Massive abdominal lymphadenopathy in hairy cell leukaemia: a report of 12 cases

Autor: Christine Costello, Daniel Catovsky, G. Flandrin, Estela Matutes, J. Mercieca, Francis Matthey, A. Brizard, Judith Behrens, K. Maclennan, S. Fairhead, S. Basu, E. Moskovic, S. Roath
Rok vydání: 1992
Předmět:
Zdroj: British Journal of Haematology. 82:547-554
ISSN: 1365-2141
0007-1048
DOI: 10.1111/j.1365-2141.1992.tb06465.x
Popis: 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.
Databáze: OpenAIRE
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