Khai Li Chai1*, Thomas Morris2 and Ali Bazargan1
1Department of Hematology, St Vincent’s Hospital, Melbourne, Australia
2Department of Neurosurgery, St Vincent’s Private Hospital, Melbourne, Australia
Hairy cell leukemia is a rare B cell lymphoproliferative disorder. Patients typically present with peripheral cytopenias, circulating leukemia cells, marked splenomegaly and marrow infiltration. We describe a case of a 68 year old man who presented solely with psychiatric symptoms of delusions, without the classical features of cytopenias or splenomegaly. Initial imaging revealed involvement with lymphoma at multiple nodal and extranodal sites, including the base of skull, skeletal bone, bone marrow and pleura. Histology of the lesions from the base of skull, bone marrow and iliac bone revealed small B cells with the typical immunophenotype for hairy cell leukemia, but with aberrant expression of cyclin D1. Only rare cases of skeletal bone disease and neurological involvement have been previously reported in the literature. He achieved only partial response to initial treatment with cladribine, and subsequently achieved complete metabolic response following treatment with rituximab. He experienced no toxicities with treatment and remains in complete remission at 12 months following treatment. This case highlights that careful hematopathological assessment and clinical correlation is required for accurate diagnosis of unusual cases of hairy cell leukemia. In addition, this case also demonstrates that a combination of cladribine and rituximab therapy can be effective in deepening frontline response in patients with hairy cell leukemia without significant treatment-related toxicity.
Chai KL, Morris T, Bazargan A. A Case Report of Hairy Cell Leukemia: An Unusual Presentation. Ann Clin Case Rep. 2017; 2: 1412.