Indications for Splenectomy in Patients with Hematologic ... presentation-lisa.pdf · Indications for Splenectomy in Patients with Hematologic Disorders LIZ SIM, MD DOWNSTATE MEDICAL

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


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


CTAP


CTAP


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


Plan IVF resuscitation

Foley insertion

Preparation of RBC

OR for exploratory laparotomy, splenectomy










Operative Course Culture of fibropurulent ascites

Omentectomy, distal pancreatectomy, splenectomy, diaphragm repair

EBL 50

Transfused 300 cc pRBC


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


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


Questions?


Indications for splenectomy Erythrocyte disorders

Platelet disorders

Bone marrow disorders

Cysts, abscess

cancers


Presenter

Presentation Notes

Erythrocyte disorders: membrane defects, hemoglobinopathies, autoimmune

Splenectomy Wait until age 5 in children in possible

Vaccinate prior if possible


Before you operate Get hematology team on board

Oxygenate, hydrate, prepare transfusion

http://www.downstatesurgery.org/files/cases/sc.pdf


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


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


Presenter

Presentation Notes

Atrophy from vascular occlusion and infarction

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% ◦ 2nd most common cause of death in first decade ◦ Abdominal pain, distension, pallor, weakness, tachycardia


Sickle Cell Disease Indications for splenectomy ◦ Persistent splenomegaly ◦ Sequestration ◦ Infarction ◦ Splenic abscess ◦ Developmental delay and delayed growth


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.


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


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


Presenter

Presentation Notes

AGAIN MAY DECREASE TRANSFUSIONS Major and minor forms Symptoms: anemia, plenomegaly, skull bossing, maxillary overgrowth, cardio myopathy, skin pigmentation, endocrine disyfuction Studies show a decrease of transfusion to half of preop value in one year, but rises subsequently

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


Presenter

Presentation Notes

Coombs test positive direct antiglobulin test Warm reactive abx affity to rbc at 37 degrees CLL can cause this and lymphomas as secondary AIHA Symptoms are anemia, sometimes jaundice, ; usually occur after infection Most respond to steroids 90%

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%


Presenter

Presentation Notes

Symptoms. BLEEDING petechaie Failure means >6 weeks on this Or in pregnant patients you will operate First line therapiesinclude corticosteroids ->inhibit RES system biding platelet antibody complex. IVIG inhibits Fc receptor, Rho D in Rh positive childrend Second line: rituximab bind CD 20 decrreaseing autoantibody production; splenectomy High rate of spont remession, delay for 12 months if possible IF RELAPSE SEARCHFOR ACCESSORY SPLEEN

Thrombotic Thrombocytopenic Purpura Deficiency of metalloproteinase that cleaves von Willebrands resulting in platelet clumping

Treatment ◦ Plasma exchange ◦ Splenectomy 8-20% relapse


Presenter

Presentation Notes

Fever, thrombocytopenia, anemia, renal dysfuction, neurologic sequela

Other platelet and WBC disorders Essential thrombocythemia

Polycythemia vera

Primary myelofibrosis

CML

CLL

HD, NHL – for staging


Presenter

Presentation Notes

ALL can cause splenomegaly, Splenectomy only for symptomatic portal hyprtension and drug refractory splenomgaly, transfusion dependence thrombocytopenia

Conclusion Hematologic disorder ◦ Splenomegaly ◦ Anemia requiring excessive transfusions ◦ Warm AIHA



references Cameron 11th edition Edited by John L Cameron, Andrew M Cameron. 2014.

Pediatric Surgery 7th 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 review. 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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Indications for Splenectomy in Patients with Hematologic ... presentation-lisa.pdf · Indications for Splenectomy in Patients with Hematologic Disorders LIZ SIM, MD DOWNSTATE MEDICAL