Asplenia Morning Report January 12, 2010 Lindsay Kruska.
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Transcript of Asplenia Morning Report January 12, 2010 Lindsay Kruska.
AspleniaAsplenia
Morning Report Morning Report
January 12, 2010January 12, 2010
Lindsay KruskaLindsay Kruska
The SpleenThe Spleen
Clear opsonized bacteriaClear opsonized bacteria Adaptive immunity - Lymphoid role, Adaptive immunity - Lymphoid role,
Antigen processing Antigen processing – 50% Ig producing B lymphocytes50% Ig producing B lymphocytes
Phagocytosis of erythrocytes, debrisPhagocytosis of erythrocytes, debris Recycles ironRecycles iron Production of FVII, vWF, extramedullary Production of FVII, vWF, extramedullary
hematopoeisis, sequester plthematopoeisis, sequester plt
EtiologiesEtiologies
CongenitalCongenital SurgicalSurgical FunctionalFunctional
– Repeated infarctions Repeated infarctions – Splenic Artery ThrombosisSplenic Artery Thrombosis– Acute engorgementAcute engorgement
Splenic sequestration crisis with SS, malaria, Splenic sequestration crisis with SS, malaria, splenic vein thrombosissplenic vein thrombosis
– InfiltrationInfiltration Sarcoidosis, amyloidosis, cysts, tumorsSarcoidosis, amyloidosis, cysts, tumors
HyposplenismHyposplenism
SLESLE RARA SarcoidosisSarcoidosis Systemic vasculitisSystemic vasculitis Ulcerative colitisUlcerative colitis Celiac diseaseCeliac disease AmyloidosisAmyloidosis Chronic GvHDChronic GvHD MastocytosisMastocytosis Congenital and acquired immunodeficiencyCongenital and acquired immunodeficiency
Visceral HeterotaxyVisceral Heterotaxy
HeterosHeteros– other + – other + TaxisTaxis – order – order 0.8% congenital heart disease associated0.8% congenital heart disease associated 2 main types – spleen is ~always involved2 main types – spleen is ~always involved
– AspleniaAsplenia– Polysplenia – no increased risk infectionPolysplenia – no increased risk infection
Cardiac malformations variableCardiac malformations variable– Asplenia: malformations of conotruncus and AV Asplenia: malformations of conotruncus and AV
canal (gestationally 30-32d)canal (gestationally 30-32d) Pulmonary malformationsPulmonary malformations
– Asplenic patients have trilobar lungs BLAsplenic patients have trilobar lungs BL– Polysplenic patients have bilobar lungs BLPolysplenic patients have bilobar lungs BL
Recognizing Recognizing Asplenia/HypospleniaAsplenia/Hyposplenia Suspect with associated Suspect with associated
conditionsconditions Most splenectomies laproscopicMost splenectomies laproscopic Peripheral smearPeripheral smear
– Howell-Jolly bodiesHowell-Jolly bodies– nRBCnRBC– PappenPappenhiemer bodieshiemer bodies
http://www.healthsystem.virginia.edu/internet/hematologyhttp://en.wikipedia.org/wiki/File:Howell.jpg
Risks of AspleniaRisks of Asplenia
Fulminant SepsisFulminant Sepsis Encapsulated organismsEncapsulated organisms
– Strep pneumo most important - 57% Strep pneumo most important - 57% sepsis, 59% deathssepsis, 59% deaths
– Haemophilus influezae – 7% sepsis, 32% Haemophilus influezae – 7% sepsis, 32% deaths (kids prior to H. flue vaccination)deaths (kids prior to H. flue vaccination)
– Neisseria – may or may not be increasedNeisseria – may or may not be increased Other bacteria: Capnocytophaga Other bacteria: Capnocytophaga
canimorsus, Bordetella holmesiicanimorsus, Bordetella holmesii Parasites: Babesiosis, malariaParasites: Babesiosis, malaria
PreventionPrevention
Avoid splenectomyAvoid splenectomy ImmunizationsImmunizations
– PneumococcalPneumococcal– Haemophilus influenzaeHaemophilus influenzae– MeningococcalMeningococcal– When to administerWhen to administer
14d prior or post14d prior or post
– InfluenzaInfluenza– Live attenuated vaccinationsLive attenuated vaccinations
Prevention, cont.Prevention, cont.
Daily prophylactic antibioticsDaily prophylactic antibiotics Oral empiric antibioticsOral empiric antibiotics
– Amoxicillin/clavulanate 875 BIDAmoxicillin/clavulanate 875 BID– Cefuroxime 500 BIDCefuroxime 500 BID– FQ: Levofloxacin 750 dailyFQ: Levofloxacin 750 daily
TreatmentTreatment
Well appearing, suspected viral illnessWell appearing, suspected viral illness– Eval, consider blood culturesEval, consider blood cultures– Broad spectrum antibiotics x7-10dBroad spectrum antibiotics x7-10d
Acutely ill: hospitalize pending bld cxAcutely ill: hospitalize pending bld cx– If in clinic – rx ceftriaxone 2g IV priorIf in clinic – rx ceftriaxone 2g IV prior– Broad coverage: Vancomycin 1g q12h Broad coverage: Vancomycin 1g q12h
initially + (Ceftriaxone 2g IV OR FQ)initially + (Ceftriaxone 2g IV OR FQ)
TreatmentTreatment
Revaccinate after 5yRevaccinate after 5y Consider 7v PCV if pneumococcal Consider 7v PCV if pneumococcal
sepsis in vaccinated personsepsis in vaccinated person
ReferencesReferences
Hoffman. Hematology: Basic Principles and Hoffman. Hematology: Basic Principles and Practices, 5Practices, 5thth ed. ed.
Keane. Nadas’ Pediatric Cardiology. 2Keane. Nadas’ Pediatric Cardiology. 2ndnd ed. ed. UTDOL. Jan 2010. UTDOL. Jan 2010. Patient knowledge of the risks of post-Patient knowledge of the risks of post-
splenectomy sepsis. ANZ J Surg. 2008 splenectomy sepsis. ANZ J Surg. 2008 Oct;78(10):867-70. Oct;78(10):867-70.
Evaluation of severe infection and survival Evaluation of severe infection and survival after splenectomy. Am J Med. 2006 after splenectomy. Am J Med. 2006 Mar;119(3):276.1-7. Mar;119(3):276.1-7.