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Asplenia

Asplenia. Morning Report January 12, 2010 Lindsay Kruska. The Spleen. Clear opsonized bacteria Adaptive immunity - Lymphoid role, Antigen processing 50% Ig producing B lymphocytes Phagocytosis of erythrocytes, debris Recycles iron

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Asplenia

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  1. Asplenia Morning Report January 12, 2010 Lindsay Kruska

  2. The Spleen • Clear opsonized bacteria • Adaptive immunity - Lymphoid role, Antigen processing • 50% Ig producing B lymphocytes • Phagocytosis of erythrocytes, debris • Recycles iron • Production of FVII, vWF, extramedullary hematopoeisis, sequester plt

  3. Etiologies • Congenital • Surgical • Functional • Repeated infarctions • Splenic Artery Thrombosis • Acute engorgement • Splenic sequestration crisis with SS, malaria, splenic vein thrombosis • Infiltration • Sarcoidosis, amyloidosis, cysts, tumors

  4. Hyposplenism • SLE • RA • Sarcoidosis • Systemic vasculitis • Ulcerative colitis • Celiac disease • Amyloidosis • Chronic GvHD • Mastocytosis • Congenital and acquired immunodeficiency

  5. Visceral Heterotaxy • Heteros– other + Taxis – order • 0.8% congenital heart disease associated • 2 main types – spleen is ~always involved • Asplenia • Polysplenia – no increased risk infection • Cardiac malformations variable • Asplenia: malformations of conotruncus and AV canal (gestationally 30-32d) • Pulmonary malformations • Asplenic patients have trilobar lungs BL • Polysplenic patients have bilobar lungs BL

  6. Heterotaxy-asplenia

  7. Recognizing Asplenia/Hyposplenia • Suspect with associated conditions • Most splenectomies laproscopic • Peripheral smear • Howell-Jolly bodies • nRBC • Pappenhiemer bodies http://www.healthsystem.virginia.edu/internet/hematology http://en.wikipedia.org/wiki/File:Howell.jpg

  8. Risks of Asplenia • Fulminant Sepsis • Encapsulated organisms • Strep pneumo most important - 57% sepsis, 59% deaths • Haemophilus influezae – 7% sepsis, 32% deaths (kids prior to H. flue vaccination) • Neisseria – may or may not be increased • Other bacteria: Capnocytophaga canimorsus, Bordetella holmesii • Parasites: Babesiosis, malaria

  9. Prevention • Avoid splenectomy • Immunizations • Pneumococcal • Haemophilus influenzae • Meningococcal • When to administer • 14d prior or post • Influenza • Live attenuated vaccinations

  10. Prevention, cont. • Daily prophylactic antibiotics • Oral empiric antibiotics • Amoxicillin/clavulanate 875 BID • Cefuroxime 500 BID • FQ: Levofloxacin 750 daily

  11. Treatment • Well appearing, suspected viral illness • Eval, consider blood cultures • Broad spectrum antibiotics x7-10d • Acutely ill: hospitalize pending bld cx • If in clinic – rx ceftriaxone 2g IV prior • Broad coverage: Vancomycin 1g q12h initially + (Ceftriaxone 2g IV OR FQ)

  12. Treatment • Revaccinate after 5y • Consider 7v PCV if pneumococcal sepsis in vaccinated person

  13. References • Hoffman. Hematology: Basic Principles and Practices, 5th ed. • Keane. Nadas’ Pediatric Cardiology. 2nd ed. • UTDOL. Jan 2010. • Patient knowledge of the risks of post-splenectomy sepsis. ANZ J Surg. 2008 Oct;78(10):867-70. • Evaluation of severe infection and survival after splenectomy. Am J Med. 2006 Mar;119(3):276.1-7.

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