Abstract

Chronic myeloid leukemia (CML) is the most common leukemia presenting with priapism. Due to social stigma or shyness, the real incidence of priapism in our population is difficult to estimate. Since the successful development of tyrosine kinase inhibitors, the life expectancy of CML-chronic phase (CML-CP) has been comparable to the healthy population, and the importance of priapism has further magnified. As of now, the long-term outcome and fertility issues are not very well known. We present the case of a 24-year-old previously healthy gentleman who presented with priapism and was diagnosed as CML-CP. He achieved major molecular response at 18 months. On follow-up at 2 years since the diagnosis, he offers no complaint except moderate erectile dysfunction (International Index of Erectile Function score 11), compromising his quality of life.

Introduction

For more than a century, priapism has been reported to be associated with leukemia.[1] Hematological disorders constitute the etiology of almost 20%-of cases of priapism. It is most commonly seen in sickle cell disease (40%–50%-of cases) followed by leukemia (5% of cases). The most common leukemia presenting with priapism is chronic myeloid leukemia (CML), and the incidence varies from 2%-to 25%.[2],[3],[4] Priapism has also been reported at presentation in acute myeloid leukemia and multiple myeloma.[4],[5] Due to social stigma or shyness, the real incidence of priapism in our population is difficult to estimate. Since the successful development of tyrosine kinase inhibitors (TKIs), the life expectancy of CML-chronic phase (CML-CP) has been comparable to the healthy population, and the importance of priapism has further magnified, as the long-term outcome and fertility issues are not very well known.

Case Report

A 24-year-old previously healthy gentleman presented with painful erection of the penis for 5-day duration. He was married for 1 year and was sexually active. There was no history of any injury, illicit drug use, or sexual intercourse before the occurrence of priapism. He had a history of multiple such episodes which remitted on their own after a couple of hours which he had attributed to his newly married life and sexual activity. He denied any history of constitutional symptoms. His examination revealed “Triple P” (pallor – pale conjunctiva, palpable spleen – 8 cm below the left costal margin, and priapism – rigid and edematous penile shaft with soft glans penis) [Figure 1]. His hearing and visual acuity were normal along with no evidence of papilledema. There was no evidence of perineal injury, and rest of the examination was unremarkable. His blood investigation showed hemoglobin 107 × 109/L, white blood cell (WBC) 207 × 109/L, and platelet count 545 × 109/L. Peripheral blood smear showed immature cells of granulocytic lineage with 3%-blasts. Biochemical profile was normal. Bone marrow examination was consistent with CML-CP, and real-time-polymerase chain reaction for breakpoint cluster region–Abelson murine leukemia (BCR-ABL) transcript was positive. Penile biopsy revealed infiltration by leukemia [Figure 2].

| Figure 1:Clinical image of the persistently erected, edematous penis

| Figure.2:(a) Penile biopsy (H and E, ×10) shows fibroconnective tissue with marked neutrophilic infiltration and focal necrosis. (b) Penile biopsy (H and E, ×40) shows numerous admixed myelocytes and metamyelocytes

He was started on hydroxyurea (50 mg/kg/day), intravenous hydration (3 L/m2), and daily leukapheresis (total five sessions). He also required opioid analgesics to relieve his pain but with little benefits. Penile aspiration with a large-bore needle was performed on three occasions to decrease penile swelling. There was a gradual decline in total leukocyte count over 5 days, but in view of persistent penile swelling and pain, he underwent surgery for distal penile shunt (corpus cavernosa–glans shunt). After 7 days of hospital stay and continued symptomatic care, his penile swelling and pain gradually reduced, and the opioid was discontinued. His WBC was 24 × 109/L on day 8 of hospital stay. Hydroxyurea and leukopheresis were discontinued, and imatinib 400 mg once a day was started after confirming the diagnosis of CML-CP. He had a complete hematological response after 3 weeks of therapy and a suboptimal molecular response at 3 months of imatinib (BCR-ABL transcript 15%). A kinase domain mutation assay was negative, and he was nonaffording for second-generation TKI, and therefore, continued on imatinib albeit at a higher dose of 800 mg daily. He achieved major molecular response at 18 months. Two years since the diagnosis, he offers no complaint except moderate erectile dysfunction (International Index of Erectile Function [IIFE] score 11), but he can ejaculate and has been blessed with a son 2 months back.

| Figure 1:Clinical image of the persistently erected, edematous penis

| Figure.2:(a) Penile biopsy (H and E, ×10) shows fibroconnective tissue with marked neutrophilic infiltration and focal necrosis. (b) Penile biopsy (H and E, ×40) shows numerous admixed myelocytes and metamyelocytes

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