Possible association between hairy cell leukemia and Behcet's disease. (Letters to the Editor).

To the Editor: Many vasculitides (1) and autoimmune conditions (2) have been associated with hairy cell leukemia, including polyarteritis nodosa, leukocytoclastic vasculitis, seleroderma, polymyositis and pyoderma gangrenosum. Likewise, Behcet's syndrome has been described in the setting of acute and chronic leukemia. (3-5) We encountered a patient with hairy cell leukemia who developed Behcet's syndrome, suggesting a possible association that has not been reported previously in the literature.

A 52-year-old white woman presented in June 1999 with pancytopenia (white blood cell count, 3.8/[mm.sup.3]; hemoglobin, 12.2 g/dl; platelets, 116,000/[mm.sup.3]). She had a benign medical history. She was asymptomatic, and her physical examination was unremarkable. The peripheral blood smear showed anisocytosis. A bone marrow biopsy revealed a patchy infiltrate of loosely spaced, medium-sized lymphocytes suggestive of hairy cell leukemia.

Flow cytometric examination of the bone marrow cells revealed a monoclonal B lymphocyte population expressing CD 19, CD20, HLA-DR, and CD lie surface antigens and a 330:1 [kappa]-to-[lambda] ratio. These cells were negative for CD5 and CD10. Peripheral blood lymphocytes displayed tartrate resistant acid phosphatase (TRAP). These findings were consistent with hairy cell leukemia. The patient was followed on a regular basis without any therapeutic intervention. In August 2000, she developed oral aphthous lesions. A biopsy of one of these lesions revealed mucosal spongiosis with acute inflammation along with submucosal chronic inflammation with scattered neutrophils and eosinophils. These lesions regressed spontaneously. In October 2001, she developed fatigue, fever, anorexia, and painful oral and vaginal aphthae and skin pustules, along with erythematous tender subcutaneous nodules of the lower extremities consistent with erythema nodosum. There was no improvement with Valacyclovir administered for a presumptiv e diagnosis of herpes simplex virus infection, and viral cultures remained negative. A skin biopsy of one of the pustular lesions showed neutrophils within the deep reticular dermis in an interstitial pattern consistent with unusual folliculitis reaction of Behcet's syndrome. The patient was treated with Prednisone, which resulted in marked improvement of all symptoms and signs. Throughout the course of this event, her hairy cell leukemia remained stable.

We suggest that the association of hairy cell leukemia with Behcet's syndrome may not be a random occurrence. A complex immunologic imbalance may be the common denominator leading to the manifestation of these two conditions in the same patient. Physicians should be alert to this possible association, especially for patients who present with Behcet's disease, because hairy cell leukemia may remain concealed and undiagnosed for long periods.

Them R. Oo, MD

Maria Delafuente, MD

Hani Hassoun, MD

Memorial Sloan-Kettering Cancer Center

Joan and Sanford I. Weill

Medical College

Cornell University

New York, NY

St. Elizabeth's Medical Center

Tufts University School of Medicine

Boston, MA

References

(1.) Hasler P, Kistler H, Gerber H. Vasculitides in hairy cell leukemia. Semin Arthritis Rheum 1995;25:134-142.

(2.) Westbrook CA, Golde DW. Clinical problems in hairy cell leukemia: Diagnosis and management. Semin Oncol 1984; 11(4 Suppl 2):514-522.

(3.) Rosenthal NS, Farhi DC. Myelodysplastic syndromes and acute myeloid leukemia in connective tissue disease after single-agent chemotherapy. Am J Clin Pathol 1996;106:676-679.

(4.) Kaloterakis A, Stavrianeas NG, Karagianni IN Filiotou A, Katoulis AC, Vareltzides A, et al. Adamantiades-Behcet's disease coexisting with acute myeloblastic leukaemia. Br J Dermatol l997;137:317-318 (letter).

(5.) Vaiopou]os G, Terpos E, Viniou N, Nodaros K, Rombos J, Loukopoulos D. Behcet's disease in a patient with chronic myelogenous leukemia under hydroxyurea treatment: A case report and review of the literature. Am J Hematol 2001;66:57-58.