Polycythemia Vera Disease

DESCRIPTION

A clonal cell hematologic malignant disorder with excessive erythroid, myeloid and megakaryocytic elements in the bone marrow. It is one of a group of myeloproliferative disorders.

System(s) affected: Hemic/Lymphatic/Immunologic
Genetics: Unknown genetic pattern (some suggestion that a chromosomal abnormality may be involved)
Incidence/Prevalence in USA: 0.5 per 100,000
Predominant age: Middle to late years, mean is 60 years (range 15-90)
Predominant sex: Male > Female (slightly)

SIGNS AND SYMPTOMS

Early stages may produce no symptoms
Headaches
Tinnitus
Vertigo
Blurred vision
Epistaxis
Increased blood viscosity
Spontaneous bruising
Upper GI bleeding
Peptic ulcer disease
Arterial and venous occlusive events
Pruritus
Sweating
Weight loss
Plethora (face, hands, feet)
Splenomegaly
Hepatomegaly
Hyperhistaminemia
Bone pain (ribs and sternum)
Bone tenderness (ribs and sternum)

CAUSES

Unknown, all three hematopoietic cell lines originate in a single clone

RISK FACTORS

Jewish ancestry (may have increased frequency)
Familial history (rare)

LABORATORY

Tests used for diagnosis of polycythemia vera
- A1: Increased RBC mass - female ≥ 32 mL/kg, male ≥ 36 mL/kg
- A2: Normal arterial oxygen saturation (≥ 92%)
- A3: Splenomegaly
- B1: Thrombocytosis platelet count > 400,000/µL
- B2: Leukocytosis > 12,000/µL
- B3: Leukocyte alkaline phosphatase increased
- B4: Increased serum B12 or increased unsaturated vitamin B12 binding capacity (UB12CB)
Diagnosis acceptable with following combinations:
- A1 + A2 + A3
- A1 + A2 + any 2 from B category (splenomegaly absent in about 25% of patients)
Other lab findings
- Hyperuricemia
- Hypercholesterolemia
- Elevated blood histamine level

Drugs that may alter lab results: Diuretics may cause a spurious polycythemia
Disorders that may alter lab results: Excessive use of alcohol or tobacco

PATHOLOGICAL FINDINGS

Plethoric congestion in all organs and tissues
Major vessels contain thick, viscous blood
Sinuses of spleen packed with red blood cells

SPECIAL TESTS

Bone marrow aspiration (red cell hyperplasia, absent iron stores) and biopsy (fibrosis during spent phase of the disease)

IMAGING

CT - splenomegaly

DIAGNOSTIC PROCEDURES

Bone marrow aspiration - hyperplastic and panmyelosis

APPROPRIATE HEALTH CARE

Outpatient

GENERAL MEASURES

Individualized management necessary. Dependent on many factors - age, disease duration, disease phenotype, complications, disease activity.
Currently, phlebotomy is mainstay of therapy. Beyond that, differences exist among authorities about use and effectiveness of myelosuppressives.

Phlebotomy
- To reduce hematocrit to approximately 45%
- Performed as often as every 2 or 3 days until normal hematocrit reached. Phlebotomies of 250-500 m/L. Reduce to 250-350 m/L in elderly patients or patients with cardiovascular disease.
- Concomitant therapy possibilities, e.g., some form of myelosuppression, radioactive phosphorus (in elderly patients)
- Phlebotomy repeated as necessary for maintenance
- If patient cannot tolerate phlebotomy - chemotherapy (hydroxyurea is the least mutagenic agent) or radiation therapy
Other therapy
- Maintain hydration
- Pruritus therapy
- Manage thrombotic or hemorrhagic complications the same as with nonpolycythemic patient
- Uric acid reduction therapy

SURGICAL MEASURES

N/A

ACTIVITY

No restrictions

DIET

No special diet (iron replacement not necessary)
Phlebotomy regimen will produce pica, resulting in craving for crisp green vegetables (lettuce, celery) and ice

PATIENT EDUCATION

Lifelong maintenance
Complications to watch for

PREVENTION/AVOIDANCE

No known preventive measures

POSSIBLE COMPLICATIONS

Uric acid stones
Secondary gout
Vascular thromboses (major cause of death)
Transformation to leukemia
Hemorrhage
Peptic ulcer
Increased risk for complications and mortality from surgery procedures. Assess risk-benefits and assure optimal control of disorder before any elective surgery.

EXPECTED COURSE AND PROGNOSIS

Median survival without treatment - 6 to 18 months following diagnosis
Survival up to 10 years with treatment
Some patients live, symptom-free, for 20 or more years

ASSOCIATED CONDITIONS

Budd-Chiari syndrome
Mesenteric artery thrombosis

AGE-RELATED FACTORS

Pediatric: Rare in this age group
Geriatric: Phlebotomies and other therapies need to be adjusted for patients over 70
Others: N/A

PREGNANCY

Treat with phlebotomy alone

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.