DERYA ÖZYÖRÜK, Hacı Ahmet DEMİR, Suna EMİR, Esra KARAKUŞ, Bahattin TUNÇ

Occurrence of Wilms' tumor in a child with hereditary spherocytosis

Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. It is an autosomal dominant genetic disorder characterized by cell membrane abnormalities, specifically in red blood cells. Although the association between benign, borderline and malignant tumors and HS is not clear, various tumors such as splenoma, adrenal myolipoma, pancreatic schwannoma, ganglioneuroma, extramedullary hematopoiesis, myeloproliferative disorders, multiple myeloma, B-cell lymphoma and acute lymphoblastic leukemia have been presented in case reports concerning HS patients. Here we describe a 6-year-old boy with HS who presented with a mass in the left kidney. Tru-cut biopsy revealed Wilms' tumor (WT). To the best of our knowledge, this is the first case of WT associated with HS to be reported in the literature.

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1. Hassoun H, Palek J. Hereditary spherocytosis: a review of the clinical and molecular aspects of the disease. Blood Rev 1996; 10: 129-147.

2. An X, Mohandas N. Disorders of red cell membrane. Br J Haematol 2008; 141: 367-375.

3. Eber SW, Armbrust R, Schröter W. Variable clinical severity of hereditary spherocytosis: relation to erythrocytic spectrin concentration, osmotic fragility, and autohemolysis. J Pediatr 1998; 117: 409-416.

4. Tse WT, Lux SE. Red blood cell membrane disorders. Br J Haematol 1999; 104: 2-13.

5. Gommersall LM, Arya M, Mushtaq I, Duffy P. Current challenges in Wilms' tumor management. Nat Clin Pract Onco 2005; 2: 298-304.

6. Smith J, Rahilly M, Davidson K. Extramedullary haematopoiesis secondary to hereditary spherocytosis. Br J Haematol 2011; 154: 543

7. Arisawa K, Morita S, Kojima H, et al. [Hereditary spherocytosis associated with non-Hodgkin's lymphoma in the spleen]. [Article in Japanese] Rinsho Ketsueki 1994; 35: 871-875.

8. Ishida Y, Niino M, Matsuda H, Bandou S. [Hereditary spherocytosis presenting with acute lymphoblastic leukemia]. [Article in Japanese] Rinsho Ketsueki 1987; 28: 402-407.

9. Martinez-Climent JA, López-Andreu JA, FerrisTortajada J, Pérez-Sirvent ML, Castel-Sanchez V. Acute lymphoblastic leukaemia in a child with hereditary spherocytosis. Eur J Pediatr 1995; 154: 753-754.

10. Saito A, Shimada N, Niiya M, et al. [A case of pancreatic schwannoma with hereditary.spherocytosis]. [Article in Japanese] Nihon Shokakibyo Gakkai Zasshi 2005; 102: 605-611.

11. Demir HA, Ozdel S, Kaçar A, Senel E, Emir S, Tunç B. Ganglioneuroma in a child with hereditary spherocytosis. Turk J Pediatr 2012; 54: 187-190.

12. Takegoshi T, Nishino T, Tanino M, Nonokura A, Ohta G. An autopsy case of hemochromatosis and hepatoma combined with hereditary spherocytosis. Jpn J Med 1984; 23: 48-52.

13. Conti JA, Howard LM. Hereditary spherocytosis and hematologic malignancy. N J Med 1994; 91: 95-97.

14. Hattori Y, Harada K. [Hereditary spherocytosis associated with Bence Jones type multiple myeloma: a case report]. [Article in Japanese] Rinsho Ketsueki 1988; 29: 254-257.

15. Abramowsky C, Alvarado C, Wyly JB, Ricketts R. "Hamartoma" of the spleen (splenoma) in children. Pediatr Dev Pathol 2004; 7: 231-236.

16. Sekido N, Kawai K, Takeshima H, Uchida K, Akaza H, Koiso K. Adrenal myelolipoma associated with hereditary spherocytosis. Int J Urol 1996; 3: 61-63.

17. Hirata K, Matsumoto K, Gondo K, et al. Association of hereditary spherocytosis with familial adenomatous polyposis in a pedigree: a new syndrome or coincidence? J Gastroenterol 2003; 38: 79-81.

18. Agnifili A, Carducci G, Gola P, et al. [Juvenile polyposis coli associated with hereditary spherocytosis]. [Article in Italian] Minerva Chir 1998; 53: 811-817.

19. Agnifili A, Verzaro R, Marino M, et al. Juvenile polyposis coli and associated anomalies. Review of the literature and report of a case associated with hereditary spherocytosis. Ital J Gastroenterol Hepatol 1997; 29: 186-190.

20. Dallorso S, Dini G, Faraci M, Spreafico F. SCT for Wilms' tumour. Bone Marrow Transplant 2008; 41: S128-S130.

21. White B, Leong KW, Crotty GM, McCann SR. Hereditary spherocytosis: implications in bone marrow transplantation. Bone Marrow Transplant 1998; 21: 215.