A 24-year-old female presented with a history of mild bruises and petechiae. No history of trauma. She was born with mitral valve stenosis. When she was 21, she complained of heavy menstrual bleeding, intermenstrual bleeding, gingival bleeding, shortness of breath, and severe pain in the left hypochondrium. No family history of bleeding or drug use. Chest X-ray was normal. Abdominal ultrasound (US) revealed marked hepatosplenomegaly (HSM). Complete blood picture (CBC) showed: TLC = 3.6 thousands/cmm, RBCs = 3.23 millions/cmm, Hb = 9.9 g/dl and platelet count = 41 thousands/cmm. Blood smear demonstrated giant agranular platelets (Fig. 1). Bleeding time was prolonged. Reticulocytic count = 4.8%. Erythrocyte sedimentation rate (ESR) = 5 mm/h. Bone marrow aspiration revealed no abnormalities except for a decrease in the free platelets, which were mainly large and agranular. Bone marrow biopsy revealed hypercellular bone marrow, active haematopoiesis, and associated fibrosis. No infiltration. Platelet function tests showed decreased aggregation by collagen, thrombin, and adenosine diphosphate (ADP). The diagnosis was GPS, and then the patient was discharged after receiving a blood transfusion.
Nine months later, the patient returned with the same presentations. US was done to detect the cause of the abdominal pain and showed only HSM (splenic diameter = 22.0). Contrast-enhanced computed tomography (CECT) was done and showed the same findings as in the US. CBC showed: TLC = 2.1 thousands/cmm, RBCs = 2.9 million/cmm, haemoglobin (Hb) = 9.5 g/dl, and platelet count = 18 thousands/cmm. Again, she received a blood transfusion and strong analgesics and was then discharged. The multidisciplinary team decided to do splenectomy since the enlarged spleen was supposed to be the aetiology of the left hypochondrial pain and to increase the platelet count and decrease the bleeding tendency. Unfortunately, the patient was unfit for surgery due to mitral valve stenosis and high bleeding tendency. The other option was to do PSE.
Antibiotic prophylaxis (1 gm of third-generation cephalosporin twice a day and metronidazole 500 mg/100 ml intravenous infusions daily) was started three days before procedure and maintained for seven days after. Then, for a further week, oral dose of Ciprofloxacin 500 mg/Metronidazole 500 mg twice daily. The patient was given pneumococcal, haemophilus influenza, and meningococcal vaccines. The morning of the procedure, the patient received four units of platelets. The femoral puncture was performed under local anaesthesia using a 6-F introducer sheath. The celiac trunk and splenic artery were catheterized with a 5-F Cobra catheter (Imager-Boston Scientific, Natick, Massachusetts) over a 0.035-inch hydrophilic guidewire (Terumo, Tokyo, Japan), then a microcatheter (Renegade HI-FLO microcatheter, Boston Scientific) was placed distal to the pancreatic branches (Fig. 2). Embolization was done using Embospheres microspheres 700–900 µ (Biosphere Medical, Rockland, MA) mixed with gentamicin 80 mg to diminish the incidence of splenic abscess and undiluted contrast. The fluoroscopic time was 8 min.
The patient was hospitalized for three days for observation, prophylactic antibiotic therapy, and pain management. The short-term clinical outcome was good; the patient suffered from pain controlled by analgesics. After 24 months of follow-up, the patient had a normal menstrual history, no left hypochondrial pain, and no gingival bleeding. Laboratory outcomes revealed a rise in the platelet count to 70, 55, and 51 thousand/cmm after 1, 12, and 24 months, respectively. The TLC changed to 7.9, 8.2, and 9.0 thousand/cmm after 1, 12, and 24 months, respectively. Regarding the radiological outcome, the CECT was done 30 days after the embolization. The whole spleen and the viable residual spleen were volumetrically measured in the venous phase and revealed that 61.65% of the spleen was infarcted (Fig. 3). The US was done before the discharge of the patient and revealed no complications. After 12 months, a follow-up US revealed a significant reduction in the splenic diameter (11.6 cm), which has remained relatively stable for two years (11.2 cm).