Hereditary Spherocytosis with Leg Ulcers Healing After Splenectomy

Abstract

Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity. A refractory chronic leg ulcer is an uncommon complication of HS, reported in fewer than two percent of patients. We present the case of a 28-year-old man who was suffering from a leg ulcer and was unresponsive to treatment of two years' duration with various conservative modalities. He had been suffering from repeated attacks of jaundice, pallor, and splenomegaly since he was 10 years old. Upon admission, physical and laboratory findings revealed moderate HS. However, complete clearance of the leg ulcer was not achieved until two months after a splenectomy.

Intravascular Hemolysis: A Disease Mechanism Not to Be Ignored

“…In keeping with these findings, the incidence of pulmonary hypertension and venous thrombosis is reportedly higher in thalassemia patients that have been splenectomized [4], while descriptions of major improvements in leg ulcers in postsplenectomized HS patients are indicative of reduced IVH. Whether a decease or an increase in the incidence of PH occurs in splenectomized HS is unclear, but a recent report has found no significant elevation in tricuspid regurgitant jet velocity in HS individuals despite a median 25-year interval since splenectomy [8,9]. Animal experiments indicate that splenectomy can cause leukocytosis and increased platelet counts [10], as observed in both the thalassemia patients and HS patients of these reports, following splenectomy [5,7].…”

Abnormalities of the Erythrocyte Membrane

“…uncommon: chronic leg ulceration, cardiomyopathy, neuromuscular abnormalities, “tumors” due to extramedullary erythropoiesis 32, 33 …”

Pyoderma Gangrenosum in a Patient With Hereditary Spherocytosis

“…In particular, the possibility of chronic leg ulceration that occurs as a rare complication in patients with HS needs to be ruled out. 11 Although the pathogenesis of the ulceration remains unclear, it is thought that impaired release of oxygen from spherocytes results in tissue anoxia in a poorly vascularized area of the skin that is vulnerable to trauma. 11 These patients are unresponsive to conventional treatment for chronic ulcers; however, the ulcers usually resolve after splenectomy.…”