Asplenia Morning Report January 12, 2010 Lindsay Kruska

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

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

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

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

Heterotaxy-asplenia

Recognizing Asplenia/Hyposplenia Suspect with associated conditions Suspect with associated conditions Most splenectomies laproscopic Most splenectomies laproscopic Peripheral smear Peripheral smear –Howell-Jolly bodies –nRBC –Pappenhiemer bodies

Risks of Asplenia Fulminant Sepsis Fulminant Sepsis Encapsulated organisms 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 Other bacteria: Capnocytophaga canimorsus, Bordetella holmesii Parasites: Babesiosis, malaria Parasites: Babesiosis, malaria

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

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

Treatment Well appearing, suspected viral illness Well appearing, suspected viral illness –Eval, consider blood cultures –Broad spectrum antibiotics x7-10d Acutely ill: hospitalize pending bld cx 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)

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

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