Clinical Features of polycythaemia

Polycythemia is a condition in which there is elevation of hemoglobin in the blood more than the normal. When hemoglobin level raise that increases the blood viscocity and lead to variety of manifestation. As it take time to increase the hemoglobin level the symptoms are also insidious. Level of hemoglobin at which each patient develop symptom vary, depending on bodies ability to adapt to the situation. Initially symptoms are mild but as the Hb is more than 20 g/d Llife threatening complication can occur.

General appearance

Increased hemoglobin and blood level causes noticeable A diffuse dusky redness of face which is called facial plethora, with bloodshot eyes  and diffuse purplish redness of oral mucosa and,palms

Conjuctival injection can occur.

Severe itching after hot bath or when the patient is warm is a feature of polycythemia which is called aquagenic pruritus.

Gout due to increased uric acid level is common.

Some patients experience stomach ulceration and heartburn.Severe malaise and fatigue are also reported.

Neurologic symptoms

Due to increased blood viscosity patients experience vertigo, tinnitus, headache, visual disturbances,mental clouding and TIA  (transient ischemic attack)

Hypertension is also common in these patients.

Arterial and venous occlusion

Life threatening complication can occur when the hemoglobin is more than   20 g/dL.At this level hyperviscocity is markedly increased and blood flow to vital organ decreases.The most common organs affected are brain ,heart, abdomen liver .Manifestation include stroke ,heart attack,hepatic vein occlusion,ischemia to the digits.

Bleeding manifestation.

Patients can develop life threatening hemorrhage. This is due to hyperviscosity leading to rupure of vessels. Unteated polycythemia patients are at risk of increased bleeding during surgery or trauma.Other bleeding manifestation include  easy bruising,epistaxis ,gastrointestinal hemorrhage.On examination spleenomegaly is common in Primary polycythemia patients.

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What is polycythemia ?

The term polycythemia means there is an elevation of hemoglobin above the normal level. Blood contain cellular components and fliud compartment .One of the cellular component is RBC (red cell corpuscles )and the fluid compartment is called plasma. Hemoglobin elevation can be absolute or relative. The term relative means elevation in hemoglobin is secondary to decrease in plasma volume that is fluid part of blood. RBC cell mass is measured as PCV (packed cell volume )or hematocrit. It is a disease of middle  aged people or elderly

Criteria for diagnosis of polycythemia are given below

Hemoglobin level - more than 17 g/dLfor men.

Hemoglobin level - more than 15 g/dL for women.

Hematocrit levels > 50% in men.

Hematocrit levels > 45% in women may be abnormal.

Hematocrits > 60% in men and >55% in women  is definitely associated with Elevated RBC mass.

Elevated PCV in a patient with true polycythaemia due to congenital cyanotic heart disease (left) compared to a blood sample taken from a person with a normal PCV (right ). Dark part represent the RBC mass, yellow part the plasma. In the first one there is a rise in RBC mass representing polycythemia.It is very important to identify whether the polycythemia is true or apparent. True polycythemia can be  primary or secondary.

Apparent or spurious polycythaemia

In apparent or relative polycythaemia RBC mass is not elevated and the increased packed cell volume is secondary to a fall in the plasma volume. It may be associated with dehydration  smoking,  excess alcohol intake, obesity, diuretics, and hypertension.

True polycythemia

Polycythaemia vera is the term given to Primary polucythemia. Exact cause not known, genetic factors play a significant role.

Secondary polycythaemia

Here the rise in hemoglobin is secondary to definite cause. There are a variety of secondary causes of polycythemia most common is decreased oxygen content of blood and disease of kidney, cigarette smoking. Other causes are given in the table below.

Relative polycythaemias

Hemoconcentration secondary to dehydration, diuretics, ethanol abuse, androgens or tobacco abuse

Causes of Absolute polycythaemias are the following

1.Hypoxia is seen in 

Carbon monoxide intoxication

High oxygen-affinity hemoglobin

High altitude

Pulmonary disease

Right to left cardiac or vascular shunts

Sleep apnea syndrome

2.Renal Disease

Renal artery stenosis

Focal sclerosing or membranous glomerulonephritis

Postrenal transplantation

Renal cysts

3.Tumors

Hypernephroma

Hepatoma

Cerebellar hemangioblastoma

Uterine myoma

Adrenal tumors

Meningioma

Pheochromocytoma

4.Drugs

Androgens

Recombinant erythropoietin

Detection of polycythemia is important irrespective of the cause because when there is a rise in hemoglobin or RBC mass it increase the the whole blood viscosity. As a result of rise in viscosity there can be reduction of  blood supply to the organ and reduced oxygen supply which in turn lead to a variety of clinical symptoms. 

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