Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sickle cell disease (SCD) is a congenital hemolytic anemia with various clinical findings. In some cases hematological neoplasias and some solid tumors may accompany this disease but these have rarely been reported. Here we report five cases with SCD and accompanying hematological neoplasias including lymphoblastic lymphoma, multiple myeloma and hairy cell leukemia in four cases with sickle cell trait and one case of Hodgkin' disease in sickle cell anemia. All of the cases except one had no previously diagnosed congenital hemolytic anemia and/or family history. Peripheral blood findings suggestive for an underlying hemolytic anemia were the first step and the most important initial lead in the detection of SCD. Severe musculoskeletal signs during the first presentation was seen in the lymphoma case, residual renal dysfunction after remission of multiple myeloma, and areas of infarction in the spleen in CT scans in the patient with sickle cell anemia were the most interesting findings in these cases. A standard therapeutic approach without any additional toxicity was relevant in all cases. Detailed clinical presentation and outcome of these five cases are documented here and the literature has been reviewed.
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PMID:Sickle cell anemia and hematological neoplasias. 1238 25

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