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  Table of Contents 
Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 32-34

Polycythemia vera: Perioperative anesthetic challenges and review of literature

Department of Anesthesiology and Critical Care, VMMC and Safdarjung Hospital, New Delhi, India

Date of Submission16-Dec-2018
Date of Acceptance10-Jan-2019
Date of Web Publication6-May-2019

Correspondence Address:
Dr. Swati Singhal
F35 A1, 1st Floor, Gautam Nagar, New Delhi - 110 049
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_68_18

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Among the hematopoietic stem cells disorders, polycythemia vera (PV) constitutes a rare entity. The excess production of erythrocytes with thrombocytosis and leukocytosis in patients lead to both thrombotic and hemorrhagic complications. These complications may cause significant perioperative morbidity and mortality. Anesthetic challenges in a patient of PV and brief review of literature are described in this report.

Keywords: Complications, erythrocytes, polycythemia vera

How to cite this article:
Saini S, Singhal S, Wason R. Polycythemia vera: Perioperative anesthetic challenges and review of literature. Indian Anaesth Forum 2019;20:32-4

How to cite this URL:
Saini S, Singhal S, Wason R. Polycythemia vera: Perioperative anesthetic challenges and review of literature. Indian Anaesth Forum [serial online] 2019 [cited 2023 Jun 2];20:32-4. Available from: http://www.theiaforum.org/text.asp?2019/20/1/32/257689

  Introduction Top

Polycythemia vera (PV), a hematopoietic stem cells disorder, is characterized by the proliferation of clones of hematopoietic precursors leading to overproduction of red blood cells. Erythrocytosis and resultant hyperviscosity predispose these patients to increased risk of arterial or venous thrombosis leading to multiorgan involvement causing myocardial infarction, cerebrovascular accident, and pulmonary embolism. There is also risk of paradoxical hemorrhage during the perioperative period due to acquired Von Willebrand's disease and platelet dysfunction secondary to thrombocytosis. If uncontrolled, PV can lead to significant perioperative morbidity and mortality. Hence, careful evaluation and management of such patients is of paramount importance.

  Case Report Top

A 26-year-old male, recently diagnosed case of PV was admitted to our hospital with right anterior cruciate ligament (ACL) injury following a road traffic accident. He was posted for arthroscopic ACL reconstruction surgery. The preanesthetic checkup revealed a history of hypertension since 5 years; off and on unilateral headache since 6 months with one episode of severe occipital headache 5 years back which revealed cortical venous sinus thrombosis as the cause when investigated radiologically. The patient also had palmar erythema and splenomegaly since 4 months.

At the time of diagnosis of PV, his blood profile showed hemoglobin (Hb) 18.7 gm%, hematocrit (Hct): 61.4%, total leukocyte count (TLC): 7150/mm3, and platelet count: 356 × 103/μL. Rest of the blood investigations including coagulation profile and serum erythropoietin were normal. Two-dimensional echocardiography, lower limb Doppler, and magnetic resonance imaging brain were unremarkable. All probable causes for splenomegaly were also ruled out.

The patient was put on oral anticoagulation with vitamin K antagonist nicoumalone 1 mg PO daily for 1 year after the episode of cortical sinus thrombosis. He also underwent phlebotomies but irregularly since then. At the time of presentation, he was on low-dose aspirin 75 mg PO daily and antihypertensive medications (moxonidine 0.3 mg/day, telmisartan 20 mg/day, and chlorthalidone 12.5 mg/day).

The patient was prepared for elective surgery and was made to undergo six phlebotomies of 350 mL each over a period of 1 month. Postphlebotomy investigations on the day before surgery showed Hb: 12.5 gm%, Hct: 39.4%, TLC: 7000/mm3, platelet count: 408 × 103/μL, INR 1.2. Arterial blood gas analysis showed normal oxygenation with PaO2 of 92 mm of Hg on room air.

After explaining the possible complications to the patient, informed consent was taken. The patient opted for regional anesthesia. The patient was kept fasting overnight and was premedicated with Tab. pantoprazole 40 mg PO and Tab. alprazolam 0.5 mg on the previous night and morning of surgery. All the antihypertensive medications and Tab. aspirin 75 mg were continued till the morning of surgery. With the aim to maintain adequate hydration and hemodilution, the patient was advised to take plenty of fluids orally before keeping nil per oral and Ringer's lactate was infused intravenously @ 100 ml/h during the fasting period. The subarachnoid block was planned. In OR, standard monitoring was established, and baseline parameters: heart rate, noninvasive blood pressure, electrocardiogram, and SpO2 were recorded. Taking all aseptic precautions, subarachnoid block was given using 26G Quincke's spinal needle in L3–L4 interspinous space. 2.6 mL of 0.5% bupivacaine (Heavy) with 15 μg fentanyl was injected after ensuring free flow of clear cerebrospinal fluid. The adequate level for surgical anesthesia was achieved. Intra-operatively, 1500 mL of RL was infused. The surgery lasted for 80 min. Intraoperative vitals remained stable. Postoperative period was uneventful, and the patient was discharged on the third postoperative day.

  Discussion Top

Primary polycythemia or PV is a myeloproliferative disorder of hematopoietic stem cells secondary to mutation in the JAK-2 gene.[1] Etiology of the disease remains unknown. PV is a rare disease with an incidence of 0.7–2.6/1 lakh persons per year.[2] Males are affected more commonly than females, and the incidence increases with age.

Non-vera types are further classified into secondary and relative polycythemia. Secondary polycythemia may be due to prolonged oxygen deprivation (at high altitudes, chronic smoking, chronic obstructive pulmonary disease, and cyanotic heart disease) while diuretics and dehydration leads to relative polycythemia. PV is associated with overproduction of erythrocytes; thrombocytosis and leukocytosis seen in some patients. Mostly symptoms are due to hyperviscosity of blood caused by erythrocytosis leading to impaired microcirculation. These include headache, dizziness, vertigo, visual disturbance, angina, and intermittent claudication.

The diagnosis of PV is considered when Hct is >52% in males and >48% in females with normal arterial oxygenation and serum erythropoietin levels; splenomegaly not attributable to any other cause.[3] Our patient had a similar presentation with no associated secondary cause. Our patient also presented with a history of hypertension which can be explained on the basis of high blood viscosity leading to increased resistance to blood flow.[4]

The perioperative complications of major concern are both thrombosis and hemorrhage with an incidence of 7.7% and 7.3%, respectively, as reported by Ruggeri et al. in their survey.[5] Thrombosis can be arterial or venous. Baseline erythrocytosis, thrombocytosis, and increased hypercoagulability secondary to surgery predispose these patients to increased risk of thrombosis and thereby hemorrhage. Hct >60% leads to decreased tissue perfusion secondary to sluggish circulation.[3] Oxygen delivery must be optimized by reducing Hct <45% by phlebotomies, myelosuppressive drugs in selected patients and avoidance of prolonged period of perioperative fasting without supplemental fluid therapy. This goal was well achieved in our patient by serial phlebotomies to decrease Hct to 39.4% preoperatively. In addition, intravenous fluid was continued throughout the fasting period.

Among myelosuppressive agents, drug of choice for PV is hydroxyurea due to better safety profile compared to other drugs. However, there are concerns of leukemic transformation and azoospermia following prolonged usage. Low-dose aspirin therapy has been considered the mainstay therapy for PV in young and low-risk patients. Our patient, a young male was on Aspirin 75 mg PO OD at the time of presentation which was continued till the morning of surgery.

Prior thrombosis is a well-established risk factor for re-thrombosis in patients with PV.[6] Since our patient had a history of cortical venous sinus thrombosis, risk of thrombosis overweighed the risk of hemorrhage. Regional anesthesia should be used cautiously in these patients because of risk of abnormally low levels of Von Willebrand's factor (vWf) (related to absorption of vWf on platelets) and impaired platelet function leading to bleeding diathesis. We planned subarachnoid block as the anesthetic technique in our patient after confirmation of normal coagulation studies and platelet function. In addition, neuraxial blocks are known to reduce the incidence of thrombotic complications. Our patient had an uneventful perioperative period.

  Conclusion Top

Raised hematocrit, normal arterial blood oxygenation and serum erythropoietin level with splenomegaly are the classical features of polycythemia vera. Associated risk of thrombosis and hemorrhage may complicate perioperative course of these patients. Regular phlebotomy and aggressive hydration must be ensured to optimize tissue microcirculation and avoid hypoxia during anesthetic management. Postoperative period may be complicated by cardio-pulmonary or cerebral thrombosis/hemorrhage especially in a patient with a prior history of such an event. Therefore, vigilance and thorough understanding of anticipated complications is imperative to reduce perioperative morbidity and mortality.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Hines RL, Marschall KE, editors. Hematologic disorders. Stoelting's Anesthesia and Co-existing Diseases. Pennsylvania: Churchill Livingstone; 2008. p. 417.  Back to cited text no. 1
Johansson P. Epidemiology of the myeloproliferative disorders polycythemia vera and essential thrombocythemia. Semin Thromb Hemost 2006;32:171-3.  Back to cited text no. 2
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.  Back to cited text no. 3
  [Full text]  
Cirillo M, Capasso G, DeSanto NG. Relationship between hematocrit and blood pressure: Implications for primary hypertension. Nephron 1993;65:505-10.  Back to cited text no. 4
Ruggeri M, Rodeghiero F, Tosetto A, Castaman G, Scognamiglio F, Finazzi G, et al. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: A retrospective survey. Blood 2008;111:666-71.  Back to cited text no. 5
Khan FA, Khan RA, Iqbal M, Hussain S. Polycythemia vera: Essential management protocols. Anaesth Pain Intensive Care 2012;16:91-7.  Back to cited text no. 6

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