LETTER TO EDITOR
Year : 2012 | Volume
| Issue : 1 | Page : 87-88
A case of polycythemia vera for orthopedic surgery: Perianesthetic considerations
Shaila Kamat1, C Deepa2, Samita Priolkar1, Marilyn Nazareth1
1 Department of Anaesthesiology, Goa Medical College, Goa, India
2 Department of Critical Care Medicine, Narayana Hrudayalaya, Bangalore, Karnataka, India
'Shaila' Green Valley Colony, Chogum Road, Alto Porvorim, Goa 403 521
Source of Support: None, Conflict of Interest: None
|Date of Web Publication||21-Feb-2012|
|How to cite this article:|
Kamat S, Deepa C, Priolkar S, Nazareth M. A case of polycythemia vera for orthopedic surgery: Perianesthetic considerations. Saudi J Anaesth 2012;6:87-8
|How to cite this URL:|
Kamat S, Deepa C, Priolkar S, Nazareth M. A case of polycythemia vera for orthopedic surgery: Perianesthetic considerations. Saudi J Anaesth [serial online] 2012 [cited 2020 Jan 17];6:87-8. Available from: http://www.saudija.org/text.asp?2012/6/1/87/93077
Polycythemia vera (PV) is a stem cell disorder giving rise to the proliferation of clones of hematopoeitic precursors, arising from a mutation in the JAK-2 gene.  Hematocrit is usually found to be >54% in these patients. It can be primary (PV) or secondary (hypoxia, raised erythropoietin levels). The criteria for a diagnosis of PV include an elevated hematocrit, normal arterial oxygenation, normal erythropoietin levels, and splenomegaly not attributable to another cause. When there is increase in the hematocrit beyond 50%, there is increased viscosity of blood, which causes decreased oxygen delivery. These patients are at increased risk of arterial and venous thrombosis.They are also at risk for paradoxical hemorrhage, which results from platelet dysfunction and Von Willebrand's disease.
We report the case of a 45-year-old man with PV posted for intramedullary nailing of fracture of right femur. Our patient was admitted with fracture of right femur following a road traffic accident. There was no head injury, spine injury, chest trauma, or abdominal trauma. He had no previous significant medical or surgical history.
Routine hematologic investigations revealed hemoglobin level of 19 g/dL, hematocrit of 57%, total WBC count of 15,000/μL, and a platelet count of 4 lakhs/μL. Hemoglobin and hematocrit were repeated from another laboratory to exclude laboratory errors, which showed similar results. Serum erythropoietin level was found to be normal. Arterial blood gas analysis showed normal oxygenation. The diagnosis of PV was confirmed. The patient did not give any history of symptoms suggestive of the condition except for occasional episodes of mild headache. His coagulation profile, ECG, chest X-ray, liver function tests, and renal function tests were within normal limits.
The patient was posted electively for intramedullary nailing of femur. After the possible risk of bleeding diathesis was explained to the patient, he opted for regional anesthesia for himself. A written informed consent was obtained from the patient.
Preoperative phlebotomy was done (about 450 mL) to bring down the hematocrit to 40%. Adequate hydration and hemodilution was achieved with intravenous infusion of 1000 mL Ringer's lactate solution. Combined spinal-epidural anesthesia was planned. Monitors were attached, which included non-invasive BP cuff, SpO 2 , ECG, and EtCO 2 . The patient was positioned carefully in sitting position. Under strict asepsis, an 18G Tuohy's needle was advanced into L2-L3 interspinous space and confirmation of the needle-tip in the epidural space was done by "loss of resistance to saline" technique. Epidural catheter was threaded in and fixed at 8 cm mark. A test dose of 3 cc of 2% lignocaine with adrenaline (1:2,00,000) was administered via the epidural catheter to rule out its placement in the subarachnoid or intravascular space. Thereafter, lumbar puncture was done with a 25G spinal needle passed into the subarachnoid space through L3-L4 interspinous space. The solution containing 3 cc of 0.5% heavy bupivacaine and 0.5 cc (150 μg) of buprenorphine was injected via the spinal needle after ensuring free flow of clear cerebrospinal fluid.
The patient was carefully positioned for surgery and had an uneventful intraoperative course. Blood loss was replaced with Ringer's lactate solution. Postoperative analgesia was ensured with 12 hourly administration of 5 mL solution of 0.125% bupivacaine and 150 μg of buprenorphine via epidural catheter during the first 72 h. Thereafter, the catheter was removed. Postoperative coagulation profile was normal. The patient was discharged on the 7 th postoperative day.
PV is considered when hematocrit is >48% in women and >52% in men. A steep increase in blood viscosity, which is thrombogenic, occurs with hematocrits >55%. It is associated with increased atherosclerosis and cardiovascular disease. Hematocrit levels >60% can be life threatening; and are at risk of venous and arterial thrombosis. There may be associated leukocytosis and thrombocytosis.  The treatment modalities include phlebotomy and myelosuppressive therapy with hydroxyurea.
Surgeons and anesthetists are confronted with a PV patient whose ability to tolerate surgery is complicated by the presence of hematologic abnormalities. The increased viscosity of blood constitutes the most common cause of hypoxia in these patients. These patients requiring surgery are at increased risk for perioperative thrombosis and bleeding diathesis.  The increased risk of thrombosis is due to the patient's baseline hypercoagulability augmented by the hypercoagulability associated with surgery. Abnormally low amounts of the ultra-large Von Willebrand's factor multimers, which are essential for normal platelet adhesion is responsible for the bleeding diathesis.  Phlebotomy and avoidance of extreme dehydration lower the risk of both thrombosis and hemorrhage in such patients in the perioperative period.
In our patient, the main anesthetic considerations were possible increased risk of perioperative thrombosis and bleeding diathesis. Preoperative phlebotomy to bring down the hematocrit and adequate hydration were done. The decision to proceed with combined spinal-epidural anesthesia was based on the following considerations: (1) normal coagulation profile; (2) no previous history of bleeding diathesis or thrombotic complications; (3) central neuraxial blockade is associated with reduced incidence of thrombotic complications; and (4) no history of treatment with drugs affecting coagulation. Our patient had an uneventful perioperative period.
To conclude, the presence of a raised hematocrit with normal arterial blood oxygenation and normal erythropoietin levels point to the diagnosis of PV. Because avoidance of hypoxia is a fundamental component of ideal anesthesia, it is important to recognize a patient with PV. Phlebotomy, adequate hydration, and recognition of thrombotic and bleeding problems are the keystones in the successful anesthetic management of such patients. Performing this duty requires sound clinical judgement and thorough understanding of the pathophysiology of PV. Our case demonstrates that combined spinal-epidural technique can be safely used in a PV patient whose coagulation profile is normal.
| References|| |
|1.||Hematologic disorders In: Hines RL, Marschall KE., Editors. Stoelting's Anesthesia and Co-existing diseases. Pennsylvania: Churchill Livingstone 2008. p. 417. |
|2.||Schafer AI. Essential (primary) thrombocythemia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, Editors. William's hematology. 5 th ed. New York: McGraw Hill: 1995. p. 340-5. |
|3.||Wassermann LR, Gilbert HS. Surgical bleeding in polycythemia vera. Ann N Y Acad Sci 1964;115:122-38. |
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