We present a case of juvenile myelomonocytic leukemia (JMML) accompanied by immune-mediated hemophagocytic lymphohistiocytosis (HLH) and Kikuchi's disease, both as a paraneoplastic phenomenon. As this combination, to the best of our knowledge, has not been described before, consensus on preferable treatment is lacking. Our patient was treated with prednisolone according to the few described cases of HLH and Kikuchi's disease in non-JMML patients, resulting in disappearance of the clinical symptoms.

Autoimmune phenomena, Hemophagocytic lymphohistiocytosis, Juvenile myelomonocytic leukemia, Kikuchi's disease
dx.doi.org/10.1016/j.leukres.2005.12.027, hdl.handle.net/1765/68453
Leukemia Research: clinical and laboratory studies
Department of Pathology

Gerritsen, A, Lam, K.H, Schneider, E.M, & van den Heuvel-Eibrink, M.M. (2006). An exclusive case of juvenile myelomonocytic leukemia in association with Kikuchi's disease and hemophagocytic lymphohistiocytosis and a review of the literature. Leukemia Research: clinical and laboratory studies, 30(10), 1299–1303. doi:10.1016/j.leukres.2005.12.027