The Spleen

Lecture ConferenceSteven J. Binenbaum, MD

SLRSeptember 12th, 2007

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic Diseases

Rarely cures the disease

Alleviates symptoms

Corrects hematologic abnormalities

Staging & Diagnosis

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic Diseases

Red Cell-Related IndicationsRed Cell-Related Indications

Hereditary Spherocytosis (HS)Hereditary Spherocytosis (HS) Most common type of hemolytic anemia Autosomal dominant Spectrin deficiency (RBC membrane) → Loss of osmotic stability Osmotic fragility testing Splenomegaly & Gall stones Dx by (+) spherocytes in the blood

TOC is Splenectomy in the 4th year of life +/- cholecystectomy if (+) cholelithiasis

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic Diseases

Autoimmune Hemolytic Anemia (AIHA)Autoimmune Hemolytic Anemia (AIHA) Warm-antibody autoimmune HA Autoantibody opsonization and phagocytosis Intravascular destruction or by spleen macrophages Treatment Of Choice

#1 is Corticosteroids 1-2 mg/kg/day#1 is Corticosteroids 1-2 mg/kg/day PRBC’s for severe anemia SplenectomySplenectomy (80% favorable clinical response) if:

medical tx fails Intolerance to steroids or its side-effects

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic Diseases

Pyruvate kinase DeficiencyPyruvate kinase Deficiency Autosomal-recessive disease Splenomegaly TOC

SplenectomySplenectomy Reduces PRBC’s requirements

G6PD DeficiencyG6PD Deficiency Splenomegaly is rare Splenectomy is not indicated!

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic Diseases

Sickle Cell Disease (SCD)Sickle Cell Disease (SCD) B-globin gene A→T substitution (Hb S/SS) autosomal dominant Sickling of RBCs in tissues with low O2 tension Red & White pulps Splenic microinfarcts

Painful Abscess Infections Anemia

Splenectomy (palliative)Splenectomy (palliative) Acute sequestration crises (recurrence = 40%-50% with 20% Mortality rate) Rapid hypersplenism Abscess formation

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic Diseases

ThalassemiaThalassemia Thalassemia major (homozygous B) autosomal-dominant disease Decreased expression of beta-chains Pallor, Growth retardation, head enlargement Splenectomy indicated if:

Symptomatic splenomegaly Anemia Pain due to infarctions increased PRBC’s requirements (>200 ml/kg/year)

↑ rate of infections after splenectomy Risk vs. Benefit

Splenectomy for Hematologic DiseasesSplenectomy for Hematologic DiseasesPlatelet Related IndicationsPlatelet Related Indications

ITPITP Spleen is not enlarged ♀ > ♂; Renal insufficiency children vs. adults

Self-limited in children (70%) Splenectomy is for rare cases

Insidious onset in adults ↓ Plts < 10,000/mm3

#1 TOC is PO Steroids 1-1.5 mg/kg/d (up to 70% response)

IVIG 1 g/kg/d x2-3 days Splenectomy if fail steroid tx

TTPTTP Splenomegaly Microvascular thrombosis Petechiae, fever, neurologic changes #1 TOC

Plasmaphoresis Splenectomy is #2

Durable remission? Avoid PLT transfusions

↑ morbidity

White Cell - Related IndicationsWhite Cell - Related Indications

LeukemiaLeukemia CLL & Hairy cell leukemia (HCL) Splenectomy

improves cytopenias (75%) Ameliorates symptomatic splenomegaly

Non-Hodgkin’s Lymphoma (NHL)Non-Hodgkin’s Lymphoma (NHL) Splenectomy

Painful splenomegaly Cytopenia

No role for staging

White Cell - Related IndicationsWhite Cell - Related Indications

Hodgkin’s DiseaseHodgkin’s Disease Current indications for surgical staging:

Stage I or Stage II with NS histology and w/o B-symptoms

Staging procedure Wedge liver biopsy LN sampling:

Retroperitoneal Mesenteric Hepatoduodenal Splenectomy

Bone Marrow – Related Indications

Myelofibrosis & Myeloproliferative disorders Splenectomy for symptoms due to enlarged spleen

Splenectomy ComplicationsSplenectomy Complications

LLL atelectasis, pneumonia, effusion Hemorrhage (mostly with laparoscopic) Intraabdominal abscess (LUQ) Pancreatitis or fistula formation DVT PVT

Hemolytic anemia or myeloproliferative dz with splenomegaly

OPSI

Splenectomy ComplicationsSplenectomy Complications

OPSI Incidence ≈1% (up to 5%); 50% Mortality Encapsulated gram-positive bacteria = Streptococcus Risk factors

Children < 15 yrs old Immunosupression Hematologic dz (thalassemia, SCD, etc) Highest within the first 2 yrs post splenectomy

Pnemococcus, H.influenza, meningococcusPnemococcus, H.influenza, meningococcus vaccination 7-14 days prior to splenectomy

Pneumovax booster Q5yrs and annual H.influenza immunizations Abx prophylaxis for children x2 yrs post splenectomy Lower incidence of OPSI in adults (vs children) and after trauma

Tumors, Cysts, and Tumors, Cysts, and Abscesses of the SpleenAbscesses of the Spleen

Steven J. Binenbaum, MD

September 12th, 2007

SLR

Malignant TumorsMalignant Tumors

Spleen - mostly secondary involvement non-Hodgkin’s Lymphoma – most common malignancy

Main Tx: Chemo +/- RT

Spleen is the primary site 10% Hodgkin’s disease 30% of resected spleens (staging procedure) have (+) histology

Hairy cell leukemia Resect for symptomatic splenomegaly

Improved survival

CML & CLL symptomatic splenomegaly = splenectomy

Malignant TumorsMalignant Tumors

AngiosarcomaAngiosarcoma Nonlymphoid malignant tumor of the spleen Early metastatic disease Aggressive with rapid growth Spontaneous splenic rupture and hemolytic anemia Palliation

Benign TumorsBenign Tumors Hemangioma

Risk of rupture + platelet sequestration (Syndrome?) No tx unless symptomatic

Hamartoma Lymphangioma

Splenic CystsSplenic Cysts

Nonparasitic Epithelium-lined cysts Epidermoid cysts – most common Mostly asymptomatic Young children + young adults LUQ pain, N/V, early satiety Dx: CTscan (+)unilocular +/- Ca++

Complications Infection, bleeding, rupture Tx: Splenectomy

partial

Parasitic 5% in US Abroad – Hydatid disease Echinococcus granulosusEchinococcus granulosus Mostly asymptomatic

Associated with liver cysts

If only spleen is involved? Tx: Splenectomy

Splenic CystsSplenic Cysts

Splenic PseudocystsSplenic Pseudocysts Lack epithelial lining Account for most cystic splenic dz in US

Pancreatic pseudocyst Posttraumatic

Splenectomy is indicated when: Size >10 cm or symptomatic

Splenic AbscessSplenic Abscess Uncommon, but fatal

Erode into adjacent structures Most are secondary in etiology

Bacterial endocarditis Intrabdominal infections (pyelo-, etc) IVDA Infected splenic hematoma Infected splenic infarctions (embolizations, ischemia, etc)

S/S: fever, WBC; 50% (+) blood cultures

Dx by CT scan + IV contrast Staphylococcus & Streptococcus E.coli, Salmonella, anaerobes Tx:Tx:

Splenectomy + IV Abx Percutaneous drainage

Splenic Salvage Procedures: Splenic Salvage Procedures: Therapeutic OptionsTherapeutic Options

SLR

September 12th, 2007

Nonoperative Management of Splenic Nonoperative Management of Splenic TraumaTrauma

Indications for initial nonoperative management hemodynamic stability absence of peritonitis CT scan

No contrast extravasation absence of other injuries

Transfusions - >2 PRBC’s

Protocol for Nonoperative Management

Grade I & IIGrade I & II Awake + alert, isolated injury

monitored observation BR, H/H q6h, serial abdominal exams Regular floor in 48º If remain stable and asymptomatic – D/C in 5 days F/U CT scan in 4 wks

Avoid prophylactic and therapeutic heparinization Grade III, IV, & VGrade III, IV, & V

Monitored observation x5 days Repeat CT scan Transfer to floor if stable F/U CT scan in 6-8 wks after discharge

SplenorrhaphySplenorrhaphy

Topical Hemostasis Small injuries (I & II)

Bovie electrocautery Argon beam Gelfoam Surgicel Avitene

Suture Repair & Partial Resection Segmental blood supply Monofilament sutures

Pledgeted horizontal mattress sutures

SplenorrhaphySplenorrhaphy

Mesh Splenorrhaphy Delaney (1982)

Autotransplantation controversial