Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. Extramedullary hematopoiesis (EMH) mimicking a mass may develop in the lymph nodes, kidneys, pleura, mediastinum, adrenal gland, and in particular the spleen and liver. Other than EMH, B-cell lymphoma, acute lymphoblastic leukemia, and pancreatic schwannoma cases were reported in patients with HS. We present a 13-year-old female patient with HS and ganglioneuroma in the adrenal gland. This association is probably coincidental; however, with increasing cancer cases in HS and the genetic studies being made, this association will be clarified.
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PMID:Ganglioneuroma in a child with hereditary spherocytosis. 2273 9

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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PMID:Occurrence of Wilms' tumor in a child with hereditary spherocytosis. 2669 Jun 9