Enigmatic Kikuchi-Fujimoto Disease: A Comprehensive Review

Xavier Bosch, MD; Antonio Guilabert, MD; Rosa Miquel, MD; Elias Campo, MD

Disclosures

Am J Clin Pathol. 2004;122(1) 

In This Article

Immunohistochemical Features

The immunophenotype of KFD typically consists of a predominance of T cells, with very few B cells. There is a predominance of CD8+ cells over CD4+ cells, along with a decreased ratio in the affected areas of the lymph node (Figure 4), owing to the aforementioned intense CD8+ T-cell apoptosis in the necrotic foci. The histiocytes express histiocyte-associated antigens such as lysozyme, myeloperoxidase (MPO), and CD68 (Figure 5). This observation suggests that peripheral blood CD68+/MPO+ monocytes might be attracted to lymph nodes to fulfill the role of lacking granulocytes. It seems that the MPO needed for the inflammatory and cell-death mechanisms is supplied in this particular case by histiocytes. Striking plasmacytoid monocytes also are positive for cutaneous lymphocyte-associated antigen and CD68 but not for MPO.[49] They also express CD4 and CD74 and are positively stained by the pan-macrophage monoclonal antibody Kim1P. On the other hand, immunoblast cells in KFD-affected foci have the T-cytotoxic phenotype.[16,17,18,46,47]

Figure 4.

Kikuchi-Fujimoto disease. CD8 immunostaining showing a characteristic membrane pattern of T cells. Most CD8+ T (cytotoxic) cells are constituted by lymphoid cells and large immunoblasts (avidin-biotin-immunoperoxidase technique; EnVision, DakoCytomation, Carpinteria, CA) (CD8, original magnification x400).

Figure 5.

Kikuchi-Fujimoto disease. The histiocytes in the karyorrhectic foci are positive for the CD68 antigen (avidin-biotin-immunoperoxidase technique; EnVision, DakoCytomation, Carpinteria, CA) (CD68, original magnification x400).

Although KFD is considered to be a paracortical T-zone process, it is not uncommon to observe some reactive lymphoid follicles in the background, which can be interpreted as residual follicles or as a participation of the B-cell component in the disease process.[16] Some authors have observed monocytoid B-cell clusters in some cases of KFD.[1,2,16,18] Pileri and associates6 reported focal distention of sinuses by monocytoid B lymphocytes ("immature sinus histiocytes") in some patients with KFD.

In cases of KFD with cutaneous involvement, immunohistochemical analysis of skin lesions has revealed essentially the same findings as those in lymph nodes.[58,61]

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