SPLEEN

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SPLEEN

• Hashmi

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INTRODUCTION

• has variously been described as the seat of
  laughter, the source of black bile in the body,
  and the locus of conflicting emotions.
• "spleen" in English is "ill temper.

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• The spleen is the organ most often damaged in
  cases of abdominal trauma.
• Any injury to the spleen can cause severe
  internal hemorrhage and shock.
• If surgically removed, the liver and red bone
  marrow can take over the functions normally
  carried out by the spleen.

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• During periods of high infection, the spleen may
  become enlarged, increasing its ability to become
  injured.
• Infectious mononeucleosis is the most likely
  cause of spleen enlargement.

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ANATOMY OF THE SPLEEN

• The human spleen is a dark bean shaped organ
  about the size of a persons fist.
• weighing approximately 5-6 Ib.
• located in the upper left quadrant, just beneath
  the diaphragm and posterior to the stomach.
• The shape and structure of the spleen is similar
  to a lymph node, but much larger. This makes the
  spleen the largest lymphatic organ in the body.

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• The spleen is protected by the rib cage
  anteriorly, laterally, and posteriorly.
• Physically the spleen is in contact with other
  vital organs, specifically the left diaphragm
  superiorly, the pancreatic tail medially, the
  stomach anteromedially, the left kidney and
  adrenal gland posteromedially, as well as the
  flexure of the colon inferiorly

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• To keep its position in the upper left quadrant,
  the spleen is secured by four suspensory
  ligaments
• 1-the gastrosplenic, which connects the stomach
  to the spleen.
• 2-the splenorenal, which connects the spleen to
  the kidney.
• 3-the splenocolic, which connects the spleen to
  the colic flexure and to the thoracic diaphragm.
• 4-the splenophrenic, which connects the spleen to
  the diaphragm.

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• The spleen is surrounded by a connective tissue
  capsule, called trabeculae, which extends inward
  to divide the organ into lobules (compartments).
• Two types of tissue are found in the spleen which
  correspond to its two most important functional
  roles. This tissue is called white pulp and red
  pulp.

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• The white pulp is lymphatic tissue consisting
  mainly of B T-lymphocytes around the arteries
  with the purpose of immunity and phagocytic
  action.
• The red pulp consists of venous sinuses filled
  with blood and cords of lymphatic cells, such as
  lymphocytes and macrophages, which is the primary
  site for mechanical filtration of the blood and
  the removal of senescent red blood cells.

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• At any given time, 1 unit or 450mL of blood is
  contained in the spleen.
• In emergencies, such as a hemorrhage, smooth
  muscle in the vessel walls and in the capsule of
  the spleen contracts due to sympathetic
  stimulation, and squeezes 200mL of reserved
  blood out of the organ into general circulation.

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SPLEEN FUNCTION

• The primary filtering element for the blood.
  Acts as a filter against foreign organisms that
  infect the blood stream.
• The site of red blood cell and platelet storage.
• Filters out old red blood cells and recycles
  them.

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EVALUATION OF SIZE AND FUNCTION

• Ultrasound (US) is the most cost-effective mode
  of splenic imaging for routine elective
  splenectomy. The sensitivity of US for detecting
  textural lesions of the spleen is as high as 98
  in experienced hands.
• (CT) scanning affords a high degree of resolution
  and detail of the spleen as well.

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SPLENECTOMY

• INDICATIONS
• 1-Red cell disorders
• A. Congenital
• Hereditary spherocytosis
• Hemoglobinopathies (SCD,Thalassemia,Enzyme
  deficiencies)
• B. Acquired
• Autoimmune hemolytic anemia
• Parasitic diseases

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•  2.   Platelet disorders
• a.   Idiopathic thrombocytopenic purpura (ITP)
• b.   Thrombotic thrombocytopenic purpura (TTP)
•  3.   White cell disorders
• a.   Leukemias
• b.   Lymphomas
• c.   Hodgkin's disease

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•   4.   Bone marrow disorders (myeloproliferative
  disorders)
• a.Myelofibrosis (myeloid metaplasia)
• b.Chronic myeloid leukemia (CML)
• c.Acute myeloid leukemia (AML)
• d.Chronic myelomonocytic leukemia (CMML)
• e.Essential thrombocythemia
• f.Polycythemia vera

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• 5. Miscellaneous disorders and lesions
• a.  Infections/abscess
• b.   Storage diseases/infiltrative disorders
• i. Gaucher's disease
• ii. Nieman-pick disease
• iii. Amyloidosis
• c.   Felty syndrome's
• d.  Sarcoidosis
• e.  Cysts and tumors
• f.   Portal Hypertension
• g.  Splenic artery aneurysm

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• 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

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• 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

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• 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 PRBCs requirements (gt200 ml/kg/year)
• ? rate of infections after splenectomy
• Risk vs. Benefit

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• Pyruvate kinase Deficiency
• Autosomal-recessive disease
• Splenomegaly
• TOC
• Splenectomy
• Reduces PRBCs requirements
• G6PD Deficiency
• Splenomegaly is rare
• Splenectomy is not indicated!

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• 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
• PRBCs for severe anemia
• Splenectomy (80 favorable clinical response) if
• medical tx fails
• Intolerance to steroids or its side-effects

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Platelet Related Indications

• ITP
• Spleen is not enlarged
• ? gt ?
• children vs. adults
• Self-limited in children (70)
• Splenectomy is for rare cases
• Insidious onset in adults
• ? Plts lt 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

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• White cell disorders
• Leukemia
• CLL Hairy cell leukemia (HCL)
• Splenectomy
• improves cytopenias (75)
• Ameliorates symptomatic splenomegaly
• Non-Hodgkins Lymphoma (NHL)
• Splenectomy
• Painful splenomegaly
• Cytopenia
• No role for staging

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• Hodgkins 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

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• Myelofibrosis Myeloproliferative disorders
• Splenectomy for symptoms due to enlarged spleen

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SPLENECTOMY OUTCOMES

• COMPLICATIONS
• LLL atelectasis, pneumonia, effusion
• Hemorrhage (mostly with laparoscopic)
• Intraabdominal abscess (LUQ)
• Pancreatitis or fistula formation
• DVT
• PVT
• -myeloproliferative dz with splenomegaly
• OPSI

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• HEMATOLOGICAL OUTCOMES
• appearance of Howell-Jolly bodies and
  siderocytes.
• Leukocytosis and increased platelet counts
  commonly occur following splenectomy as well.
• For thrombocytopenia, the initial response is
  typically defined as a rise in platelet count
  within several days of splenectomy. A long-term
  response is defined as a platelet count greater
  than 150,000/mL more than 2 months after surgery
  without medications.
• For chronic hemolytic anemias, a rise in
  hemoglobin levels to above 10 g/dL without the
  need for transfusion signifies a successful
  response to splenectomy.

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• Overwhelming Postsplenectomy Infection (OPSI)
• Incidence 1 (up to 5) 50 Mortality
• Encapsulated gram-positive bacteria
  Streptococcus
• Risk factors
• Children lt 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

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TUMORS

• Spleen - mostly secondary involvement
• non-Hodgkins Lymphoma most common malignancy
• Main Tx Chemo /- RT
• Spleen is the primary site
• 10 Hodgkins disease
• 30 of resected spleens (staging procedure) have
  () histology
• Hairy cell leukemia
• Resect for symptomatic splenomegaly
• Improved survival
• CML CLL
• symptomatic splenomegaly splenectomy

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

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• Benign Tumors
• Hemangioma
• Risk of rupture platelet sequestration
• No tx unless symptomatic
• Hamartoma
• Lymphangioma