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MIM.134570 6p25-p24

Tuesday 14 October 2003

factor XIIIa, F13A1


- Juvenile xanthogranuloma (CD68+/S100-/CD1a-) with unusual nuclear factor XIIIA positivity (clone AC-1A1).


- subunit A functions as an intracellular enzyme in platelets and monocytes/macrophages
- cytoskeletal remodelling associated with the activation stages of platelets
- mainly localised in the cytoplasm, in association with cytoskeletal filaments, but at a relatively early stage of macrophage differentiation it also appears transiently in the nucleus

Expression profile

- platelets
- megakaryocytes
- monocytes/macrophages
- papillary dermal dendrocytes

Pathology entities:

- congenital factor XIII deficiency

Immunochemistry in tumoral pathology

- dermatofibroma (benign fibrous histiocytom a)
- angiofibroma (fibrous papule )
- juvenile xanthogranuloma (12717244)
- aneurysmal fibrous histiocytoma (12484439)
- solitary fibrous tumor (12357210)