Abstract
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.
References
- 1. Choung B.S, Yoo W.H, Successful Treatment with intravenous immunglobulin of severe trombocytopenia complicated in primary Sjogren’s syndrome, Rheumatology International 2012;32(5):1353- 1355. 5
- 2. Hastürk A.B, Sjögren Sendromu, Ataman Ş, Yalçın P. Romatoloji 1.Baskı, 855-870, MN Medikal&Nobel Tıp Kitap Sarayı, Ankara, 2012. 1
- 3. Jun JW, Bae JK, Lee HS, Kim JH, Ju HW, Choi SW. Treatment with High Dose Dexamethasone of Severe Thrombocytopenia in Primary Sjögren’s Syndrome. Journal of Rheumatic Diseases 2012;19:6. 9
- 4. Kamath V, Prabhakar B, Veena, Lachikarathman D, Sjogren’s Trombocytopenia, Journal Of The Assosiation of Physicians of India, 2011;59: 114-115. 4
- 5. Khattri S, Barland P, Primary Sjogren’s syndrome and autoimmune cytopenias: a relation often overlooked, Bulletin Of The Hospital For Joint Diseases, 2012;70(2):130-132. 2
- 6. Kikawada M, Watanabe D, Kimura A, Hanyu H, Serizawa H, Iwamoto T, Autoimmune hemolytic anemia in an elderly patient with primary Sjögren’s syndrome, Journal of Internal Medicine, 2005; 44(12):1312-1315. 3
- 7. Ramakrishna R, Chaudhuri K, Sturgess A, Manoharan A. Haematological manifestations of primary Sjögren’s syndrome: a clinicopathological study. Q J Med 1992;83: 547-54. 7
- 8. Ramos-Casals M, Cervera R, García-Carrasco M, Vidal J, Trejo O, Jiménez S, et al. Cytopenia and past human parvovirus B19 infection in patients with primary Sjögren’s syndrome. Semin Arthritis Rheum 2000;29: 373-8. 8
- 9. Ramos-Casals M, Font J, Garcia-Carrasco M, Brito MP, Rosas J, Calvo-Alen J, et al. Primary Sjögren syndrome: hematologic patterns of disease expression. Medicine (Baltimore) 2002;81: 281-92.
Abstract
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.
Keywords
References
- 1. Choung B.S, Yoo W.H, Successful Treatment with intravenous immunglobulin of severe trombocytopenia complicated in primary Sjogren’s syndrome, Rheumatology International 2012;32(5):1353- 1355. 5
- 2. Hastürk A.B, Sjögren Sendromu, Ataman Ş, Yalçın P. Romatoloji 1.Baskı, 855-870, MN Medikal&Nobel Tıp Kitap Sarayı, Ankara, 2012. 1
- 3. Jun JW, Bae JK, Lee HS, Kim JH, Ju HW, Choi SW. Treatment with High Dose Dexamethasone of Severe Thrombocytopenia in Primary Sjögren’s Syndrome. Journal of Rheumatic Diseases 2012;19:6. 9
- 4. Kamath V, Prabhakar B, Veena, Lachikarathman D, Sjogren’s Trombocytopenia, Journal Of The Assosiation of Physicians of India, 2011;59: 114-115. 4
- 5. Khattri S, Barland P, Primary Sjogren’s syndrome and autoimmune cytopenias: a relation often overlooked, Bulletin Of The Hospital For Joint Diseases, 2012;70(2):130-132. 2
- 6. Kikawada M, Watanabe D, Kimura A, Hanyu H, Serizawa H, Iwamoto T, Autoimmune hemolytic anemia in an elderly patient with primary Sjögren’s syndrome, Journal of Internal Medicine, 2005; 44(12):1312-1315. 3
- 7. Ramakrishna R, Chaudhuri K, Sturgess A, Manoharan A. Haematological manifestations of primary Sjögren’s syndrome: a clinicopathological study. Q J Med 1992;83: 547-54. 7
- 8. Ramos-Casals M, Cervera R, García-Carrasco M, Vidal J, Trejo O, Jiménez S, et al. Cytopenia and past human parvovirus B19 infection in patients with primary Sjögren’s syndrome. Semin Arthritis Rheum 2000;29: 373-8. 8
- 9. Ramos-Casals M, Font J, Garcia-Carrasco M, Brito MP, Rosas J, Calvo-Alen J, et al. Primary Sjögren syndrome: hematologic patterns of disease expression. Medicine (Baltimore) 2002;81: 281-92.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Clinical Sciences |
| Journal Section | Articles |
| Authors | |
| Publication Date | September 1, 2016 |
| Published in Issue | Year 2016 Volume: 1 Issue: 1 |
