A RARE CAUSE OF THROMBOCYTOPENIA; PRIMARY SJÖGREN’S SYNDROME

Öz

Sjögren’s Syndrome (SS) is a chronic, systemic and autoimmune disease which mainly affects lacrimal and salivary glands via lymphocyte cell infiltration, destruction and dysfunction of the exocrine glands (2). This inflammation is speculated to be caused by B lymphocytes (5). SS is characterised by dry eye (xerophthalmia, keratoconjunctivitis sicca), dry mouth (xerostomia) and hypertrophy of the parotid gland (2). During the course of SS anemia, leucopenia, thrombocytopenia, and lymphoproliferative disorders can occur as extraglandular hematological involvement (6). Here we present a case with isolated thrombocytopenia (33,000 / mm3) dedected on routine tests since the last 3 months and has been referred to us with the suspicion of an underlying rheumatologic diesease. The case was diagnosed as primary Sjögren’s Syndrome and hydroxychloroquine 200 mg 1x1 / day and prednisolone 4 mg 1x1 / day was started. Improvement of platelet count (195,000 / mm3) and decline in joint complaints occured in the first month. Sjögren’s syndrome should be kept in mind as a rare cause of thrombocytopenia and rheumatological evaluation must be included in differential diagnosis.

Anahtar Kelimeler

• Primary Sjögren’s Syndrome,Thrombocytopenia

NADİR BİR TROMBOSİTOPENİ SEBEBİ: PRİMERSJÖGREN SENDROMU

Öz

Sjögren Sendromu (SS) başta gözyaşı, tükürük bezi olmak üzere ekzokrin bezlerin lenfosit hücre infiltrasyonu, destrüksiyonu ve disfonksiyonu sonucu gelişen kronik, sistemik ve otoimmün bir hastalıktır (2). Bu inflamasyona B lenfositlerin neden olduğu düşünülmektedir (5). Kuru göz (kseroftalmi, keratokonjonktivitis sikka), kuru ağız (kserostomi), parotis bezi büyümesi ile karakterizedir (2). Anemi, lökopeni, trombositopeni ve lenfoproliferatif hastalıklar SS’ nda ekstraglanduler hematolojik tutulum olarak görülebilir (6). Burada 3 aydır rutin tetkiklerinde izole trombositopeni (33.000/mm3) tespit edilen ve romatolojik hastalık araştırılması için tarafımıza konsülte edilen, kliniğimizce SS tanısı konulan ve hidroksiklorokin 200 mg 1x1/ gün ve prednizolon 4 mg 1x1/gün tedavisi ile birinci ayda trombosit sayısında (195.000/ mm3) düzelme, eklem şikayetlerinde gerileme meydana gelen bir olgu sunulmuştur. Sjögren Sendromunun trombositopeninin nadir bir sebebi olabileceği akılda tutulmalı, ayırıcı tanısında romatolojik değerlendirme yapılmalıdır. 

Anahtar Kelimeler

• Primer Sjögren Sendromu,Trombositopeni

Kaynakça

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