[ ] 203. (splenomegaly) 44 B. angioma) 2010; 20: splenomegaly, littoral cell angioma, splenectomy [3] [1,2]

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1 [] ,4 1,4 2,4 3 (splenomegaly) 44 B (splenectomy)(littoral cell angioma) 2010; 20: splenomegaly, littoral cell angioma, splenectomy [1,2] [3]

2 Taiwan J Fam Med Vol.20 No.4 [2] 44 B (hemoglobin) 13.2 gm/dl (mean corpuscular volume) 90.9 fl (white blood cell) 4100/µL 72% 26%2% 0% 0% (platelet) 141x103/ µl (reticulocyte) 1.6% (prothrombin time)11.9 (active partial thromboplastin time) 36.7 (glucose AC) 100 mg/dl (direct bilirubin) 0.2 mg/dl (total bilirubin) 0.6 mg/dl (alkaline phosphatase) 42 U/L (glutamate oxaloacetate transaminase)26 U/L (glutamate pyruvate transaminase) 34 U/L(erythrocyte sedimentation rate)5 mm/hour C (C-reactive protein)<0.10 mg/dl (antinuclear factor)1 40 DNA (anti-double strand DNA) C3(complement C3) 39 mg/dl C4(complement C4) 13 mg/dl (total complement activity CH50) 13.2 U/mL 1

3 (lymphoma) (metastatic neoplasm) 3, [1] 3-5% (peritonitis) (splenic infarction) [1,2,4] 3 (vascular channel) (splenic sinus) (H&E, 100X) 4 (cuboid) (atypia)(h&e, 200X)

4 Taiwan J Fam Med Vol.20 No.4 [1,2] 1. [1-6] 2. (sarcoidosis) (Felty's syndrome) (Brucellosis) [1-6] 3. [1-6] 4. (myelodysplastic anemia) (polycythemia vera) [1-6] 5. [1-6] 6. ( G a u c h e r disease) [1-6] C

5 (complete blood count) Falk 1991 [7] [8-14] (hypersplenism) [9,10] B [15-17] (friction rub) [1,5] (overwhelming postsplenectomy infection) (Streptococcus pneumoniae) (pneumococcal vaccine) [1,4,5] [17]

6 Taiwan J Fam Med Vol.20 No.4 1. David HB: Lymphadenopathy and splenomegaly. In: Jay HS ed. Internal Medicine. 5th ed. St. Louis: Mosby, 1998: James OA: Approach to the patient with lymphadenopathy and splenomegaly. In: Lee G, Daniel AA, eds. Goldman: Cecil Medicine. 23rd ed. Philadelphia: Saunders, 2007: D a v i d S, J o n a s A S : D i f f e re n t i a l Diagnosis of Infectious Diseases. 2nd ed. Philadelphia: Williams & Wilkins, 1996: Patrick HH, Dan LL: Enlargement of lymph nodes and spleen. In: Anthony SF, Eugene B, Dennis LK, eds. Harrison's Principles of Internal Medicine. 17th ed. United States of America: The McGraw- Hill Companies, 2008: Fred FF: Ferri's Clinical Advisor 2011: Instant Diagnosis and Treatment. 1st ed. Philadelphia: Mosby, 2010: Susan BS: The Spleen and its Disorders. In: Ronald H, Helen H, Bruce F, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia: Churchill Livingstone, 2008: Falk S, Stutte HJ, Frizzera G: Littoral cell angioma: a novel splenic vascular lesions demonstrating histiocytic differentiation. Am J Surg Pathol 1991; 15: Levy AD, Abbott RM, Abbondanzo SL: Littoral cell angioma of the spleen: CT features with clinicopathologic comparison. Radiology 2004; 230: Dascalescu CM, Wendum D, Gorin NC: Littoral cell angioma as a cause of splenomegaly. New Engl J Med 2001; 345: Ziske C, Meybehn M, Sauerbruch T, Schmidt-Wolf IGH: Littoral cell angioma as a rare cause of splenomegaly. Ann Hematol 2001; 80: Grantham M, Einstein D, McCarron K, Lichtin A, Vogt D: Littoral cell carcinoma of the spleen. Abdom Imaging 1998; 23: Qu ZB, Liu LX, Wu LF, Zhao S, Jiang HC: Multiple littoral cell angioma of the spleen: a case report and review of literature. Onkologie 2007; 30: Liu YP, Cheng SM, Huang JK: Littoral cell angioma of the spleen: a case report. Chin J Radiol 2005; 30: Lin CH, Yu JC, Shih ML, Peng YJ, Hsieh CB: Littoral cell angioma of the spleen in a patient with hepatocellular carcinoma. J Formos Med Assoc 2005; 104: Hansen T: Littoral cell angioma of the spleen. Association with colon and hepatocellular carcinoma. Pathologe 2007; 31: Wang CN: Littoral cell angioma of the

7 spleen in a patient with chronic hepatitis B and hepatocellular carcinoma. Chin J Pathol 2007; 36: Takehiro O, Kazuhiro H: Overwhelming postsplenectomy infection syndrome in adults a clinically preventable disease. World J Gastroenterol 2008; 14: 176-9

8 210 [Case Report] Case Report: Littoral Cell Angioma A Rare Disease Causing Splenomegaly Wei-Kuang Chen 1, Yi-Shan Lin 1,4, Ching-Ping Hsu 1,4, Tsang-En Wang 2,4 and Tsen-Long Yang 3 The approach to a patient with splenomegaly has remained a challenge for primary care physicians because patients with mild splenomegaly do not usually present with significant symptoms and it is often discovered incidentally. Splenomegaly always requires further evaluation and management because it has been associated with a wide variety of causes, among which viral infections, hematologic, circulatory and inflammatory diseases are the most common. An accurate diagnosis is based on detailed history taking, physical examination and associated lab or image studies. Here we present the case of a 44-year-old female with a past medical history of chronic hepatitis B and a two year history of persistent splenomegaly. She came to our hospital with complaints of epigastralgia and low back pain. Physical examination did not reveal any positive finding and the lab results were unremarkable. Abdominal ultrasonography showed splenomegaly with several hypoechoic lesions; abdominal CT scan revealed multiple different-sized tumors. The patient was then referred to the Department of General Surgery for splenectomy. The pathology report showed a littoral cell angioma. In addition to introducing the littoral cell angioma, the purpose of this report is to remind primary care physicians to be more vigilant of the manifestations associated with splenomegaly and to have a better understanding of the appropriate diagnostic approach and subsequent management, so that delivery of a higher quality of care may be achieved. (Taiwan J Fam Med 2010; 20: ) Departments of Family Medicine 1 and Gastroentorology 2, Mackay Memorial Hospital; Department of General Surgery 3, Shin Kong Memorial Hospital; Mackay Medicine, Nursing and Management College 4, Taipei, Taiwan, R.O.C. Received: September 6, 2010; Accepted: November 7, 2010.

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