Haemophagocytic Syndrome Treated With Cyclosporin A: A T Cell Disorder?

Splenectomy in haemophagocytic lymphohistiocytosis: report of histopathological changes with CD19+ B‐cell depletion and therapeutic results

“…Some authors have attributed splenomegaly to excessive cytokine activity in studies of animal experiments, clinical application of IL-2 and in patients with malignancy-related splenomegaly (Ratcliffe et al, 1992;Athanassakis & Vassiliadis, 1995, Pozniak et al, 1995. Although none of our HLH patients had mass lesions in the spleen at the time of splenectomy, all showed increased serum levels of interferon (IFN)-gamma, sIL-2R or both (Table II).…”

“…T cells or NK cells (Chan & Ng, 1989;Oyama et al, 1989;Okuda et al, 1991;Imashuku et al, 1997). That is, the hyperstimulated granular lymphocytes could induce apoptosis of B cells through the Fas±FasL system (Tanaka et al, 1995(Tanaka et al, , 1997Elenitoba-Johnson et al, 1998;Hasegawa et al, 1998;Yamashita et al, 1998).…”

Tuberculosis-Associated Haemophagocytic Syndrome: A Report of Two Cases and a Review of the Literature

“…Jaffe et al [16] presented data indicating that the systemic activation of histiocytes in HS associated with lymphoma probably occurs as a result of macrophage-activating lymphokines released from neoplastic lymphocytes. Oyama et al [17] presented data indicating that there is an alteration in T cell function in HS with uncontrolled T cells producing large amounts of interfer on (IFN)-y and other lymphokines. Elevated levels of IFN-y, soluble interleukin (IL)-2 receptor (sIL-2R), solu ble CD8, and macrophage-colony stimulating factor (M-CSF) have been found in patients with HS [18][19][20][21], The elevated levels of sIL-2R and sCD8 in active HS, and the normal levels observed in remission, indicate that an acti vation of T cells, especially CD8-positive T cells, contrib utes to the development of HS [21], Uncontrolled IFN-y and M-CSF can lead macrophages to a haemophagocytosing and tumour-necrosis-factor-a-and IL-6-secreting state [17,21], Successful treatment of the syndrome with cyclosporin A or VP-16 (etoposide) and méthylpredniso lone would favour this suggestion [10,17,22].…”

IVIG Treatment of Adenovirus Infection-Associated Macrophage Activation Syndrome in a Two-Year-Old Boy: Case Report and Review of the Literature

“…The mode of action of high-dose immunoglobulin in this application is not yet fully understood and subject of ongoing investigation [8,26]. In addition, other forms of causal treatment, like blockade of soluble IL-2 receptor alpha by administration of anti-IL-2R antibody or administration of anti-thymocyte globulin (ATG), as well as plasmapheresis or anti-TNF-α compounds, have been attempted in experimental approaches to inhibit the pathologic cytokine feedback loop between T cells and monocytes leading to macrophage activation (see below) [25,[28][29][30][31][32]. These additional treatment options certainly require clinical evaluation in study settings in the near future.…”