What are the causes of splenomegaly in the elderly?

Causes of Splenomegaly in the Elderly

The most common causes of splenomegaly in the elderly include liver disease (particularly cirrhosis with portal hypertension), hematologic malignancies, and infections, with myeloproliferative disorders being a leading cause of massive splenomegaly. 1, 2, 3

Hepatic Causes

• Cirrhosis with portal hypertension is a frequent cause of splenomegaly in elderly patients, often presenting as isolated splenomegaly due to clinically inapparent cirrhosis 4, 5
• Wilson's disease can present with isolated splenomegaly due to clinically inapparent cirrhosis with portal hypertension, though this is less common in the elderly population 4, 2
• Chronic liver disease may lead to splenomegaly through portal hypertension, with patients potentially having other signs of liver disease such as jaundice and ascites 2, 5

Hematologic Causes

• Myeloproliferative disorders, particularly myelofibrosis, are associated with massive splenomegaly (>10cm below costal margin) and are common in elderly patients 2, 6
• Smoldering multiple myeloma (SMM) should be considered in patients over 65 years, as the median age at diagnosis ranges from 65 to 70 years 4
• Chronic leukemias, particularly chronic monocytic leukemia, can cause splenomegaly in the elderly 7, 3
• Lymphoproliferative disorders including lymphomas are important causes of splenomegaly in older adults 1, 3

Infiltrative and Storage Disorders

• Acid sphingomyelinase deficiency (ASMD) can present with hepatosplenomegaly, which may be massive (>10x normal size), though this is less commonly diagnosed initially in elderly patients 1, 2
• Other lysosomal storage diseases like Gaucher disease and Niemann-Pick disease should be considered, though they are typically diagnosed earlier in life 1, 2
• Multiple capillary angiomas of the spleen can cause splenomegaly and may be discovered incidentally in elderly patients 7

Infectious Causes

• Splenic tuberculosis, though uncommon, should be considered in the differential diagnosis of splenomegaly in the elderly, particularly in endemic areas 7
• Splenic abscess, particularly in patients with endocarditis, presents with persistent fever and left upper quadrant pain 2
• Parasitic infections such as splenic echinococcosis are rare but reported causes 7

Vascular Causes

• Splenic vein thrombosis can lead to isolated splenomegaly due to localized portal hypertension 2, 5
• Portal vein thrombosis may be associated with splenomegaly and carries a poor prognosis in certain conditions 2

Diagnostic Approach

• Abdominal ultrasound should be performed to confirm splenomegaly and assess for other abdominal pathology, including signs of portal hypertension 1, 8
• Complete blood count is essential to assess for cytopenias (anemia, thrombocytopenia, leukopenia) which may indicate portal hypertension or hematologic disorders 8, 3
• Liver function tests should be obtained to evaluate for hepatic causes of splenomegaly 8
• Bone marrow examination may be informative in patients older than 60 years or those with systemic symptoms 4, 8
• CT angiography or MRI may be needed to evaluate vascular structure and potential complications 2, 9

Clinical Pearls and Pitfalls

• Do not assume immune thrombocytopenia (ITP) is the cause of splenomegaly, as less than 3% of ITP patients have splenomegaly 8
• Mild splenomegaly may be found in younger patients with ITP, but moderate or massive splenomegaly suggests an alternative cause 4
• Constitutional symptoms such as fever or weight loss, hepatomegaly, or lymphadenopathy might indicate underlying disorders such as HIV, systemic lupus erythematosus, or lymphoproliferative disease 4
• In some cases, diagnostic splenectomy with histological examination may be necessary as the final stage of differential diagnosis when other methods fail to identify the cause 7, 9