Diseases and Disorders
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Polycythemia

INTRODUCTION

DRG Category: 841

Mean LOS: 6.7 days

Description MEDICAL: Lymphoma and Non-Acute Leukemia with CC

classification section:



Polycythemia as a generic term refers to an increased concentration of red blood cells (RBCs, or erythrocytes). This blood disorder has several causes and can be classified as primary, secondary, or relative polycythemia (Table 2).

Types of Polycythemia

TYPEDEFINITIONSIGNS AND SYMPTOMSPHYSICAL EXAMINATION
Primary polycythemia (polycythemia vera)Chronic proliferative bone marrow disorder that leads to overproduction of RBCs, white blood cells (WBCs), and platelets; results in increased blood viscosity and platelet dysfunction

• Early: feeling of fullness in the head, tinnitus, headache, dizziness, hypertension, blurred vision, night sweats, epigastric pain, joint pain, pain on walking

• Late: pruritus (abnormal histamine metabolism), abdominal fullness, pleuritic pain, epistaxis, gingival bleeding

Engorged veins in the fundus and retina of the eye on fundoscopic examination, congestion of conjunctiva, congested oral mucous membranes, tenderness of ribs and sternum on palpation, ruddy cyanosis, enlarged liver and spleen
Secondary polycythemiaExcessive production of RBCs because of hypoxemia, tumors; often triggered by overproduction of erythropoietin (hormone produced primarily in the kidney and necessary for RBC production)Shortness of breath from underlying pulmonary disease, symptoms from underlying disease processesRuddy cyanosis, ecchymosis, spoon-shaped nails, clubbing of fingers
Relative polycythemiaCaused by reduced plasma volume; actual RBC count is normal or even reduced; increased blood concentration occurs because of increased concentration of cells compared with plasmaSymptoms often vague: headache, dizziness, fatigue, dyspnea, diaphoresis, claudication, ruddy appearance, enlarged liver and spleen, hypoventilationRuddy appearance, enlarged liver and spleen, hypoventilation



Most complications of all types of polycythemia occur as a result of increased blood viscosity (hyperviscosity) or sudden blood loss (hemorrhage). Hyperviscosity may lead to thromboembolic events, such as organ thromboses or splenomegaly. Hemorrhage in any system can occur from platelet dysfunction. Hemorrhage and vasculitis may occur together because an excessive number of RBCs exert pressure on capillary walls. Specific complications include gangrene of the fingers and toes, hypertension, peptic ulcer disease, and cerebrovascular accident. Life expectancy for patients with polycythemia if it is left untreated is roughly 2 years.

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