Vaquez's disease

Alternative names

Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Polycythemia vera; Osler's disease; Polycythemia with chronic cyanosis - Myelopathic polycythemia; Erythrocytosis megalosplenica; Cryptogenic polycythemia

Definition

Polycythemia vera is an abnormal increase in blood cells (primarily red blood cells) resulting from excess production by the bone marrow.

Causes, incidence, and risk factors

Polycythemia vera is an acquired disorder of the bone marrow that causes the overproduction of all three blood cell lines: white blood cells, red blood cells, and platelets.

It is a rare disease that occurs more frequently in men than women, and rarely in patients under 40 years old. It is not known what causes polycythemia vera.

The disease usually develops slowly, and most patients do not experience any problems related to the disease after being diagnosed. However, the abnormal bone marrow cells may begin to grow uncontrollably in some patients leading to acute myelogenous leukemia.

Patients with polycythemia vera also have an increased tendency to form blood clots that can result in strokes or heart attacks. Some patients may experience abnormal bleeding because their platelets are abnormal.

Symptoms

• Headache
  
• Dizziness
  
• Itchiness, especially following a warm bath
  
• Fullness in the left upper abdomen
  
• Red coloration, especially of the face
  
• Shortness of breath
  
• Breathing difficulty, lying down
  
• Symptoms of phlebitis
  

Note: Symptoms are due to increased blood viscosity and clotting.

Additional symptoms that may be associated with this disease:

• Vision abnormalities
  
• Skin spots, red
  
• Skin discoloration, bluish
  
• Fatigue
  

Signs and tests

• A CBC with differential
  
• Hematocrit, elevated
  
• White blood count, elevated
  
• Platelet count, elevated
  
• Bone marrow biopsy