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Asplenia Morning Report January 12, 2010 Lindsay Kruska

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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

Heterotaxy-aspleniaHeterotaxy-asplenia

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.