Splenectomy in a Patient with Polycythemia Vera: Case Report and Review of the Literature

1
Splenectomy in a Patient with Polycythemia Vera
Case Report and Review of the Literature MS
Logan MD CM Watson MD JM Nottingham
MD University of South Carolina School of
Medicine, Columbia, South Carolina

• 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

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. Physic
al 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.

• 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

Fig 1 Computer tomography of the abdomen
Fig 2 Mesenteric angiograph highlighting the
splenic vasculature prior to embolization.
From Brenner B, Nagler A, Tatarsky I, Hashmonai
M. Splenectomy in agnogenic myeloid metaplasia
and postpolycythemic myeloid metaplasia. Arch
Intern Med. 19881482501-5.

• 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.

Fig 3 Intraoperative Photograph
Fig 4 Duration of Disease Control versus
Incidence of Complications From Wasserman LR,
Gilbert HS. Surgery in polycythemia vera. NEJM.
1963269(23)1226-30.