Priapism following splenectomy in an unstable hemoglobin: Hemoglobin Olmsted β141 (H19) Leu→Arg

Abstract

We report a case of severe priapism occurring in a patient with an unstable hemoglobin, Hb Olmsted ( p l 4 l Leu-Arg). This is a rare hemoglobin variant, which until now has been reported only once. The clinical course of the 12-year-old boy was characterized by severe hemolytic anemia leading to splenectomy and cholecystectomy at the age of 3.5 years. The priapism occurred 8 years after splenectomy, during a hemolytic febrile episode and required aspiration of the corpora cavernosa. This report raises the question of the benefit of splenectomy in patlents suffering from a chronic hemolytic anemia such as that due to an unstable hemoglobin. This treatment lowers the frequency and the severity of acute hemolytic attacks, but several cases of vascular complications have been reported alter splenectomy. o 19% wiey-~iss. Inc.

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

“…PH has been reported in patients with several unstable haemoglobinopathies (Honig et al, 1989;Krishnan et al, 1994;Lode et al, 2007). The same is true for priapism (Thuret et al, 1996;Gyan et al, 2001;Andrieu et al, 2003). Thromboembolic disease has been seen in patients with unstable haemoglobin (Pavlovic et al, 2004;Kim et al, 2005).…”

Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease

“…1 These clinical features have also been reported in thalassemia intermedia, hereditary spherocytosis, and other forms of severe hemolytic anemia. [47][48][49][50][51][52][53][54][55][56][57][58][59][60][61][62] This suggests that sickling is not required for this subphenotype and instead implicates the severe hemolysis of sickle cell disease in its pathobiology. Consistent with this model, the Jamaican sickle cell population, characterized by severe hemolysis, has a much higher prevalence of leg ulcers and priapism than those in Greece and India, who have less severe hemolysis, but comparable rates of VOC pain crisis.…”

Deconstructing sickle cell disease: Reappraisal of the role of hemolysis in the development of clinical subphenotypes

“…7,47 Similar to pulmonary hypertension, priapism may also complicate unstable hemoglobinopathy, β thalassemia intermedia, paroxysmal nocturnal hemoglobinuria and other types of hemolytic anemia where NO scavenging by plasma hemoglobin is likely. [48][49][50][51][52][53][54][55] Since NO is generally believed to play a role in normal penile erection, it is paradoxical that chronically impaired NO bioavailability is associated with priapism. For example, it is well known that increasing NO-dependent cGMP levels by inhibition of phosphodiesterase 5 (PDE5) with drugs like sildenafil increases erectile responses.…”