Case Report

Hereditary Spherocytosis with Leg Ulcers Healing After Splenectomy

Authors: Samira Rabhi, MD, Hind Benjelloune, MD, Mariam Meziane, MD, Moncef Amrani, MD, Rhizlane Berrady, MD, Ouafae Mikou, MD, PhD, Fatima Zohra Mernissi, MD, PhD, Wafaa Bono, MD, PhD

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.


Key Points


* Hereditary spherocytosis (HS) is a disease with clinical features ranging anywhere from asymptomatic to fulminant hemolytic anemia.


* Hemolysis sometimes occurs in patients with HS because of the relationship between an intact spleen and an intrinsic membrane-protein defect, leading to abnormal red blood cell morphology.


* In the event of altered red cell deformability associated with increased blood viscosity, a patient may experience impaired skin nutrition, which can lead to ulceration.


* The main determinant of erythrocyte survival is splenic sequestration. Thus, splenectomy cures almost all patients with this disorder.

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