title: Massive abdominal lymphadenopathy in hairy cell leukaemia: a report of 12 cases.
abstract: Lymphadenopathy is an uncommon finding in hairy cell leukaemia (HCL). We report 12 HCL patients in whom relapse was associated with massive abdominal lymphadenopathy. All but one had long-standing HCL (range 3-25 years; median 10 years); in one it was discovered at presentation. Nine patients had been splenectomized and seven had previously been treated with 2'deoxycoformycin (DCF) and/or alpha-interferon (alpha IFN): three had achieved complete remission and four a partial response. The computerized tomography (CT) scan appearances were similar in all cases with a primary lymph node mass centred around the coeliac axis and involving upper para-aortic and retropancreatic regions. Histology and/or cytology confirmed nodal involvement by HCL in six patients. Large immature hairy cells were seen in both lymph nodes and bone marrow, suggesting a degree of transformation. Nine patients were treated with DCF: one had complete resolution, six responded with 50-90% reduction of the lymphadenopathy, one did not respond and one is still on treatment; alpha-IFN was used concomitantly or sequentially in two of the responders. One responding patient died of sepsis after four injections of DCF. Three patients received either alpha- or beta-IFN alone with no response. One elderly patient was not treated. Abdominal lymphadenopathy could be part of the natural history of HCL and/or may represent a transformation analogous to that seen in other low-grade lymphoproliferative disorders. Routine abdominal CT scanning should be part of the work up of all patients with HCL.
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007372:Interferons / Q000627:therapeutic use; D007943:Leukemia, Hairy Cell / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008206:Lymphatic Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015649:Pentostatin / Q000627:therapeutic use; D011860:Radiography, Abdominal; D014057:Tomography, X-Ray Computed
chemical_list: D015649:Pentostatin; D007372:Interferons