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Esansiyel Trombositemi’li bir hastada Sistemik Lupus Eritematozus

Systemic Lupus Erythematosus in a Patient with Essential Thrombocytemia

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It is possible to see various hematological ab-normalities in systemic lupus erythematosus (SLE). These abnormalities include anemia, thrombocytopenia, and leucopenia (1). Throm-bocytosis is a condition in which there is a number of platelets over 450000 in the blood (2). This can stem from the reactively stimula-tion of platelet production in various diseases (iron deficiency anemia, malignances, infecti-ons, postsplenectomy or asplenic conditions), familial mutations, essential thrombocythemia, and other myeloproliferative disorders (3). Thrombocytosis due to autosplenectomy is an unusual hematologic finding in SLE. Essential thrombocythemia (ET), one of the reasons for thrombocytosis, is one of the chronic myelop-roliferative disorders which JAK 2 gene muta-tion can be detected, and it is characterized by thrombocytosis and abnormal proliferation of megakaryocytes. Here, we have presented a patient who was observed with the coexisten-ce of ET and SLE. 69 years old male patient complaining of recur-rent epistaxis was admitted in May 2007. Phy-sical examination of the patient was not re-markable. No pathology explaining the epis-taxis was detected in the ear, nose, and throat examination. Urinalysis, liver and kidney func-tion tests were normal. The patient's PT and aPTT values were normal as well. The number of platelet was found to be 1.200x10³/uL. Pe-ripheral smear of the patient was not remar-kable except for thrombocytosis. Possible cau-ses of reactive thrombocytosis were excluded. While hyperplasia was detected in the mega-karyocytic series in bone marrow biopsy, reti-culin fibrosis, erythroid hyperplasia and neut-rophil granulopoiesis were not observed (Figu-re 1). Jak2 gene mutation was positive, ET was ac-cepted and hydroxyurea treatment was star-ted. The patient readmitted in June 2009 with pain in hand joints, swelling, temperature rise, photosensitivity, and malar rash. The eryth-rocyte sedimentation rate was 100 mm/h, leu-kocyte count was 5600/mm3, lymphocytes 800/mm3, and platelets 450x10³/uL. ANA was found to be 1/100 nucleolar, anti-dsDNA was 78.5 IU/ml, and accordingly, the patient was diagnosed as SLE (4,5). It was thought that the autosplenectomy as the factor for SLE associa-ted with secondary antiphospholipid syndrome in thrombocytosis etiology. Lupus anticoagu-lant, antikardiyolin IgM and IgG were negative. While the performed peripheral smear to de-termine autosplenectomy was normal, only a little splenic infarct was observed in the captu-red Tc-99m heat denaturated red blood cell (RBC) scintigraphy (Figure-2).
doi: 10.5505/abantmedj.2013.13008



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