Transcript The Spleen - St. Luke's Roosevelt Hospital Center

The Spleen
Lecture Conference
Steven J. Binenbaum, MD
SLR
September 12th, 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 4th 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
PRBC’s for severe anemia
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

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 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 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
TTP

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



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





Pnemococcus, H.influenza, meningococcus




Children < 15 yrs old
Immunosupression
Hematologic dz (thalassemia, SCD, etc)
Highest within the first 2 yrs post splenectomy
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 12th, 2007
SLR
Malignant Tumors


Spleen - mostly secondary involvement
non-Hodgkin’s Lymphoma – most common malignancy


Spleen is the primary site



Main Tx: Chemo +/- RT
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





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 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


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;


Erode into adjacent structures
50% (+) blood cultures
Dx by CT scan + IV contrast
Staphylococcus & Streptococcus
E.coli, Salmonella, anaerobes
Tx:


Splenectomy + IV Abx
Percutaneous drainage
Splenic Salvage Procedures:
Therapeutic Options
SLR
September 12th, 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






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