History

Fact Explanation
Introduction This is a malignant condition in bone marrow stem cells associated with increased blood cell volume. The condition occurs as a result of a mutation( JAK2 mutation) in a single haemopoietic stem cells[1].
Asymptomatic Patients may be asymptomatic and may present with vague symptoms of hyperviscosity[4].
Lethargy, confusion, headache, dizziness These symptoms are due to central nervous system disturbances with hyperviscosity causing poor perfusion[2][3].
Spontaneous bleeding This bleeding can be gestrointestinal causing fresh per rectal bleeding / malena, it can be genitourinary causing haematuria, nasal bleeding or gum bleeding[5] .
Visual disturbances Visual disturbances can occur due to retinopathy.This visual problems is call 'slow- flow retinopathy' , in here patients will describe the visual problem as 'lloking through a water car windscreen'[2][6].
Itchiness after warm bath Warm bath can stimulate the histamine release from basophils and mast cells. With the disease there is increased levels of basophils and mast cells. This leads to excessive release of histamine causing itchyness[7].
Burning sensation in fingers and toes With the hyperviscosity perfusion becomes poor causing ischemic pains[8].
Features suggestive of arterial thrombosis With the hyperviscosity there is a increased risk of thrombotic complications. Arterial thrombotic complications can damage various systems giving specific features of hypoperfusion. Eg; In cardiac complications, patient will present with chest pain, difficulty in bresthing, dizziness, sweating and other features of myocardial infarction/ angina[5]. If thrombotic complications occur in central nervous system, patient will have seizures, paralysis and paresthesia like features[9]. If thrombosis occur in a artery supplying to limbs, patients will present with acute limb ischemia( sever pain at rest, coldness, limited movements, numbness) following features of occlusive arterial disease like pain starting few minutes after initiating walking, pain relief with resting and reproduce with walking in a same distance[3].
Features suggestive of venous thrombosis In deep vein thrombosis patient will present with acute onset severe pain, swelling, redness and warmth of the limb. Hepatic venous thrombosis will present with abdominal distension(ascites), features of liver insufficiency like nausea, vomiting, loss of appetite, yellowish discoloration of eyes, generalized swelling and gastrointestinal bleeding( either as haemoptysis/ malena)[10]. In cerebral venous thrombosis patient will be confused, drowsy, may develop seizures due to cerebral venous disease[11].
Severe continuous small joint pain Patients will present with symptoms of gout as a result of increased uric acid production. Gout mainly affects big toe[2].
Epigastric pain/ burning sensation, incresed with meals, nausea, vomiting and regurgitation with or with out blood stained vomitus/ tary colour stools. These patients are at risk of developing peptic ulcers. Increased in histamine levels and increased gastric acidity may leads to this presentation in these patients[2].
Weight loss This may be due to the hypermetabolism associated with the disease[12].
Early satiety Patients can complain early satiety because splenomegaly causes limitation in gastric stretching by taking extra space from the abdomen[13].
References
  1. SCHAFER PW. The Etiology and Treatment of Polycythemia Rubra Vera : Observations Based upon Studies of Body Fluid Changes in Dogs Subjected to Proprioceptor Depressor Neurotomy and Extensive Sympathectomy, Including the Case Report of a Man with Polycythemia Rubra Vera Treated by Extensive Para-Vertebral Sympathectomy Ann Surg [online] 1945 Dec, 122(6):1098-1124 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1618335
  2. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124
  3. ZORASTER RM, RISON RA. Acute embolic cerebral ischemia as an initial presentation of polycythemia vera: a case report J Med Case Rep [online] :131 [viewed 20 September 2014] Available from: doi:10.1186/1752-1947-7-131
  4. MCLORNAN D, PERCY M, MCMULLIN MF. JAK2 V617F: A Single Mutation in the Myeloproliferative Group of Disorders Ulster Med J [online] 2006 May, 75(2):112-119 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1891745
  5. AL-FADHLI J, AL-SHAMMARI F, AL-DUAIJ A, AL-SARRAF N. Coronary artery bypass grafting in a patient with polycythaemia rubra vera - a rare indication with a spectrum of complication: a case report Cases J [online] :8126 [viewed 20 September 2014] Available from: doi:10.4076/1757-1626-2-8126
  6. TEH MM, ZAMAN MJ, BROOKS AP, LI VOON CHONG JS. When is a high potassium not a high potassium? J R Soc Med [online] 2003 Jul, 96(7):354-355 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC539545
  7. YONOVA D. Pruritus in certain internal diseases Hippokratia [online] 2007, 11(2):67-71 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2464269
  8. MIHALJ M, TITLIć M, BONACIN D, ĐOGAš Z. Sensomotor axonal peripheral neuropathy as a first complication of polycythemia rubra vera: A report of 3 cases Am J Case Rep [online] :385-387 [viewed 20 September 2014] Available from: doi:10.12659/AJCR.884016
  9. AHMAD F, ASIMI R, RASOOL S. Polycythemia and chorea Ann Saudi Med [online] 2009, 29(1):63-64 [viewed 20 September 2014] Available from: doi:10.4103/0256-4947.51815
  10. ORLOFF MJ, DAILY PO, ORLOFF SL, GIRARD B, ORLOFF MS. A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome Ann Surg [online] 2000 Sep, 232(3):340-352 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1421148
  11. PARIJA S, MOHAPATRA MM, PATTNAIK BK. Polycythemia vera presenting with bilateral papilledema: A rare case report Indian J Ophthalmol [online] 2008, 56(4):327-329 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2636158
  12. MESSINEZY M, PEARSON TC. ABC of clinical haematology. Polycythaemia, primary (essential) thrombocythaemia and myelofibrosis. BMJ [online] 1997 Feb 22, 314(7080):587-590 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2126042
  13. MUGHAL TI, VADDI K, SARLIS NJ, VERSTOVSEK S. Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes Int J Gen Med [online] :89-101 [viewed 20 September 2014] Available from: doi:10.2147/IJGM.S51800

Examination

Fact Explanation
Plethoric appearance Patient will have a plethoric appearance with cyanosis( due to incresed de oxygenated haemoglobin level with excess red blood cell production), congenital suffusion and retinal vein engorgement. This appearance will be prominent in face, palms, nailbeds, mucosa and conjunctiva[8].
Splegnomegaly majority of patients with Polycythemia Rubra Vera will have splenomegaly(75%) on examination.This is one of category A criteria for diagnosis of Polycythemia Rubra Vera[9].
Evidence of bleeding patient will have evidence of excessive bleeding likr haemoptysis on examination of the vomitus, malena/ fresh per rectal bleeding on stool examination. haematuria on urine examination and per vaginal bleeding. On examination there will be ecchymotic patches as well[3].
High blood pressure One third of the patients will have hypertension[1][2].
signs of arterial thrombotic complications With the hyperviscosity there is a increased risk of thrombotic complications. Arterial thrombotic complications can damage various systems giving specific features of hypoperfusion. Eg; In cardiac complications, patient will in a pain, dyspnoic following myocardial infarctions[3]. If thrombotic complications occur in central nervous system, patient will develop seizures and/ or signs of motor/ sensory impairment and crenial nerve palsy[4]. If thrombosis occur in a artery supplying to limbs, patients will in a severe pain, limb will be discoloured, cold and movements will be limited[5].
signs of venous thrombotic complications In deep vein thrombosis patient will in a severe pain with out moving affected limb, limb will be swollen, reddish and warm. When hepatic venous thrombosis examination will reveals generalized oedema, ascites, Jaundice due to liver insufficiency[6]. In cerebral venous thrombosis patient will be confuse and drowsy[7].
Hepatomegaly Some patient(30%) will have associated hepatomegaly[10].
Opthalmic examination There will be retinopathy with retinal haemorrhages, exudate and retinal venous congestion[11].
References
  1. BIGGS B, ANDERTON JL. Reno-vascular disease in polycythaemia rubra vera. Postgrad Med J [online] 1983 Jun, 59(692):382-383 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2417517
  2. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124
  3. AL-FADHLI J, AL-SHAMMARI F, AL-DUAIJ A, AL-SARRAF N. Coronary artery bypass grafting in a patient with polycythaemia rubra vera - a rare indication with a spectrum of complication: a case report Cases J [online] :8126 [viewed 20 September 2014] Available from: doi:10.4076/1757-1626-2-8126
  4. AHMAD F, ASIMI R, RASOOL S. Polycythemia and chorea Ann Saudi Med [online] 2009, 29(1):63-64 [viewed 20 September 2014] Available from: doi:10.4103/0256-4947.51815
  5. ZORASTER RM, RISON RA. Acute embolic cerebral ischemia as an initial presentation of polycythemia vera: a case report J Med Case Rep [online] :131 [viewed 20 September 2014] Available from: doi:10.1186/1752-1947-7-131
  6. ORLOFF MJ, DAILY PO, ORLOFF SL, GIRARD B, ORLOFF MS. A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome Ann Surg [online] 2000 Sep, 232(3):340-352 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1421148
  7. PARIJA S, MOHAPATRA MM, PATTNAIK BK. Polycythemia vera presenting with bilateral papilledema: A rare case report Indian J Ophthalmol [online] 2008, 56(4):327-329 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2636158
  8. MCLORNAN D, PERCY M, MCMULLIN MF. JAK2 V617F: A Single Mutation in the Myeloproliferative Group of Disorders Ulster Med J [online] 2006 May, 75(2):112-119 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1891745
  9. BENEDICT EM, TURNER KB. THE SERUM CALCIUM IN POLYCYTHEMIA VERA J Clin Invest [online] 1930 Oct, 9(2):263-264 [viewed 20 September 2014] Available from: doi:10.1172/JCI100303
  10. HUSSEIN K, GRANOT G, SHPILBERG O, KREIPE H. Clinical utility gene card for: familial polycythaemia vera Eur J Hum Genet [online] 2013 Jun [viewed 20 September 2014] Available from: doi:10.1038/ejhg.2012.216
  11. PARIJA S, MOHAPATRA MM, PATTNAIK BK. Polycythemia vera presenting with bilateral papilledema: A rare case report Indian J Ophthalmol [online] 2008, 56(4):327-329 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2636158

Differential Diagnoses

Fact Explanation
Essential thrombocythaemia In this disease there is a clonal proliferation of megakaryocytes causing continuous increase in platelet levels with poor quality resulting bleeding, arterial/ venous thrombosis and symptoms of microvascular ischemia[1]. In Polycythemia Rubra Vera there is increased red blood cell volume. So from simple investigations like full blood count will reveals the different count changes helping the differentiating both conditions.
Chronic myelogenous leukemia This is a condition associated wit clonal proliferation of myeloid cells. They wll present with cronic symptoms of weight loss, tiredness, fever, sweats, features of gout and bleeding. Philadelphia chromosome is present in majority of patients[2]. In Polycythemia Rubra Vera there won't be a Philadelphia chromosome in genetic testing and there will be JAK2 mutation in a single haemopoietic stem cells[5].
Myeloid metaplasia In myeloid metaplasia there is hyperplasia of megakaryocytes. It produces platelet derived growth factor causing marrow fibrosis and myeloid metaplasia. Patients will have night sweats, fever, weight loss, splenomegaly and evidence of bone marrow failure( bleeeding, recurrent infections, anaemia) Bone marrow biopsy helpful in diagnosis[3]. In Polycythemia Rubra Vera bone marrow bipsy will shows hyper cellular bone marrows with prominent megakaryocytes[6].
Secodary polycythemia secondary polycytemia can occur due to several reasons like high altitude, pulmonary disease with alveolar hypoperfusion(COPD), cardiovascular diseases, heavy smoking with increase in compensatory eruthropoietin, renal disease disease, with some tumours like hepatocellular carcinoma due to inapppropriate increase in erythropoietin. These causes need to be excluded in history, examinations and investigations[4]. In Polycythemia Rubra Vera there will be polycythemia and investigation findings suggestive of the disease( described in investigation section) in the absence of above mentioned causes.
References
  1. BRIèRE JB. Essential thrombocythemia Orphanet J Rare Dis [online] :3 [viewed 20 September 2014] Available from: doi:10.1186/1750-1172-2-3
  2. KELLIHER MA, MCLAUGHLIN J, WITTE ON, ROSENBERG N. Induction of a chronic myelogenous leukemia-like syndrome in mice with v-abl and BCR/ABL. Proc Natl Acad Sci U S A [online] 1990 Sep, 87(17):6649-6653 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC54594
  3. MARKS C, AHMADI M. Agnogenic myeloid metaplasia: role of splenectomy Postgrad Med J [online] 1969 Apr, 45(522):261-265 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2466587
  4. BHATT VR. Secondary Polycythemia and the Risk of Venous Thromboembolism J Clin Med Res [online] 2014 Oct, 6(5):395-397 [viewed 20 September 2014] Available from: doi:10.14740/jocmr1916w
  5. HUSSEIN K, GRANOT G, SHPILBERG O, KREIPE H. Clinical utility gene card for: familial polycythaemia vera Eur J Hum Genet [online] 2013 Jun [viewed 20 September 2014] Available from: doi:10.1038/ejhg.2012.216
  6. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124

Investigations - for Diagnosis

Fact Explanation
Full blood count Due to excessive production of red blood cells, red blood cell count, haemoglobin, haematocrit and pack cell volume will be high. Total red blood cell mass in males need to be > 35ml/Kg and in females should be > 32ml/Kg in diagnosing the disease[1]. Often WBC(60%) will be high. In half of the patients there will be neurophil leucocytosis and some will have increased levels of basophils. To diagnose Polycythemia Rubra Vera WBC count need to be >12x 109/L as a category B criteria and platelet counts( elevated in 60%) need to be > 4000x 109/L[9].
Karyotyping In all most all patients will have cytoplasmic tyrosine kinase Janus-associated kinase 2(JAK2) mutation in haemopoietic cells.This is one of category A criteria for diagnosis of Polycythemia Rubra Vera[2].
Neutrophil Alkaline phosphatase score This will be usually high in patients with Polycythemia Rubra Vera.This is another category B criteria in diagnosing[3].
Vitamin B12 levels and Vitamin B12 binding capacity Result of both of these tests will be high due to increase in haptocorrin. Raised Vitamin B levels also full fill a category B criteria in diagnosing the disease[3][4].
Bone marrow biopsy This will shows hyper cellular bone marrows with prominent megakaryocytes[1].
Serum erythropoietin level This will be low due o excess usage of the hormone[4].
Plasma urate level This is also increased ion these patents[1][5].
Arterial oxygen saturation measure by Arterial Blood Gas analysis This should be >92% to full fill category A criteria in diagnosing Polycythemia Rubra Vera[6].
Total red blood cell mass This can be measured by 51 Cr studies. and this is usually increased[7].
Peripheral blood film This will showa incresed red cell mass with normochromic and normocytic red blood cells. If patient is having history of bleeding/ present witth peptic ulcer disease there will be microcytic, hypochromic red cells[8].
References
  1. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124
  2. HUSSEIN K, GRANOT G, SHPILBERG O, KREIPE H. Clinical utility gene card for: familial polycythaemia vera Eur J Hum Genet [online] 2013 Jun [viewed 20 September 2014] Available from: doi:10.1038/ejhg.2012.216
  3. BENNETT M, STRONCEK DF. Recent advances in the bcr-abl negative chronic myeloproliferative diseases J Transl Med [online] :41 [viewed 20 September 2014] Available from: doi:10.1186/1479-5876-4-41
  4. PARIJA S, MOHAPATRA MM, PATTNAIK BK. Polycythemia vera presenting with bilateral papilledema: A rare case report Indian J Ophthalmol [online] 2008, 56(4):327-329 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2636158
  5. ELLMAN P, BOWDLER AJ. Pernicious Anaemia and Polycythaemia Vera: A Case Report Postgrad Med J [online] 1958 Dec, 34(398):638-642 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2501638
  6. CASSELS DE, MORSE M. THE ARTERIAL BLOOD GASES, THE OXYGEN DISSOCIATION CURVE, AND THE ACID-BASE BALANCE IN POLYCYTHEMIA VERA J Clin Invest [online] 1953 Jan, 32(1):52-59 [viewed 20 September 2014] Available from: doi:10.1172/JCI102711
  7. WEIL JV, JAMIESON G, BROWN DW, GROVER RF. The red cell mass-arterial oxygen relationship in normal man: Application to patients with chronic obstructive airway disease J Clin Invest [online] 1968 Jul, 47(7):1627-1639 [viewed 20 September 2014] Available from: doi:10.1172/JCI105854
  8. AHMAD F, ASIMI R, RASOOL S. Polycythemia and chorea Ann Saudi Med [online] 2009, 29(1):63-64 [viewed 20 September 2014] Available from: doi:10.4103/0256-4947.51815
  9. LUCIE NP, YOUNG GA. Marrow cellularity in the diagnosis of polycythaemia. J Clin Pathol [online] 1983 Feb, 36(2):180-183 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC498146

Investigations - Fitness for Management

Fact Explanation
Full blood count This is useful in assessing the patients polycythemic condition[1].
PT/INR, APTT If the patient is presenting with mucosal bleeding these tests are useful in assessing the clotting atatus of the patient and to exclude any associated clotting disorders[2].
References
  1. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124
  2. SELTMAN HJ, DEKKER A, VAN THIEL DH, BOGGS DR, STARZL TE. Budd-Chiari Syndrome Recurring in a Transplanted Liver Gastroenterology [online] 1983 Mar, 84(3):640-643 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2997624

Investigations - Followup

Fact Explanation
Full blood count This is useful in assessing the patients respond to the treatments by looking at blood counts[1].
ECG and Echocardiogram If the patient present with thrombotic complicatients in cardiovascular system these tests are helpful in confirmation and assessing the patients current condition[2][4].
CT/ MRI brain If the patient present with thrombotic complications in central nervous system these test are useful[3].
Doppler studies of lower limbs/ upper limbs If patient present with peripheral thrombotic complications causing limb ischemia doppler studies will useful in assessing the current condition and in deciding the further treatment[3].
References
  1. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124
  2. VENEGONI P, CYPRUS G. Polycythemia and the heart. A review. Tex Heart Inst J [online] 1994, 21(3):198-201 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC325165
  3. ZORASTER RM, RISON RA. Acute embolic cerebral ischemia as an initial presentation of polycythemia vera: a case report J Med Case Rep [online] :131 [viewed 20 September 2014] Available from: doi:10.1186/1752-1947-7-131
  4. PANDURANGA P, MUKHAINI M, SALEEM M, AL-DELAMIE T, ZACHARIAH S, AL-TAIE S. MOBILE RIGHT HEART THROMBUS WITH PULMONARY EMBOLISM IN A PATIENT WITH POLYCYTHEMIA RUBRA VERA AND SPLANCHNIC VEIN THROMBOSIS Heart Views [online] 2010, 11(1):16-20 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2964707

Investigations - Screening/Staging

Fact Explanation
Genetic screening by karyotyping In all most all patients will have JAK2 mutation in haemopoietic cells. prenatal screening is not indicated in this condition[1][2].
References
  1. HUSSEIN K, GRANOT G, SHPILBERG O, KREIPE H. Clinical utility gene card for: familial polycythaemia vera Eur J Hum Genet [online] 2013 Jun [viewed 20 September 2014] Available from: doi:10.1038/ejhg.2012.216
  2. MCLORNAN D, PERCY M, MCMULLIN MF. JAK2 V617F: A Single Mutation in the Myeloproliferative Group of Disorders Ulster Med J [online] 2006 May, 75(2):112-119 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1891745

Management - General Measures

Fact Explanation
Health education Patient should be educated regarding thee disease, symptoms associated with, possible complications, available treatment options and prognosis[1][2].
References
  1. SCHAFER PW. The Etiology and Treatment of Polycythemia Rubra Vera : Observations Based upon Studies of Body Fluid Changes in Dogs Subjected to Proprioceptor Depressor Neurotomy and Extensive Sympathectomy, Including the Case Report of a Man with Polycythemia Rubra Vera Treated by Extensive Para-Vertebral Sympathectomy Ann Surg [online] 1945 Dec, 122(6):1098-1124 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1618335
  2. WASSERMAN LR. Polycythemia Vera-Its Course and Treatment: Relation to Myeloid Metaplasia and Leukemia Bull N Y Acad Med [online] 1954 May, 30(5):343-375 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1804490

Management - Specific Treatments

Fact Explanation
Venesection(phlebotomy) Venesection is aimed to reduce haematocrit value less then 0.45 to reduce the risk of thrombosis.This is useful in requiring rapid reduction of red blood cell volume. This is indicated in low risk young patients[1][2][3].
Cytotoxic myelosuppression This is indicated in patients with poor response to venesection, symptomatic/progressive splenomegaly, thrombocytosis, weight loss or presence of night sweats. Hydroxycarbamide (Hydroxyuria) mainly, Busulfan, Pipobroman are the drugs used here and it need to continue for long period[3][4][7].
Phosphorus 32 therapy This is useful in either severe disease(history of prior blood clots, increasing age, high blood pressure, diabetes) or in elderly patients. This is another myelosuppressive agent[5][7].
Alpha interferon This is suppress excess production in bone marrow and is preferred in women in child bearing age[6][7].
Aspirin Low dose aspirin is known to be reduce thrombotic complications in these patients[8].
References
  1. SCOTT RB. Treatment of Polycythaemia Rubra Vera Br Med J [online] 1953 May 23, 1(4820):1128-1131 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2016124
  2. VENEGONI P, CYPRUS G. Polycythemia and the heart. A review. Tex Heart Inst J [online] 1994, 21(3):198-201 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC325165
  3. KHETERPAL S, SAYEGH F, CADER A. A case of polycythaemia vera presenting with intramuscular chest wall haematoma. Postgrad Med J [online] 1992 May, 68(799):381-382 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2399407
  4. ISRAëLS MC. Polycythaemia Proc R Soc Med [online] 1966 Nov, 59(11 Pt 1):1100-1102 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1901315
  5. SCHMIDT M. Correspondence (letter to the editor): Radioactive Phosphorus in Polycythemia Vera Therapy Dtsch Arztebl Int [online] 2008 Jun, 105(26):480 [viewed 20 September 2014] Available from: doi:10.3238/arztebl.2008.0480a
  6. XIONG Z, YAN Y, LIU E, SILVER RT, VERSTOVSEK S, YANG F, WANG H, PRCHAL J, YANG XF. Novel tumor antigens elicit anti-tumor humoral immune reactions in a subset of patients with polycythemia vera Clin Immunol [online] 2007 Mar, 122(3):279-287 [viewed 20 September 2014] Available from: doi:10.1016/j.clim.2006.10.006
  7. CARIO H, MCMULLIN MF, PAHL HL. Clinical and hematological presentation of children and adolescents with polycythemia vera Ann Hematol [online] 2009 Aug, 88(8):713-719 [viewed 20 September 2014] Available from: doi:10.1007/s00277-009-0758-y
  8. PANDURANGA P, MUKHAINI M, SALEEM M, AL-DELAMIE T, ZACHARIAH S, AL-TAIE S. MOBILE RIGHT HEART THROMBUS WITH PULMONARY EMBOLISM IN A PATIENT WITH POLYCYTHEMIA RUBRA VERA AND SPLANCHNIC VEIN THROMBOSIS Heart Views [online] 2010, 11(1):16-20 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2964707