Splenomegaly is not usually associated with benign conditions, and is often a sign of a serious underlying pathology. The causes of splenic enlargement are numerous, and it helps to think of them as part of three distinct categories: Congestive, Infiltrative, and Proliferative. Regardless of the cause, increased vigilance is needed when approaching a patient with splenomegaly.

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Hematology – Splenomegaly
Whiteboard Animation Transcript
with Saqib Abbasi, MD and Abbas Husain, MD
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An enlarged spleen is a physical exam finding that has a broad differential.

Let’s investigate the three primary causes; congestion, infiltration, and proliferation: we can remember it as C-I-P.

First let’s look at congestion.

Increased blood pressure in the splenic vein can cause a cascade of congestion, hyperplasia and fibrosis in the spleen. This can be due to direct occlusion of the splenic vein such as in a splenic vein thrombosis (a complication of pancreatitis) or any pathology that causes portal hypertension – such as cirrhosis or right heart failure. Remember to look for signs and symptoms of these diseases.

In cirrhosis, we see jaundice, varices, and spider angiomas. Pancreatitis can present with severe epigastric pain radiating to the back. Identifying an enlarged spleen in this case can point towards an increased risk for thrombotic complications.

Next let’s look at infiltration.

The spleen is highly vascularized and plays a central role as a peripheral lymphatic organ. As such, it is prone to invasion by malignant and non-malignant causes.

Malignancies include lymphomas and leukemias, as well as metastatic solid tumours. Depository diseases such as amyloidosis and sarcoidosis will likely manifest in other organs as well. Also, the spleen is often one of the primary sites of extramedullary hematopoiesis in myeloproliferative disorders.

That brings us to proliferation.

The spleen is a lymphatic organ which, instead of filtering lymph; filters blood. In doing so, it is a major source of protection against blood borne pathogens as well as other blood abnormalities which can cause the spleen to become hyperfunctioning.

This can be the result of acute infections, which can be viral (CMV, HIV, EBV), bacterial (TB), fungal (histoplasmosis) as well as parasitic (malaria, visceral leishmaniasis) in nature.

Filtering the blood of abnormal cells / structures can cause hyperplasia of the spleen. These include spherocytosis, hemoglobinopathies, and sickle cell disease.

An enlarged spleen should never be ignored and a proper history and physical can make this broad differential much easier to stratify. Remember it’s a simple as C-I-P.

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