OVERVIEW

What is hypersplenism?

Hypersplenism is a syndrome clinically characterized by splenomegaly, reduction of one or more types of blood cells, and active bone marrow hematopoiesis. It is classified into primary and secondary types.

Primary hypersplenism has no identifiable cause and is also called idiopathic hypersplenism, which is relatively rare. Secondary hypersplenism refers to hypersplenism that occurs as a complication of various underlying diseases.

What are the functions of the spleen?

The spleen is the largest immune organ in the human body and plays a crucial role in clearing aged blood cells and removing harmful substances. Additionally, the spleen is an important blood storage organ, containing a large number of blood cells that can be released into circulation when needed.

SYMPTOMS

What are the symptoms of hypersplenism?

• Hypersplenism causes a reduction in one or more types of blood cells:

• A decrease in red blood cells may lead to anemia, with symptoms such as pale complexion and fatigue;

• A decrease in white blood cells may weaken immune function, making patients prone to infections and fever;

• A decrease in platelets may cause subcutaneous bruising, bleeding gums, or nosebleeds.

• Patients with hypersplenism may also exhibit symptoms of the underlying disease, such as ascites, hematemesis, or bloody stools in cases of post-hepatitis cirrhosis and portal hypertension, or fever and weight loss in malaria patients.

What severe consequences can hypersplenism cause?

• Hypersplenism reduces blood cells, leading to anemia (low red blood cells), impaired immunity (low white blood cells), and bleeding tendencies (low platelets). Among these, bleeding tendencies are more common and can have serious consequences, such as prolonged bleeding from wounds or gums, subcutaneous hematomas from minor trauma, or even spontaneous bleeding.
• If hypersplenism is caused by portal hypertension, it may be accompanied by esophageal or rectal varices, which can result in uncontrollable gastrointestinal bleeding that may be life-threatening.
• In some patients, the spleen may become significantly enlarged. A massively enlarged spleen is vulnerable to injury from abdominal trauma, leading to splenic rupture and severe hemorrhage, requiring emergency surgical intervention.

CAUSES

What are the common causes of secondary hypersplenism?

• Portal hypertension: Includes cirrhosis of various causes (post-hepatitis, alcoholic, chronic toxicity, etc.), portal vein and splenic vein thrombosis, hepatic vein obstruction (Budd-Chiari syndrome), etc.
• Infectious diseases: Malaria, schistosomiasis, kala-azar, viral hepatitis, subacute bacterial endocarditis, etc.
• Hematologic disorders: Leukemia, malignant lymphoma, thalassemia, idiopathic thrombocytopenic purpura, etc.
• Connective tissue diseases: Systemic lupus erythematosus, rheumatoid arthritis, etc.
• Other rare causes: Lipid storage diseases, etc.
• In China, the most common causes of hypersplenism are post-hepatitis B cirrhosis and portal hypertension.

DIAGNOSIS

Which department should be consulted for hypersplenism?

Patients are usually referred to the hematology department due to a reduction in one or more types of blood cells. When surgical treatment for hypersplenism is required, they should consult general surgery or hepatobiliary surgery. However, for treating the underlying disease causing hypersplenism, patients should visit internal medicine specialties such as infectious diseases or gastroenterology.

What tests should be done for hypersplenism?

• For splenomegaly, if the spleen is not palpable below the ribs, a B-mode ultrasound of the splenic area can provide clinical reference.
• Red blood cell count, white blood cell count, or platelets may be reduced individually or simultaneously, but a hyperplastic bone marrow may be observed.
• Splenectomy can restore blood cell counts to near or normal levels.

TREATMENT

What are the treatment options for hypersplenism?

Currently, the primary treatment is splenectomy.

Historically, the spleen was considered a dispensable organ in adults, but recent immunological and physiological studies have overturned this misconception. Some spleen-preserving techniques, such as splenic artery embolization and partial splenectomy, have been reported, though their efficacy remains under observation.

Concurrently, addressing the underlying cause is crucial. For example, patients with post-hepatitis cirrhosis require long-term antiviral therapy to prevent further progression of cirrhosis and worsening hypersplenism, while those with malaria or schistosomiasis should receive prompt antiparasitic treatment.

What are the effects of splenectomy for hypersplenism on the human body?

• Splenectomy impacts immune function and blood cell metabolism.
• Overwhelming post-splenectomy infection (OPSI) is the most severe complication, typically occurring 2–3 years after surgery. Initial symptoms may resemble mild flu-like illness but can rapidly progress to septic shock with high mortality. While rare in adults, the incidence is significantly higher in children. Therefore, total splenectomy is generally avoided in children aged 5 or younger.

Why is a "spleen" still detected after total splenectomy for hypersplenism?

Some individuals have accessory spleens—small, functional spleen-like structures separate from the main spleen.

Accessory spleens may be adjacent to or distant from the primary spleen, ranging in size from micrometers to centimeters, and can be single or multiple. If small and non-adjacent, preoperative imaging may miss them, leading to unintentional retention during surgery.

These accessory spleens can hypertrophy, forming a new "spleen." Patients may experience temporary relief of hypersplenism symptoms post-surgery, followed by recurrence.

DIET & LIFESTYLE

What dietary precautions should patients with hypersplenism take?

For patients with cirrhosis and portal hypertension, if esophageal and gastric varices are detected during gastroscopy, a low-residue diet is recommended to prevent coarse fiber in food from damaging the varicose veins.

Focus on soft foods like porridge and noodles. Vegetables should be cut into small pieces and thoroughly cooked. Chew slowly during meals and avoid overeating. Avoid spicy, raw, cold, or highly acidic foods. At the same time, ensure adequate and balanced nutrition.

What daily life precautions should patients with hypersplenism take?

Prevent injuries and avoid strenuous exercise. If bleeding from a wound does not stop, seek medical attention promptly at a qualified healthcare facility.

PREVENTION

Can Hypersplenism Be Prevented?

Some secondary hypersplenism can actually be prevented:

• Patients with chronic viral hepatitis should receive early and long-term antiviral treatment to prevent cirrhosis and portal hypertension, thereby preventing hypersplenism;
• Patients with malaria or schistosomiasis should undergo timely and standardized antiparasitic treatment to eliminate the infection before splenomegaly or hypersplenism occurs.
• For patients with idiopathic hypersplenism, prevention is difficult due to unknown causes.

Can Hypersplenism Recur?

In patients with accessory spleens, symptoms of hypersplenism may reappear once the accessory spleen reaches a certain size and functional state. These patients may require accessory splenectomy.