​Symptoms and Signs – Differential Diagnosis of Splenomegaly in Children
DISORDERS OF THE BLOOD
• Hemolytic anemia: congenital/acquired
• Thalassemia
• Sickle cell disease
• Leukemia
• Osteopetrosis
• Myelofibrosis, myeloid metaplasia, thrombocythemia
INFECTIONS: ACUTE AND CHRONIC
Viral
• Congenital (e.g., TORCH association)
• Mononucleosis (e.g., EBV, CMV infection)
• Virus-associated hemophagocytic syndrome
• Human immunodeficiency virus infection
Bacterial
• Sepsis/abscess
• Brucellosis
• Salmonellosis
• Tularemia
• Tuberculosis
• Subacute bacterial endocarditis
• Syphilis
• Lyme disease
Fungal
• Histoplasmosis (disseminated)
Rickettsia
• Rocky Mountain spotted fever
• Cat-scratch disease
Parasitic
• Toxoplasmosis
• Malaria
• Leishmaniasis (kala-azar)
• Schistosomiasis
• Echinococcosis
HEPATIC/PORTAL SYSTEM DISORDERS
• Acute/chronic active hepatitis
• Cirrhosis, hepatic fibrosis, biliary atresia
• Portal or splenic venous obstruction (Banti’s syndrome)
AUTOIMMUNE DISEASE
• Juvenile rheumatoid arthritis
• Systemic lupus erythematosus
• Autoimmune lymphoproliferative syndrome (Canale-Smith
syndrome)
NEOPLASMS/CYSTS
• Lymphomas (Hodgkin’s and non-Hodgkin’s)
• Hemangiomas/lymphangiomas
• Hamartomas
• Congenital or acquired (post-traumatic) cysts
STORAGE DISEASES/INBORN ERRORS OF METABOLISM
• Lipidoses: Gaucher’s disease, Niemann-Pick disease, others
• Mucopolysaccharidoses
• Defects in carbohydrate metabolism: galactosemia, fructose
intolerance
• Sea-blue histiocyte syndrome
MISCELLANEOUS DISORDERS
• Histiocytoses
• Reactive Langerhans cell
• Malignant sarcoidosis
• Congestive heart failure
• Familial Mediterranean fever