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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.

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the spleen

The Spleen

Lecture Conference

Steven J. Binenbaum, MD

SLR

September 12th, 2007

splenectomy for hematologic diseases
Splenectomy for Hematologic Diseases
  • Rarely cures the disease
  • Alleviates symptoms
  • Corrects hematologic abnormalities
  • Staging & Diagnosis
splenectomy for hematologic diseases3
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 4th year of life
    • +/- cholecystectomy if (+) cholelithiasis
splenectomy for hematologic diseases4
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
    • PRBC’s for severe anemia
    • Splenectomy (80% favorable clinical response) if:
      • medical tx fails
      • Intolerance to steroids or its side-effects
splenectomy for hematologic diseases5
Splenectomy for Hematologic Diseases

Pyruvate kinase Deficiency

  • Autosomal-recessive disease
  • Splenomegaly
  • TOC
    • Splenectomy
      • Reduces PRBC’s requirements

G6PD Deficiency

  • Splenomegaly is rare
  • Splenectomy is not indicated!
splenectomy for hematologic diseases6
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 O2 tension
  • Red & White pulps
  • Splenic microinfarcts
    • Painful
    • Abscess
    • Infections
    • Anemia
  • Splenectomy (palliative)
    • Acute sequestration crises (recurrence = 40%-50% with 20% Mortality rate)
    • Rapid hypersplenism
    • Abscess formation
splenectomy for hematologic diseases7
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/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

TTP

Splenomegaly

Microvascular thrombosis

Petechiae, fever, neurologic changes

#1 TOC

Plasmaphoresis

Splenectomy is #2

Durable remission?

Avoid PLT transfusions

↑ morbidity

Splenectomy for Hematologic DiseasesPlatelet Related Indications
white cell related indications
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 indications10
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
Bone Marrow – Related Indications
  • Myelofibrosis & Myeloproliferative disorders
    • Splenectomy for symptoms due to enlarged spleen
splenectomy complications
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 complications13
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
    • 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

Tumors, Cysts, and Abscesses of the Spleen

Steven J. Binenbaum, MD

September 12th, 2007

SLR

malignant tumors
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 tumors21
Malignant Tumors
  • 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

Mostly asymptomatic

Associated with liver cysts

If only spleen is involved?

Tx: Splenectomy

Splenic Cysts
splenic cysts23
Splenic Cysts
  • 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
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:
    • Splenectomy + IV Abx
    • Percutaneous drainage
nonoperative management of splenic trauma
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
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
    • Monitored observation x5 days
    • Repeat CT scan
    • Transfer to floor if stable
    • F/U CT scan in 6-8 wks after discharge
splenorrhaphy
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
splenorrhaphy31
Splenorrhaphy
  • Mesh Splenorrhaphy
    • Delaney (1982)
  • Autotransplantation
    • controversial