Difference Between Thrombocytopenia and Hemophilia

The presence of abnormally low levels of platelets in the blood is termed as thrombocytopenia. It does not have any genetic predisposition and is most often due to various acquired causes that impair the production of platelets. At the same time, it is more appropriate to consider thrombocytopenia as a clinical sign rather than an individual disease. Apart from thrombocytopenia and a handful of few other conditions, a vast majority of the hematological disorders are due to the inherited genetic effects most often transmitted to the next generation of offsprings via the female carriers. Hemophilia is one such hematological disorder that is almost exclusively seen in males which is due to the deficiency of factor VIII or factor IX. The key difference between thrombocytopenia and hemophilia is that the thrombocytopenia is the decrease in platelet level while hemophilia is the decrease in the concentration of factor VIII or IX.

CONTENTS

1. Overview and Key Difference
2. What is Thrombocytopenia
3. What is Hemophilia
4. Similarities Between Thrombocytopenia and Hemophilia
5. Side by Side Comparison – Thrombocytopenia vs Hemophilia  in Tabular Form
6. Summary

What is Thrombocytopenia?

The presence of abnormally low levels of platelets in the blood is termed as thrombocytopenia. Patients with this condition have a tendency to bleed, and the bleeding prominently happens from small capillaries and venules rather than from large vessels. Consequently, there can be numerous punctuate hemorrhages throughout the body tissues. On the skin, this is manifested as a thrombocytic purpura that is characterized by the presence of small purplish blotches.

Bleeding does not happen until the platelet level drops below 50000/.

Clinical Features

• Petechiae
• Easy bruising
• Prolonged bleeding even after a minor injury
• Bleeding from gums
• Epistaxis
• Hematuria
• Heavy menstrual bleeding
• Yellowish discoloration of the skin (jaundice)

Causes

• Splenomegaly
• Leukemia
• Idiopathic thrombocytopenia
• Chronic alcoholism
• Viral infections such as hepatitis C
• Pregnancy
• Adverse effects of different drugs such as heparin
• Hemolytic uremic syndrome

Investigation

A full blood count can reveal the abnormally low levels of platelets. Further investigations may be required based on the clinical suspicion of underlying pathology.

Management

• If thrombocytopenia is due to an immune response, immune suppressants have to be administered. Corticosteroids are the drug of choice to suppress undue inflammatory processes and to boost the platelet production
• A lethally low level of platelets may require the immediate transfusion of blood products and platelets to avoid life-threatening
• If splenomegaly is the cause of thrombocytopenia, surgical resection of the spleen is necessary.
• Different other medical and surgical interventions may be required depending on the underlying pathology

What is Hemophilia?

Hemophilia is a hematological disorder that is almost exclusively seen in males. In a majority of the cases, this disease is due to the deficiency of clotting factor VIII in which case it is known as classic hemophilia or hemophilia A. The other less frequently seen form of hemophilia, which is known as hemophilia B, is due to the deficiency of clotting factor IX.

The inheritance of both these factors is through the female chromosomes. Consequently, the chance of a female getting hemophilia is extremely low since it is unlikely for both of their chromosomes to be mutated simultaneously. The women whose only one chromosome is deficient are called hemophilia carriers.

Clinical Features

• Severe Hemophilia (factor concentration is less than 1IU/dL)

This is characterized by spontaneous bleeding from early life, typically into the joints and muscles. If not treated properly, the patient can get joint deformities and can even be crippled.

• Moderate Hemophilia ( factor concentration is between 1-5 IU/dL)

This is associated with severe bleeding following an injury and occasional spontaneous bleedings.

• Mild Hemophilia (factor concentration is more than 5 IU/dL)

There are no spontaneous bleedings in this condition. Bleeding occurs only after an injury or during surgeries.

Investigations

• Prothrombin Time is normal
• APTT is increased
• The factor VIII or factor IX levels are abnormally low

Treatment

• Intravenous infusion of factor VIII or factor IX to normalize their levels

The half-life of factor VIII is 12 hours. Therefore it has to be administered at least twice a day to maintain the appropriate levels. On the other hand, it is sufficient to infuse factor IX once in a week because it has a longer half-life of 18 hours.

What are the Similarities Between Thrombocytopenia and Hemophilia?

• Both are hematological disorders.
• Abnormal bleeding is the most common and prominent clinical feature of both thrombocytopenia and hemophilia.

What is the Difference Between Thrombocytopenia and Hemophilia?

Summary – Thrombocytopenia vs Hemophilia

The presence of abnormally low levels of platelets in the blood is termed as thrombocytopenia. Hemophilia, on the other hand, is a hematological disorder caused by the deficiency of factor VIII or factor IX and is almost exclusively seen in males. The principal difference between thrombocytopenia and hemophilia is that in thrombocytopenia the platelet level drops down, but in hemophilia, it is the concentration of factor VIII or factor IX that is abnormally decreased.

References

1. Kumar, Parveen J., and Michael L. Clark. Kumar & Clark clinical medicine. Edinburgh: W.B. Saunders, 2009.
2. Hall, John E., and Arthur C. Guyton. Guyton and Hall textbook of medical physiology. 12th ed. Philadelphia, PA: Elsevier, 2016.

Image Courtesy:

1. “Purpura” By User:Hektor – the English Wikipedia (CC BY-SA 3.0) via Commons Wikimedia
2. “Hemophilia 02” By National Heart Lung and Blood Institute (NIH) – National Heart Lung and Blood Institute (NIH) (Public Domain) via Commons Wikimedia