Management of adult Langerhans cell histiocytosis based on the characteristic clinical features

Table 1 Summary of 18 cases of non-pulmonary adult langerhans cell histiocytosis

Cases	Age (yr)/sex	Initial symptoms/signs	Subsequent symptoms/disease progression	Treatment			Outcome	Follow-up(mo)
				Surgicalresection	Radiation	Immune-chemoRx/HSCT
1	27/F	CDI/endocrine	None	Y	Y	Y4	LTF	-
2	25/F	CDI/endocrine	Nasal/thyroid/skin masses			Y	DOD1	216
3	35/F	MBL	MBL			Y/Y	ASAD	132+
4	37/M	CDI	MBL/skin lesions			Y/Y	ASAD	128+
5	40/M	MBL	MBL		Y	Y	ASAD	133+
6	46/F	Thyroid mass/spine (Th4)	MBL/LNs	Y		Y	AWAD	27+
7	40/M	Spine (C3)	Parietal bone	Y			ASAD	72+
8	40/M	Spine (C6)	Inguinal LNs			Y	AWAD	17+
9	27/F	Temporal bone	None	Y			ASAD	26+
10	31/M	Spine (Th1)	MBL			Y4	AWAD	108+
11	53/F	CDI	MBL	Y		Y	AWAD	166+
12	36/F	Endocrine	HPR mass			Y3	AWAD	28+
13	20/F	CDI/endocrine	Skin		Y	Y	ASAD	156+
14	36/F	CDI/endocrine	None			Y3	AWAD	52+
15	38/M	Hypothyroid	CDI/endocrine			Y3	AWAD	48+
16	46/M	Endocrine	CDI/MBL/ND-CNS			Y3	AWAD	60+
17	23/M	CDI	Lungs/ND-CNS			Y4	AWAD	72+
18	18/M	Spine (multiple)	LNs/Lungs		Y	Y/Y	DOD2	72

¹From disseminated intravascular coagulation;

²From Hodgkin disease;

³Systemic chemotherapy with 2-deoxychloroadenosine;

⁴Steroid alone with or without bisphosphonate. CDI: Central diabetes insipidus; MBL: Multiple bone lesions; HPR: Hypothalamic pituitary region; LNs: Lymph nodes; ND-CNS: Neurodegenerative central nervous system disease; Y: Yes; LTF: Lost to follow-up; ASAD: Alive without active disease; AWAD: Alive with active disease; DOD: Died of disease.

Table 2 Therapeutic options in the treatment of adult langerhans cell histiocytosis

Protocol	Drugs	Ref.
A1 protocol	VBL/PSL	[6]
JLSG-96	VCR/AraC/MTX/6MP/PSL	[13]
Cladribine-based	2CDA/PSL, 2CDA/AraC	[37-41]
Clofarabine-based	Clofarabine	[43,44]
JLSG-special C	VBL/MTX/6MP/PSL	[11]
Others	AraC alone	[45]
Molecular targetting	Vemurafenib	[46]
Bone therapy	Zoledronic acid	[47]

VBL: Vinblastine; PSL: Prednisolone; MTX: Methotrexate; 6MP: 6-mercatopurine hydrate; 2CDA: 2-deoxychloroadenosine.