The Spleen Lecture Conference Steven J. Binenbaum, MD SLR September 12 th, 2007

Splenectomy for Hematologic Diseases Rarely cures the disease Alleviates symptoms Corrects hematologic abnormalities Staging & Diagnosis

Splenectomy for Hematologic Diseases Red Cell-Related Indications 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 4 th year of life +/- cholecystectomy if (+) cholelithiasis

Splenectomy for Hematologic Diseases 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 Splenectomy Splenectomy (80% favorable clinical response) if: medical tx fails Intolerance to steroids or its side-effects

Splenectomy for Hematologic Diseases Pyruvate kinase Deficiency Autosomal-recessive disease Splenomegaly TOC Splenectomy Splenectomy Reduces PRBC’s requirements G6PD Deficiency Splenomegaly is rare Splenectomy is not indicated!

Splenectomy for Hematologic Diseases Sickle Cell Disease (SCD) B-globin gene A→T substitution (Hb S/SS) autosomal dominant Sickling of RBCs in tissues with low O 2 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 Diseases Thalassemia 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 Diseases Platelet Related Indications ITP 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/mm 3 #1 TOC is PO Steroids mg/kg/d (up to 70% response) IVIG 1 g/kg/d x2-3 days Splenectomy if fail steroid txTTP Splenomegaly Microvascular thrombosis Petechiae, fever, neurologic changes #1 TOC Plasmaphoresis Splenectomy is #2 Durable remission? Avoid PLT transfusions ↑ morbidity

White Cell - Related Indications Leukemia CLL & Hairy cell leukemia (HCL) Splenectomy improves cytopenias (75%) Ameliorates symptomatic splenomegaly Non-Hodgkin’s Lymphoma (NHL) Splenectomy Painful splenomegaly Cytopenia No role for staging

White Cell - Related Indications Hodgkin’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 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 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, meningococcus Pnemococcus, 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 Abscesses of the Spleen Steven J. Binenbaum, MD September 12 th, 2007 SLR

Malignant 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 Tumors Angiosarcoma Angiosarcoma Nonlymphoid malignant tumor of the spleen Early metastatic disease Aggressive with rapid growth Spontaneous splenic rupture and hemolytic anemia Palliation Benign Tumors Hemangioma Risk of rupture + platelet sequestration (Syndrome?) No tx unless symptomatic Hamartoma Lymphangioma

Splenic 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 granulosus Echinococcus granulosus Mostly asymptomatic Associated with liver cysts If only spleen is involved? Tx: Splenectomy

Splenic Cysts Splenic Pseudocysts Splenic 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 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: Therapeutic Options SLR September 12 th, 2007

Nonoperative Management of Splenic Trauma 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 & 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, & V Grade 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

Splenorrhaphy 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

Splenorrhaphy Mesh Splenorrhaphy Delaney (1982) Autotransplantation controversial