	12 y
15b		7		+	+	+	+	+	+		AwD
15c		12
16°	M	8	ESVP							Thalidomide	4 y
16b		12	Nasal NK/T-cell lymphoma	+	+	+	+	+	+	Chemotherapy	DoD
16c		12
17	F	6	ESVP, HMB							—	LFU
17	F	6	4 y history	−	+	+	+	+	−	—	LFU
18	F	6	ESVP	+	+	+	+	+	−	—	LFU
19	M	8	ESVP, HMB							—	LFU
19	M	8	3 y history	+	+	+	+	+	−	—	LFU
20	M	15	HVLL, HMB							Chemotherapy	2 mo
20	M	15	2 y history	−	+	+	+	+	−	Chemotherapy	DoD

AwD, alive with disease; DoD, died of disease: DwD, died without disease; ESVP, edematous, scarring vasculitic panniculitis; FTTH, failure to thrive; HS, hepatosplenomegaly; IFN, interferon; LFU, lost to follow-up.

Died of hepatic failure, no active skin lesions.

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