scholarly journals Extramedullary Hematopoiesis Presenting as a Right Adrenal Mass in a Patient With Beta Thalassemia

2014 ◽  
Vol 6 (5) ◽  
Author(s):  
Hossein Karami ◽  
Mehrnoush Kosaryan ◽  
Mehrdad Taghipour ◽  
Rayka Sharifian ◽  
Aili Aliasgharian
Keyword(s):  
Adrenal Mass ◽  
Extramedullary Hematopoiesis ◽  
Beta Thalassemia

scholarly journals SAT-208 An Adrenal Incidentaloma with Extramedullary Hematopoiesis

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Tyler Zachary ◽  
Lillian Lien
Keyword(s):  
Adrenal Mass ◽  
Hepatic Duct ◽  
Adrenal Adenoma ◽  
Adrenal Incidentaloma ◽  
Thalassemia Major ◽  
Extramedullary Hematopoiesis ◽  
Osseous Metaplasia ◽  
Common Hepatic Duct ◽  
Beta Thalassemia ◽  
Adrenal Myelolipoma

Abstract Introduction: Adrenal adenomas are incidentally noted during nonadrenal disease imaging at a rate up to 4%. Frequency of incidentalomas increases with age, most being adrenocortical adenomas. This case highlights an uncommon etiology of such an adrenal mass finding. Case Description: A 37-year-old with a history of hypertension, hypothyroidism, and tobacco use was admitted after post-operative complication. Prior to surgery, she had been experiencing right upper quadrant pain along with 70 lb weight loss and diarrhea. She otherwise had frequent palpitations. Patient had undergone cholecystectomy and common hepatic duct injury was noted. Imaging revealed a 5.3 x 3.5 cm right adrenal mass previously unknown prior to surgery. Hormonal workup was negative for overproduction of aldosterone, cortisol, DHEA-S or metanephrines. Discussion: Patient underwent successful resection of adrenal mass, revealing adrenal adenoma with osseous metaplasia and hematopoiesis. Extramedullary hematopoiesis is a usually discovered incidentally as in this case. Typical sites are the spleen and liver. Cases of adrenal gland manifestations have been reported in the presence of hemoglobinopathies (thalassemia, hereditary spherocytosis) or myelofibrosis. At four-month follow-up, laboratory testing on this patient didn’t suggest any erythrocytic or leukocytic disorder. Conclusion: This case highlights an uncommon finding of hematopoiesis in an adrenal incidentaloma without any underlying hematologic defect or disease. References: Motta, I., Boiocchi, L., Delbini, P., Migone De Amicis, M., Cassinerio, E., Dondossola, D., Rossi, G. and Cappellini, M. D. (2016), A giant adrenal myelolipoma in a beta-thalassemia major patient: Does ineffective erythropoiesis play a role?. Am. J. Hematol., 91: 1281-1282. doi:10.1002/ajh.24446. Stewart P, Newell-Price J. (2016). The Adrenal Cortex. In Melmed S, Polonsky K, Larsen P, Kronenberg H, Williams Textbook of Endocrinology. (13th ed., pp 489-555). Philadelphia, PA: Elsevier

Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia

2020 ◽  
Vol 78 (10) ◽  
pp. 663-664
Author(s):  
Renan Ramon Souza LOPES ◽  
Larissa Soares CARDOSO ◽  
Franz ONISHI
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Compression ◽  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Beta Thalassemia

scholarly journals A rare case of extramedullary hematopoiesis in adrenal mass

2020 ◽  
Vol 30 ◽  
pp. 101120 ◽  
Author(s):  
N.H. Kolev ◽  
P.P. Genov ◽  
V.R. Dunev ◽  
B.A. Stoykov
Keyword(s):  
Adrenal Mass ◽  
Rare Case ◽  
Extramedullary Hematopoiesis

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

2017 ◽  
Vol 137 (3) ◽  
pp. 175-182 ◽  
Author(s):  
Nikolaos Sousos ◽  
Despoina Adamidou ◽  
Philippos Klonizakis ◽  
Alexandra Agapidou ◽  
Stamatia Theodoridou ◽  
...  
Keyword(s):  
Middle Eastern ◽  
Thalassemia Major ◽  
Extramedullary Hematopoiesis ◽  
Biological Factor ◽  
Beta Thalassemia ◽  
Eastern Region ◽  
Hematopoietic Tissue ◽  
Multicenter Studies ◽  
Clinical Issue

Extramedullary hematopoiesis (EMH) results from the extension of hematopoietic tissue beyond the confines of the bones. Since the initiation of regular transfusion programs from an early age for all thalassemia major (ΤΜ) patients, EMH has not been considered a clinical issue anymore. The present study aims to record the prevalence of EMH in chronically transfused ΤΜ patients followed at our institution and to investigate possible risk factors associated with its occurrence. The project was designed as a retrospective, nonexperimental, descriptive, exploratory study. In total, the study enrolled 104 patients. EMH was revealed in 15/104 (14%) patients. The presence of intravening sequence (IVS)-I-6 was significantly related with the development of EMH (p < 0.05). No other demographic or biological factor studied was found to be related with the presence of EMH. The study stresses a profound incidence of asymptomatic EMH in a solid group of well-transfused ΤΜ patients. Given the high incidence of the IVS-I-6 allele in the Mediterranean and Middle Eastern region, high-quality, prospective, multicenter studies could confirm the association of EMH occurrence with the presence of the IVS-I-6 mutation and further evaluate the exact role of this mutation in the EMH process.

Extramedullary Hematopoiesis in a Man With β-Thalassemia: An Uncommon Cause of an Adrenal Mass

2015 ◽  
Vol 3 (2) ◽  
Author(s):  
Shahryar Zeighami ◽  
Seyed Ali Eslahi ◽  
Mohammad Mehdi Hosseini ◽  
Ali Ariafar ◽  
Sara Pakbaz ◽  
...  
Keyword(s):  
Adrenal Mass ◽  
Extramedullary Hematopoiesis
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