www.downstatesurgery.org Indications for Splenectomy in Patients with Hematologic Disorders LIZ SIM, MD DOWNSTATE MEDICAL CENTER
www.downstatesurgery.org Case presentation 12 yom with three day history of abdominal pain, fevers and AMS PMHx: Sickle cell requiring transfusions q2 weeks; CVA, developmental delay, seizure PSHx: denies Meds: miralax, folic acid, exjade, hydroxyurea, oxcarbazepine, keppra, clobazam Allergies: Ceftriaxone
www.downstatesurgery.org Case presentation Vitals: 101 116/76 149 30 99% PE: ◦ Lethargic, nonverbal ◦ Tachypneic, tachycardic ◦ Abd: rigid, splenomegaly, diffusely tender Labs: ◦ Chem: 139/3.6/111/18/15/0.24/119 ◦ CBC 22>7.8/22.9<154 BASELINE:6.7/20.9 ◦ LA 2.5->0.8
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www.downstatesurgery.org Case Presentation U/S: hepatomegaly, cholelithiasis, enlarged spleen with no flow CTAP: Enlarged spleen 14.2 cm with poor enhancement, cholelithiasis, hepatomegaly 14 cm, ascites
www.downstatesurgery.org Plan IVF resuscitation Foley insertion Preparation of RBC OR for exploratory laparotomy, splenectomy
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www.downstatesurgery.org Operative Course Culture of fibropurulent ascites Omentectomy, distal pancreatectomy, splenectomy, diaphragm repair EBL 50 Transfused 300 cc pRBC
www.downstatesurgery.org Post Op Course POD 0: Chest tube insertion POD 1: ◦ Febrile, tachycardic ◦ 1 unit pRBC transfused POD 2: ◦ Extubated, ◦ mucous plug of RUL POD 3- ◦ CT removed ◦ Rx for pneumonia begun
www.downstatesurgery.org Post Op Course POD 5 -10 ◦ Bowel function ◦ Seizure activity ◦ NGT removed and PO feeds initiated POD 11 ◦ DC JP and patient Plan for vaccinations and antibiotic rx in PCP office All Cx negative; path necrotic spleen
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www.downstatesurgery.org Indications for splenectomy Erythrocyte disorders Platelet disorders Bone marrow disorders Cysts, abscess cancers
www.downstatesurgery.org Splenectomy Wait until age 5 in children in possible Vaccinate prior if possible
www.downstatesurgery.org Before you operate Get hematology team on board Oxygenate, hydrate, prepare transfusion http://www.downstatesurgery.org/files/cases/sc.pdf
www.downstatesurgery.org Membrane defects Spherocytosis ◦ Ankyrin, spectrin, band 3 protein defects -> increase susceptibility to destruction ◦ anemia, when severe do splenectomy (children in hb 6-8 range) ◦ CURRATIVE Eliptocytosis ovalocytosis
www.downstatesurgery.org Sickle Cell Disease Autosomal recessive substitution of valine for glutamine in B chain More susceptible to oxidative stress leading to deformed RBC Spleen goes from splenomegaly to atrophy within first decade of life Unless.. ◦ Splenic sequestration
www.downstatesurgery.org Splenic Sequestration Splenic sequestration: red blood cells become entrapped in spleen causing splenomegaly and anemia of 2 gram drop Acute vs chronic ◦ Incidence 7-30% ◦ 2 nd most common cause of death in first decade ◦ Abdominal pain, distension, pallor, weakness, tachycardia
www.downstatesurgery.org Sickle Cell Disease Indications for splenectomy ◦ Persistent splenomegaly ◦ Sequestration ◦ Infarction ◦ Splenic abscess ◦ Developmental delay and delayed growth
www.downstatesurgery.org Splenectomy vs Conservative management for acute sequestration crises in people with sickle cell disease Cochrane review Limited to case studies, no RCT tirals Outweigh risks vs. benefits.
www.downstatesurgery.org Overwhelming post splenectomy infection Typically encapsulated organisms Usually within first 3-4 years In trauma less then 1%, in hematolgic disorders 15-20% High mortality
www.downstatesurgery.org Thalassemia Autosomal recessive resulting in defective alpha and beta globulin subunits ◦ Abnormal shaped RBC destroyed by the spleen May require chronic transfusion and iron chelators Splenectomy: ◦ Painful hypersplenism ◦ Transfusions>250ml/kg/year
www.downstatesurgery.org Autoimmune Hemolytic Anemia Antibodies to RBC antigens ◦ Shortened red blood cell survival ◦ Positive Combs test Rx: STEROIDS to decrease autoantibody formation ◦ Splenectomy in warm reactive antibodies only if: ◦ Failed high dose steroids ◦ 20% cured ◦ 50% have decrease in steroid requirement
www.downstatesurgery.org Immune/Idiopathic thrombocytopenic Purpura ITP Most common indication for elective splenectomy Autoantibodies to platelets and removal in the RES Treatment based on bleeding and platelet count ◦ Corticosteroids works 20%, high remission rate 60-80% ◦ IV Ig, immunosuppressives (rituximab, cyclophosphamide, azathioprine) ◦ Failure of medical management or persistent platelet count <10000-> splenectomy ◦ Long term response 60%
www.downstatesurgery.org Thrombotic Thrombocytopenic Purpura Deficiency of metalloproteinase that cleaves von Willebrands resulting in platelet clumping Treatment ◦ Plasma exchange ◦ Splenectomy 8-20% relapse
www.downstatesurgery.org Other platelet and WBC disorders Essential thrombocythemia Polycythemia vera Primary myelofibrosis CML CLL HD, NHL – for staging
www.downstatesurgery.org Conclusion Hematologic disorder ◦ Splenomegaly ◦ Anemia requiring excessive transfusions ◦ Warm AIHA
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www.downstatesurgery.org references Cameron 11th edition Edited by John L Cameron, Andrew M Cameron . 2014. Pediatric Surgery 7 th edition. Edited by Arnaol Coran, NB Adzick, T Krummel, J. Laberage, etc. 2012 Shirley Owusu-Ofori, Tracey Remmington. Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane revi ew. 2015. Issue 9, Art No CD003425 Packman CH. The Clinical Pictures of Autoimmune Hemolytic Anemia. Transfusion Medicine and Hemotherapy . 2015;42(5):317-324. doi:10.1159/000440656. Akhtar IK, Ashraf M, Khalid IU, Hussain M. Surgical outcome of spelenectomy in Thalassemia major in children . Pakistan Journal of Medical Sciences . 2016;32(2):305-308. doi:10.12669/pjms.322.8815.
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