Priapism Associated with Homozygous Hb E State: A Causal Association or an Incidental Finding?

Abstract

Dear editor, Priapism, that is, persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation, is a relatively uncommon disorder and is a medical emergency. Typically, only the corpora cavernosa are affected [1]. The term priapism was derived from the Greek god Priapus, son of Aphrodite who was born with oversized genitals [2]. The haematological causes for priapism include Sickle cell anaemia, Leukaemia, Multiple myeloma, Paroxysmal nocturnal haemoglobinuria, Thalassaemia, Thrombocythemia and Henoch–Schonlein purpura [3]. As far as hemoglobinopathies are concerned, priapism is most often encountered in sickle cell disease in 38–42 % of cases followed by sickle/beta thalassemia [4, 5]. Priapism is also noted in patients with sickle cell trait even though the incidence is low as compared with sickle cell anaemia [6–9]. However, so far priapism in Hb E homozygosity is not reported in literature. In this correspondence we intend to bring to notice an unusual association of priapism with Hb E homozygosity. A 27 years old male, resident of North Eastern part of India was referred to our haematology OPD to rule out any underlying haematological disorder as the individual had an episode of priapism 3 months ago. The patient had an episode of unprovoked painful persistent erection for more than 8 h for which he sought medical advice. The surgeon at that medical center had managed with therapeutic needle aspiration from corpora cavernosa combined with flushing of cavernosa with normal saline to clear the sludged blood. The patient’s symptom subsided subsequently with the intervention and was referred to our center for further evaluation. On detailed clinical history, the individual was not on any medication for chronic illnesses; neither was he abusing any psychoactive drugs, alcohol, no history of prior trauma to the perineal region. His physical examination revealed mild pallor. There was no hepatosplenomegaly or lymphadenopathy. Systemic examination did not reveal any abnormality. On USG abdomen, spleen was not reported as enlarged (Span 13 cms). On investigations, haemoglobin, total leucocyte count and platelet count were 9.1 gm/dL, 7.1 9 10/lL and 118 9 10/lL respectively. Peripheral smear examination revealed microcytic hypochromic red cells and target cells with normal differential leucocyte count and reticulocyte count of one percent. There were no sickle cells or atypical cells in peripheral smear and sickling test was also negative. Subsequently Hb HPLC was performed which revealed with Hb A2 ? E of 92.6 % (Retention time3.68 min), Hb A of 6 % (Retention time 2.29 min) and Hb F (Retention time 1.06 min) of 1.6 % (Fig. 1; Table 1). This was followed by parental study which revealed Hb A2 ? E in mother and father of 29 and 27 % respectively suggestive of both parents being heterozygous for Hb E, S. Venkatesan A. Purohit (&) M. Aggarwal P. K. Singh T. Seth H. P. Pati Department of Hematology, All India Institute of Medical Sciences, New Delhi, India e-mail: purohitabhi80@gmail.com