Polycythemia vera

Venous stasis Major medical illness (e.g., congestive heart failure) Major surgery (e.g., general anesthesia for greater than 30 minutes) Paralysis (e.g., due to stroke or spinal cord injury) Polycythemia vera Obesity Varicose veins... 

Hydroxyurea is an oral drug that inhibits ribonucleotide reductase, which converts ribonucleotides into the deoxyribuon-cleotides used in DNA synthesis and repair. The time to peak concentrations of hydroxyurea is 1 to 2 hours after oral administration. Approximately 50% is degraded by the liver to form urea and respiratory carbon dioxide. The remainder is excreted by the kidney. The half-life ranges from 3.5 to 4.5 hours. Hydroxyurea has shown clinical activity in the treatment of chronic myelocytic leukemia, polycythemia vera, and thrombocytosis. The major side effects are myelo-suppression, nausea and vomiting, diarrhea, and constipation. Rash, mucositis, and renal tubular dysfunction occur rarely. 

Hydroxy urea (HU), a hydroxylated derivative of urea, has long been used in the treatment of various forms of neoplastic disorders such as polycythemia vera, head and neck cancer, chronic granulocytic leukemia, and more recently in sickle cell disease, / thalassemia and HIV infection [1],... 

Venous stasis is slowed blood flow in the deep veins of the legs resulting from damage to venous valves, vessel obstruction, prolonged periods of immobility, or increased blood viscosity. Conditions associated with venous stasis include major medical illness (e.g., heart failure, myocardial infarction), major surgery, paralysis (e.g., stroke, spinal cord injury), polycythemia vera, obesity, or varicose veins. 

Its primary indications are myeloproliferative disorders, including chronic granulocytic leukemia, polycythemia vera, and essential thrombocytosis. It is also used in combination with radiotherapy for head and neck cancer and for carcinoma of the cervix. Hydroxycarbamide is well absorbed after oral administration. It is in part metabolized in the liver and also excreted unchanged in the urine its elimination half-life is 2-5 hours. Its major toxicity consists of short lasting bone marrow depression. 

Campbell PJ, Green AR. Management of polycythemia vera and essential thrombocythemia. Hematology Am Soc Hematol Educ Program 2005 201-8. 

Pearson TC, Messinezy M, Westwood N, Green AR, Bench AJ, Green AR et al. Polycythemia vera updated diagnosis, pathobiology, and treatment. Hematology (Am Soc Hematol Educ Program) 2000 51-68. 

Allopurinol is especially indicated in the treatment of chronic tophaceous gout, since patients receiving it show a pronounced decrease in their serum and urinary uric acid levels. Because it does not depend on renal mechanisms for its efficacy, allopurinol is particularly beneficial for patients who already have developed renal uric acid stones, patients with excessively high urate excretion (e.g., above 1,200 mg in 24 hours), patients with a variety of blood disorders (e.g., leukemia, polycythemia vera), patients with excessive tophus deposition, and patients who fail to respond well to the uricosuric drugs. 

After oral administration, it is well absorbed and excreted in urine as methanesulfonic acid. It is mainly used in chronic myeloid leukaemia, polycythemia vera, essential thrombocythemia and myelofibrosis. 

It is indicated in treatment of chronic granulocytic leukaemia, polycythemia vera, essential thrombocytosis, melanoma. 

Anticoagulant therapy is also sometimes used in cases of congestive heart failure and in the treatment of polycythemia vera (elevation of the packed cell volume or the hemoglobin level) where not contraindicated. 

Radiophosphorus is used in the treatment of patients with a number of diseases, lliis element has a half-life of about 14 days and emits beta rays. It is taken up by die body in the greatest quantity by those tissues which manufacture blood cells. In polycythemia vera, a condition in which too many red blood cells arc formed, the radiation from this isotope often brings about a sufficient suppression of the blood cell-making tissues to alleviate some of the symptoms of the disease. Leukemia patients, in whom there is an excessive production of white cells, are offered added comfort and, m some instances, prolongation of life by the use of radiophosphorus. This element also may be used m treatment of metastatic cancer to the bone and, although the treatment is not used in an attempt to eradicate cancer, it can result in significant palliation of pain in some patients. 

Polycythemia vera Busulfan, chlorambucil, or cyclophosphamide Radioactive phosphorus 32... 

Platelet counts above 400,000/pil are referred to as thrombocytosis, Thrombocytosis is classified as being either primary or secondary, Primary thrombocytosis or essential thrombo-cythemia is associated with chronic myeloproliferative disorders such as chronic myeloid leukemia, polycythemia vera, and agnogenic myeloid metaplasia. Exclusion-based diagnosis of essential thrombocythemia is made when patients... 

Common risk factors for developing branch retinal vein thrombosis (BRVT) and central retinal vein thrombosis (CRVT) include increased plasma fibrinogen, diabetes, decreased exercise, hypertension, and hyperviscosity (205). Sickle cell anemia, polycythemia vera, and other proliferative disorders may also lead to this syndrome. 

Hepatic venous thrombosis, also known as Budd-Chiari syndrome, is caused by hypercoagulable disorders precipitated by pregnancy, infection, and birth control medication. An acute painful abdomen, sudden enlargement of the liver, and the presence of ascites make up a triad of clinical symptoms that are important in the diagnosis of this syndrome. Myeloproliferative disorders such as polycythemia vera and paroxysmal nocturnal dyspnea were previously thought to be responsible. Factor V Leiden and prothrombin 20210 mutations are also known to be responsible, Other intraabdominal thromboses include portal vein thrombosis, mesenteric vein thrombosis and renal vein thrombosis. 

Murphy S, Hand H, Rosnethal D, et al. Essential thrombo-cythemia an interim report from the polycythemia vera study group, Semin Hematol 1986 23 177-182,... 

Le Blanc K, Berg A, PalmUad J, Samudsson J. Stimulus-spedfic defect in platelet aggregation in polycythemia vera Euro J Haematol 1994 53 145-149... 

The presence of greater than normal amounts of thrombocytes in the circulation is known as thrombocytosis and along with reticulocytosis and leukocytosis is a manifestation of increased activity of the hematopoietic system. Zucker and Woodard (Z2) reported a series of 12 patients with thrombocytosis, consisting of two cases of polycythemia vera, three of essential thrombocytemia, three of chronic granulocytic leukemia, one myeloproliferative syndrome, one erythroleukemia, and one cancer of the bladder. The platelet counts ranged from 685 X 10 to 2500 X 10 per cubic millimeter, all much above the upper limit of normal. 

Fig. 7. The minor hemoglobin components of patients with various hematological disorders. From left to right hemolytic anemias, polycythemia vera, leukemias, iron-deficiency anemia, lupus erythematosus, malabsorbed vitamin Bu., Post splenectomy. From Horton and Huisman (H24) with permission of the authors and publisher.

There are several acquired hematological disorders which have an increased synthesis of Hb-F [summarized in (H37, K13)], Thus, an elevated level of Hb-F is frequently observed in, for example, Fanconi s anemia, megaloblastic anemia, polycythemia vera, various types of leukemia, multiple myeloma and lymphomas, macroglobulinemia, and metastatic disease of the bone marrow. The chemical heterogeneity of the Hb-F in some of these disorders (aplastic anemia, Fanconi s anemia, and various forms of leukemia) has been studied (H60, R29). In many instances, the to ratio is similar to that found in the normal newborn. This is particularly striking for patients with various forms of leukemia, and the Hb-F which is produced in increased quantities in these disorders invariably has a °y to y ratio of about 3 1. This newborn ratio of °y to y chains in Hb-F may be a common factor in these diseases as perhaps another example of fetal characteristics in red cells of patients with leukemia, it supports the hypothesis that these cells may be of fetal origin (H25). 

A patient with polycythemia vera who had undergone splenectomy received six courses of busulfan for recurrent thrombocytosis over 19 years (22). Severe pancytopenia followed and persisted for 4 months. There was marked eiythroid hjrperplasia in the bone marrow, with striking dyserythropoiesis, PAS-positive erythrocyte precursors, moderate numbers of circulating normoblasts, and evidence of chronic and acute hemolysis. 

Pezzimenti JF, Kim HC, Lindenbaum J. Eiythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera. Cancer 1976 38(6) 2242-6. 

A 70-year-old woman with polycythemia rubra vera and Guillain-Barre syndrome, but no known risk factors for thrombosis, had a cerebral infarction 10 days after receiving intravenous immunoglobulin the authors wondered whether there was a relation to the polycythemia vera (48). 

Sodium Phosphate P 32. Sodium phosphate P 32. sodium radiophosphate, is supplied as an aqueous solution of a mixture of NaH2 P04 and Na2H P04 with a pH range ofS.O to 6.0. It contains S mCi/vial (0.67 mCi/mL) of radio-xtivity. expressed as a pure /3-emitter with a half-life of 14.3 days. Sodium phosphate P 32 is indicated for treatment of polycythemia vera. chronic myelocytic leukemia, and CLL Depression of leukocytes and platelets requires monitoring of blood and bone marrow at regular intervals. 

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