Langerin, CD207

Langerin is a Type II transmembrane C-type lectin specific to Langerhans cells, which initiates the formation of Birbeck granules. PCR shows Langerin mRNA to be restricted to Langerhans cells in both normal tissues and in Langerhans cell histiocytosis2.

Immunohistochemical expression

It is expressed by the Langerhans dendritic cells of the epididymis, mucosae, lymphoid tissue (including thymus) and normal lung.

				Average number of positive cells per HPF (range)1
			number of cases studied	S-100	CD1a	Langerin
	Langerhans' cell histiocytosis of the lung	lesion	10	>100 (39 to >100)	>100 (37 to >100)	>100 (37 to >100)
	Langerhans' cell histiocytosis of the lung	uninvolved lung	10	12	7	9
	Extrapulmonary Langerhans' cell granulomatosis		4	>100 (27 to >100)	>100 (85 to >100)	>100 (70 to >100)
	Desquamative Interstitial Pneumonitis/Respiratory Bronchiolitis-Interstitial Lung Disease		7	6	4	7
	Hypersensitivity pneumonitis		7	9	5	8
	Usual Interstitial Pneumonitis		8	14	10	14
	Lymphocytic Interstitial Pneumonitis		4	18	2	3
	Cryptogenic Organising Pneumonia		5	15	8	8
	Eosinophilic Pneumonia		3	4	2	1
	Sarcoidosis		7	16	8	8
	Mycobacterial infection		3	18	7	6
	Fungal infection		2	21	2	3
	Viral infection		4	8	rare	rare
	Pleural fibrosis		3	7	3	4
	Normal lung		7	7	4	6

			Langerin	CD1a
	Langerhans cell histiocytosis		17/172, 24/243	16/172, 24/243
	Dermatopathic lymphadenopathy		12/123	12/123
	Monocyte / macrophage related	Haemophagocytic syndrome	0/72	0/72
		Fibrous histiocytoma	0/23
		Sinus histiocytosis with massive lymphadenopathy	0/42, 0/13	0/42
		Acute myelomonocytic leukaemia	0/82	0/82
		Acute monoblastic/monocytic leukaemia	0/72	0/72
		Chronic myelomonocytic leukaemia	0/102	0/102
		Histiocytic sarcoma	2/102	0/102
	Dendritic cell related	Juvenile xanthogranuloma	0/52, 0/43	0/52
		Interdigitating dendritic cell sarcoma /tumour	0/52	0/52
		Follicular dendritic cell sarcoma / tumour	0/82	0/82

In bronchoalveolar lavage fluid, the proportion of cells staining with langerin closely parallels the proportion staining with CD1a4.

Diagnostic utility

Diagnosis of Langerhans' cell granulomatosis of the lung and extrapulmonary Langerhans' cell granulomatosis.
Detection of Langerhans cells in bronchoalveolar lavage fluid for the diagnosis of Langerhans' cell histiocytosis of the lung4. A cut of of 5% has been suggested for the diagnosis of Langerhans' cell histiocytosis.

References

1 Sholl LM, Hornick JL, Pinkus JL, et al. Immunohistochemical analysis of langerin in langerhans cell histiocytosis and pulmonary inflammatory and infectious diseases. Am J Surg Pathol 2007; 31:947-52

2 Sean K. Lau, Immunohistochemical Expression of Langerin in Langerhans Cell Histiocytosis and Non-Langerhans Cell Histiocytic Disorders AJSP 2008; 32:615

3 Chikwava K,Jaffe R Langerin (CD207) staining in normal pediatric tissues, reactive lymph nodes, and childhood histiocytic disorders. Pediatr Dev Pathol 2004; 7:607-14

4 Smetana K, Jr., Mericka O, Saeland S, et al. Diagnostic relevance of Langerin detection in cells from bronchoalveolar lavage of patients with pulmonary Langerhans cell histiocytosis, sarcoidosis and idiopathic pulmonary fibrosis. Virchows Arch 2004; 444:171-4

This page last revised 12.7.2008.

©SMUHT/PW Bishop