Splenectomy in a Patient with Polycythemia Vera: Case Report and

Review of the Literature

MS Logan MD; CM Watson MD; JM Nottingham MDUniversity of South Carolina School of Medicine, Columbia, South

Carolina

Fig 1: Computer tomography of the abdomen Fig 2: Mesenteric angiograph highlighting the splenic vasculature prior to embolization.

Fig 3: Intraoperative Photograph

Case

A 39-year-old Caucasian female presented with progressive left upper quadrant pain.

On presentation, she gave a history of being diagnosed with a “blood disorder” one year previously but had failed to follow up for recommended medical care. Other pertinent history included being an avid equestrian.

Physical exam showed a tender left upper quadrant with marked splenomegaly extending across the midline and into the pelvis. Her vital signs including oxygen saturation were within normal limits.

Initial laboratory studies obtained showed a hemoglobin of 17.9 mg/dL and a hematocrit of 52.7%. CT of the abdomen and pelvis showed massive splenomegaly (30cm x 16cm) with multiple infarcts (Figure 1).

She was diagnosed with polycythemia vera.

Preoperative mesenteric angiography was performed which revealed multiple, dilated collateral vessels feeding the spleen, including inferior phrenic vessels and large pelvic collaterals (Figure 2). The celiac trunk was completely occluded therefore embolization was accomplished by a retrograde approach via the SMA, inferior and superior pancreaticoduodenal arteries.

Following splenic embolization she underwent an uncomplicated open splenectomy through a left subcostal incision (Figure 3).

Postoperatively, she had the typical thrombohemorrhagic complications expected with this disease. These were dealt with appropriately and she was discharged home.

Perioperative Considerations:

•Thorough history of any bleeding or thrombotic events

•Full coagulation profile, complete blood count and bleeding time

•Optimization of hematocrit and platelet count- see Figure 4

•Consider preoperative mesenteric angiography splenic with or without embolization for defining vascular anatomy and decreasing the organ size and vascularity.

Indications for Splenectomy in Polycythemia Vera

•Painful Splenomegaly 68%•Responders 96%

•Refractory Anemia 53%•Responders 61%

•Refractory Thrombocytopenia 15%•Responders 60%

•Asymptomatic Massive Splenomegaly ?• Non-compliant patients • Those without access to standard medical care• Lifestyles prone to increase risk of splenic rupture

Special considerations in Polycythemia Vera:

•Thrombosis:

•Leading cause of morbidity and mortality

•Blood hyperviscosity

•Aberrant activation and aggregation of platelets

•Hemorrhage:

•Leading cause of perioperative complications

•Acquired von Willebrand Disease

•Quantitative and qualitative platelet dysfunction

Fig 4: Duration of Disease Control versus Incidence of ComplicationsFrom: Wasserman LR, Gilbert HS. Surgery in polycythemia vera. NEJM. 1963;269(23):1226-30.

0

5

10

15

20

25

30

Uncontrolled Immediate (0-7 Days) Short (1wk- 4mos.) Long (4 mos andover)

Duration of Control

Nu

mb

er

of

Pa

tie

nts

Uncomplicated

Complicated

Deaths

From: Brenner B, Nagler A, Tatarsky I, Hashmonai M. Splenectomy in agnogenic myeloid metaplasia and postpolycythemic myeloid metaplasia. Arch Intern Med. 1988;148:2501-5.