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Eur J Intern Med. 2007 May;18(3):238-40. doi: 10.1016/j.ejim.2006.09.032.

Acute vasculitis as a first manifestation of hairy cell leukemia.

European journal of internal medicine

Anna Remková, Andrej Halcín, Emöke Stenová, Pavel Babál, Viera Kasperová, Andrej Vranovský

Affiliations

  1. 1st Department of Internal Medicine, Comenius University, Bratislava, Slovak Republic.

PMID: 17449398 DOI: 10.1016/j.ejim.2006.09.032

Abstract

Hairy cell leukemia (HCL) is a lymphoproliferative malignancy characterized by bone marrow, spleen, liver, and occasionally lymph node infiltration with hairy cells, usually accompanied by splenomegaly and pancytopenia. We report an unusual case of HCL in a 53-year-old woman with leukopenia and sudden onset of fever of unknown origin, arthritis, and generalized maculopapular exanthem. Skin biopsy revealed perivascular and/or vessel wall lymphocytic infiltration in the dermis. On the basis of bone marrow biopsy and flow cytometry, the diagnosis of HCL was established. A detailed, retrospective re-evaluation of the skin biopsy helped to identify hairy cells among the cells of the perivascular infiltrations. Small-vessel vasculitis, as an atypical presentation, was found to predate a diagnosis of HCL. Recognition that vasculitis can reflect or antedate lymphoid malignancy may permit early diagnosis and aggressive treatment. A rapid response was obtained with a single course of cladribine.

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