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￭ S-693 ￭
Development of Ankylosing Spondylitis in a patient with Essential Thrombocythemia
1Department of Internal Medicine, Yonsei University College of Medicine, Seoul, 2Departments of Internal Medicine, National Health Insurance Service Ilsan Hospital, Goyang, Korea
*Eun Seong Park1, Yun-Jeong Oh1, Jin Su Park2, Myung Hee Chang2, Chan Hee Lee2
Introduction: Essential thrombocythemia (ET) is one of the chronic myeloproliferative disorders (CMPD) and diagnosed by excluding reactive throm- bocytosis and other CMPDs. Ankylosing spondylitis (AS) is a chronic inflammatory disease of axial skeleton and other joints. Reactive thrombocytosis could be caused by chronic inflammatory conditions such as AS. However we experienced a case of AS in a patient with ET, and this is the third re- ported case in the world. Case Presentation: In 2006, 37-year-old man complaining of erythromelalgia, headache, and dizziness was referred to hem- atology center for evaluation of thrombocytosis. Blood tests revealed 1,441,000 /uL of platelet count and normal white blood cell count and hemoglobin. BCR/ABL gene rearrangement was negative. Bone marrow study showed normal cellularity with slightly increased number of megakaryocytes. The patient was diagnosed as ET and well controlled with anagrelide, hydroxyurea and aspirin (platelet 305,000 /uL). In 2015, this pa- tient visited to the hospital due to chronic inflammatory back pain for 10 years. Plain radiography showed total ankylosis of both sacroiliac joints and bamboo spine. Blood test revealed elevated ESR (64 mm/hr) and CRP (2.65 mg/dL). Immunological analysis showed HLA-B27 positive and others negative. He was diagnosed as AS and showed high functional inability (BASDAI 6.0). After treatment with naproxen and sulfasalazine, BASDAI was decreased (2.85). Discussions: Considering symptom duration of inflammatory back pain, it is assumed that this patient would have AS related symp- tom at the time of ET diagnosis. If we concern rheumatic disease including AS as a differential diagnosis of reactive thrombocytosis during diagnostic evaluation of ET, it would be possible to diagnose more early and could be delay progression of AS. From this case, it is important to consider that rheumatic disease such as AS for the exclusion of reactive thrombocytosis during diagnostic process of ET. Because the cases of AS with ET are very rare, further studies are needed for the evaluation of two diseases association.
￭ S-694 ￭
Non-cirrhotic portal hypertension in an ankylosing spondylitis patient
Department of Internal Medicine, Maryknoll Medical
*Sukki Park, Ji Hyun Lee, Joon Sul Choi, Hyun woo Kim, Beom jin Shim,Won Kyu Choi, Sang Hyun Kim
Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease of uncertain etiology that consists of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis (LC). Liver functions and structures remain primarily normal. The factors associated with INCPH in- clude umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and au- toimmune diseases. Approximately 70% of patients present with a history of major variceal bleeding, and treatment relies on the prevention of compli- cations related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus, and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Herein, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varix with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review
