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Causes of asplenia and hyposplenism

Causes of asplenia and hyposplenism
Iatrogenic
  • Splenic artery embolization  
  • Splenic irradiation  
  • Total or partial splenectomy
Hepatic diseases
  • Alcoholic liver disease
  • Chronic viral hepatitis
  • Cirrhosis and portal hypertension  
  • Primary biliary cirrhosis
Hematologic diseases
  • Acute leukemia  
  • Chronic lymphocytic leukemia  
  • Hematopoietic stem cell transplantation (particularly if associated graft versus host disease)  
  • Lymphoma  
  • Mastocytosis  
  • Myeloproliferative neoplasm  
  • Sickle cell disease (eg, HbSS, HbSC, HbS-beta thalassemia)
  • Thalassemia
Vascular impairment
  • Celiac artery thrombosis  
  • Splenic artery thrombosis
  • Splenic vein thrombosis
Gastrointestinal diseases
  • Celiac disease
  • Inflammatory bowel disease
  • Whipple's disease
Autoimmune diseases
  • Antiphospholipid antibody syndrome  
  • Goodpasture's syndrome  
  • Granulomatosis with polyangiitis  
  • Hashimoto's thyroiditis  
  • Polyarteritis nodosa  
  • Rheumatoid arthritis  
  • Sjogren's syndrome  
  • Systemic lupus erythematosus
Infectious diseases
  • HIV/AIDS
Infiltrative diseases
  • Amyloidosis  
  • Sarcoidosis
Congenital syndromes
  • Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome  
  • Hypoparathyroidism syndrome  
  • Isolated congenital hyposplenia
  • Ivemark's syndrome
  • Normal neonatal state (particularly if birth is premature)  
  • Other congenital or familial syndromes
  • Stormorken's syndrome
Asplenia and hyposplenism can be either anatomic or functional. Anatomic asplenia is most often due to surgical removal of the spleen. Functional asplenia and hyposplenism result from conditions that lead to atrophy, infarction, engorgement, or infiltration of the spleen.
Data from:
  1. Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. Lancet 2011; 378:86.
  2. Kirkineska L, Perifanis V, Vasiliadis T. Functional hyposplenism. Hippokratia 2014; 18:7.
  3. Rubin LG, Schaffner W. Clinical practice. Care of the asplenic patient. N Engl J Med 2014; 371:349.
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