Risks of Untreated Splenomegaly
Untreated splenomegaly carries significant risks including overwhelming post-splenectomy infection (OPSI), splenic rupture, portal vein thrombosis, and hypersplenism with resulting cytopenias that can lead to increased morbidity and mortality. 1, 2
Major Complications
Infectious Complications
- Increased susceptibility to encapsulated bacteria (Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis) leading to overwhelming infections 1
- Risk of overwhelming post-splenectomy infection (OPSI) with mortality rates of 30-70%, with most deaths occurring within the first 24 hours 1
- Higher risk of OPSI in children younger than 5 years old compared to adults 1
Vascular Complications
- Portal vein thrombosis, which significantly decreases survival in patients with hepatocellular carcinoma 1
- Splenic vein thrombosis leading to portal hypertension and potentially life-threatening esophageal varices 1, 2
- Mesenteric vein thrombosis, which can lead to intestinal infarction in 30-45% of patients, with a 19% fatality rate 1
Mechanical Complications
- Splenic infarction and rupture, which can lead to hemorrhagic shock and hemoperitoneum 1, 2
- Abdominal pain, early satiety, and mechanical discomfort from hepatosplenomegaly 1, 2
- Risk of delayed splenic rupture, particularly within the first 3 weeks after trauma (incidence 0.2%) 1
- Post-traumatic pseudocysts (incidence 0.3%) 1
Hematologic Complications (Hypersplenism)
- Sequestration and destruction of blood cells leading to cytopenias 3
- Thrombocytopenia (most common manifestation) increasing bleeding risk 3
- Anemia contributing to fatigue and decreased quality of life 2
- Leukopenia potentially increasing risk of infections 3
Disease-Specific Manifestations and Complications
Portal Hypertension Related
- Development of esophageal varices with risk of life-threatening bleeding 1, 2
- Ascites and lower extremity edema in chronic presentations 1, 2
- Worsening liver function in cases of cirrhosis 2
Myeloproliferative Disorders
- Constitutional symptoms including fever, weight loss, and night sweats 1, 2
- Cachexia and severe abdominal discomfort 1
- Progression of underlying disease (e.g., myelofibrosis) 1, 4
Splenic Infarction
- Acute left upper quadrant pain 2
- Potential development of splenic abscesses requiring surgical intervention 1
- Hemorrhagic complications 1
Special Considerations
Pediatric Patients
- Higher risk of overwhelming post-splenectomy infection compared to adults 1
- Growth impairment in children on certain treatments 1
- Neonates have more than 30% risk of OPSI 1
Pregnancy
- Increased risk of complications during pregnancy due to mechanical effects and vascular changes 2
- Potential worsening of cytopenias during pregnancy 3
Management Implications
When to Intervene
- Symptomatic splenomegaly with abdominal pain, early satiety, or mechanical discomfort 1
- Evidence of hypersplenism with significant cytopenias 3
- Presence of portal hypertension complications 1
- Splenic infarction with persistent symptoms 1
Treatment Options
- Hydroxyurea is the first-line treatment for symptomatic splenomegaly in myeloproliferative disorders, with approximately 40% response rate 1
- Splenectomy should be considered for drug-refractory symptomatic splenomegaly, though it carries 5-10% perioperative mortality 1
- Low-dose radiation therapy (0.1-1 Gy) for nonhepatosplenic extramedullary hematopoiesis 1
- Treatment of underlying cause is crucial for managing splenomegaly and its complications 3
Preventive Measures
- Immunization against encapsulated bacteria (S. pneumoniae, H. influenzae, and N. meningitidis) 1
- Annual influenza vaccination for patients over 6 months of age 1
- Antibiotic therapy for unexplained fever, malaise, or chills 1
- Avoidance of contact sports to decrease risk of splenic rupture 5