Polycythemia vera is a a chronic, life-shortening blood disorder in which there is an increase in all blood cells, particularly red blood cells. The increase causes erythrocytosis and hemoglobin concentration, which results in thicker blood. Effects of this disorder can lead to strokes or tissue and organ damage.

The cause of the condition is unknown, but it is thought to result from various potential stem cell defects.

The condition may be characterized by:

• visual disturbances

• congestion of the conjunctiva

• retina

• retinal veins

• mucous membranes of the mouth

• nose or gum bleeding

Other signs and symptoms include:

• headache

• lethargy

• syncope

• paresthesia of the fingers and toes

• hypertension

• dyspnea

• intermittent claudication

• thrombosis and angina

• hemorrhage

Other manifestations may also include:

• pruritus

• urticaria and ruddy cyanosis

• epigastric distress

• early food satisfaction (satiety) and fullness

• peptic ulcer pain

• bleeding diarrhea

• hepatomegaly and splenomegaly

• joint discomfort and bone pain may also be presented

Tests may include blood work for: complete blood work (CBC); arterial blood gases (ABG); carboxyhemoglobin; B12-binding capacity. Other tests may also include: renal sonography or computed tomography (CT) scan; urinalysis; and bone marrow biopsy.

Treatment may include: phlebotomy; myelosuppressive therapy; radioactive phosphorus; or chemotherapy agents; and drug therapy to reduce serum uric acid levels. The person must avoid crowds due to the lowered resistance to infections.

The person may be in remission for months to several years. However, presently, there is no permanent cure. There may be required, repeated blood tests and phlebotomies until treatment is effective. Close management of the person is needed to observe for bleeding episodes, joint discomfort, and complications such as hypervolemia, thrombocytosis, and impending stroke (CVA). There may be signs and symptoms of iron deficiency.

• Consider service connection for polycythemia vera on a presumptive basis as a condition associated with ionizing radiation exposure (38 CFR 3.307; §3.311(b)(3); §3.311(b)(4)) when the Veteran has cited or submitted competent scientific or medical evidence that the claimed condition is a radiogenic disease.

• Public Law 98-542 requires VA to determine whether sound medical and scientific evidence supports establishing a rule identifying polycythemia vera as a radiogenic disease. VA has determined that sound medical and scientific evidence does not support including polycythemia vera on the list of known radiogenic diseases in this regulation. Even so, VA will consider a claim based on the assertion that polycythemia vera is a radiogenic disease under the provisions of paragraph (b)(4) of this section. 38 CFR 3.311(b)(3);

• Rate complications such as hypertension, gout, stroke or thrombotic disease separately. (38 CFR 4.117 [Schedule of ratings-hematologic & lymphatic systems])

• If the condition undergoes leukemic transformation, evaluate as leukemia under diagnostic code 7703.

• A 100 percent evaluation shall be assigned as of the date of hospital admission for peripheral blood or bone marrow stem cell transplant; or during the period of treatment with chemotherapy (including myelosuppressants). Six months following hospital discharge or, in the case of chemotherapy treatment, six months after completion of treatment, the appropriate disability rating shall be determined by mandatory VA examination. Any reduction in evaluation based upon that or any subsequent examination shall be subject to the provisions of 38 CFR 3.105(e).