A 32-year-old man, a welder by profession without any prior comorbidities, was brought to the medical emergency department room with the persistent painful erection of the penis for 5-day duration. There was a gradual increase in pain, which impaired his ambulation. He gave a history of weight loss for 2 months (around 4 kg). He denied a history of any trauma to the penis, arousal stimulation, illicit drug intake, or any other medications. He had one episode of a similar attack of 4-h duration 7 days back, which detumesced on its own. There were no other symptoms of leukemia. The patient was treated at another hospital for a day before admission to our hospital. He was treated with cytosine arabinoside (100 mg), analgesics, (unfractionated heparin) as a short bolus, hydroxyurea (1 g 6 hourly), febuxostat, sodium bicarbonate, nifedipine, and lorazepam. His white blood cell (WBC) counts at admission were 450 x 103/mul. With the initiation of the above treatment, it reduced to 367 x 103/mul. There was some reduction in pain intensity after 24 h of admission. However, the pain recurred, and he was referred to our hospital on next day. The patient was conscious and oriented but unable to give the history due to severe pain.
The patient's vital signs were as shown: heart rate-102 beats/min, blood pressure-130/80 mmHg, RR-20/min, and SpO2-98% on room air. The conjunctiva looked pale, and clubbing was present. There was no icterus or lymphadenopathy or cyanosis, or edema. Physical findings revealed massive splenomegaly. Local examination showed a tumescent warm penis with preserved sensations, and rigid cavernosa. No evidence of associated skin changes was seen. Laboratory evaluation showed hyperleukocytosis, raised lactate dehydrogenase but no evidence of tumor lysis. Peripheral smear was consistent with the chronic phase of CML. Peripheral blood real-time polymerase chain reaction was sent for COVID-19 and found negative. From the peripheral smear report, the patient was diagnosed as CML with secondary hyperleukocytosis. The patient was restarted on 1-g hydroxyurea BD, allopurinol 100 mg TDS, antibiotics, analgesics, and sedatives. He underwent phenylephrine injection with 50-ml blood aspiration from the urology department in our hospital and had a 50% reduction in the erection. However, the pain was not relieved (pain score - 9/10). Hence, he was planned for rapid cytoreduction with leukapheresis.
The patient had a total leukocyte count of 270.41 x 103/mul before the procedure. After obtaining consent from the patient, we did one leukapheresis session using the Spectra Optia machine, a continuous flow automated cell collection system. A femoral vein catheter was used for accessing the circulation, and ACD (acid-citrate-dextrose) was used as the anticoagulant with an inlet: AC ratio of 12.0 and an inlet flow rate of 40 ml/min. No replacement fluid was used during the procedure. Collect rate was kept as 3 ml/min. A total of 5.8 liters of blood (1.5 total blood volume [TBV]) was processed, and the collected volume was 478 ml [Figure 1]. A sample for complete blood count was taken at the end of collection for assessing the reduction in leukocyte count. Details of pre- and postprocedure hematological parameters are given in the table below [Table 1]. The general condition of the patient was stable throughout the procedure. The patient experienced immediate pain relief after the procedure, although edema at the site was not reduced significantly. Pain score showed a reduction from a score of 9-10 to a 1-2 level. The patient was started on imatinib 400 mg the next day the following leukapheresis as his [chimeric gene of BCR (Breakpoint cluster region) gene and ABL (Abelson proto-oncogene)] result came as positive. He had undergone a surgical shunt, but there was only a 30% reduction in edema. He was discharged on day 7 following leukapheresis with a leukocyte count of 23,000/mul with advice for regular follow-up.