Obstructive uropathy due to extramedullary haematopoiesis in beta thalassaemia/haemoglobin E

Abstract

An 18 year old woman with beta thalassaemia/haemoglobin E developed a large pelvic tumour resulting in bilateral obstructive uropathy. Technetium-99m sulphur colloid marrow image, computed tomographic scan of the abdomen and needle biopsy of the mass confirmed the diagnosis of extramedullary haematopoiesis. Although radiation is the treatment of choice for decompression, the mass in this patient did not respond satisfactorily due to its multiple area of tumour autoinfarction. Obstructive uropathy due to extramedullary erythropoiesis has not to our knowledge been previously described.

Epidemiologic study of major complications in adolescent and adult patients with thalassemia in Northeastern Thailand: the E-SAAN study phase I

“…Disease-related complications seem to be the challenging problem in aging patients with thalassemia who now survive longer than in the past [3,4]. There are many thalassemiarelated complications which are found to increase morbidity and mortality of the disease that includes heart failure [5][6][7], pulmonary hypertension [8][9][10][11], extramedullary hematopoiesis [12][13][14][15], osteoporosis [16,17], cholelithiasis, infections [18][19][20], thrombosis [21][22][23] and endocrinopathies [24,25]. Several studies in the past few years have shown the clinical risk factors for disease-related complications that include: advanced age, anemia, splenectomy, iron overload, severe thalassemia genotypes and iron chelation therapy [9,19,[26][27][28][29][30].…”

Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis

“…Presacral locations have been described in rare cases in adults, leading also to a diagnostic confusion [6,7]. Other sites have been reported, resulting in upper airway distress [8], obstructive uropathy [9], or intestinal intussusception [10].…”

Exceptional localization of extramedullary hematopoiesis: presacral and periureteral masses

“…The involvement of the urinary tract is also rare; and although kidney involvement has been reported, it has a perirenal predilection. Isolated periureteral extension is exceptional with only two reported cases (3,7).…”