Management of Kikuchi disease using glucocorticoids.
Dear Editor, Sir, Kikuchi-Fujimoto disease (KFD) is a rare benign lymphoproliferative disorder affecting predominantly females with a ratio of 4:1 . It is associated with persistent fever and occasionally leukopenia and lympadenopathy especially involving cervical region not responsive to antibiotic treatment. Its pathogenesis is still unclear but recently, apoptosis of the CD8 T lymphocytes was shown as the main pathologic feature of this disease and in a minority of patients, KFD could be diagnosed before or simultaneously with autoimmune diseases such as systemic lupus erythematosus (SLE) . There were speculations about a possible link between autoimmune diseases and KFD but autoimmune studies were negative except in patients with SLE or other connective tissue diseases [3, 4]. An 18-year-old female patient had attained to hospital complaining persistent fever. On physical examination, her fever was 38.5°C; firm, painful lymph nodes were palpable on anterior and posterior cervical region. Laboratory analyses were as follows: Hg 12.7 g/dl, Htc 36.5%, MCV 89.7 fl WBC 5800/μl, Plt 392,000/μl, PT 11.05 s, INR 0. 92, APTT 31.36 s, sedimentation 63 mm/h, TSH 2.98 ulu/ml, Ferritin 9.84 ng/ml, vitamin B12 100 pg/ml, serum biochemistry including LDH and protein electrophoresis were within normal limits. Peripheral blood film revealed no abnormalities. ANA, anti-La, anti-ds DNA, anti-Scl-70, anti-Sm, anti-Jo-1, complement 4, Paul Bunnel, HbsAg, anti-Hbs, anti-Hbc IgM, anti-HCV, anti-HIV, EBV VCA IgM, Brucella agglutination test, Toxoplasma IgM, Toxoplasma IgG, and beta HCG were negative. Anti-Ro was positive, but Schirmer’s test was negative for both eyes and the patient had neither kserostomia nor dry eye. Neck USG revealed multiple lymphadenopathies reaching 12 mm at diameter so a lymph node excision was performed and diagnosis of KFD was made (Fig. 1a,b,d–f). She was given only nonsteroid anti-inflammatory (NSAID) therapy on outpatient basis and followed daily with physical examination so as to watch any possible clinical deterioration in her clinic. On follow-up, her sedimentation and C-reactive protein levels were found within normal limits with no signs of disease activity and lympadenopathies. One month after the remission, the patient came to control suffering left lateral neck pain. Physical examination revealed a lymph node of 3×3 cm in diameter with normal body temperature. Relapse of KFD was diagnosed and methyl prednisolone at 0.5 mg kg day started; 3 days after, the nodes disappeared so steroid was tapered slowly and stopped. Ann Hematol (2008) 87:71–73 DOI 10.1007/s00277-007-0342-2