Dr Fayyaz Hussain

 Spleen: A specialized abdominal organ involved in many functions

including erythrocyte clearance, innate immunity, and adaptive

immunity.

 Location: Left upper quadrant, below the diaphragm. Long axis parallel

to the tenth rib.

 Weight: 150 – 250g. Dry weight ~ 80 g.

 Size:
 8-13 cm
 >14cm is palpable.

 Number: Up to 10% of patients have an accessory spleen. Commonly

located along the course of splenic artery.

 Blood Supply:
 Splenic artery - branch of celiac trunk
 Splenic vein – tributary portal vein
 Splenic structure is designed for its two main functions:
 Filtration and quality control of red cells
 Immune response

 The structural correlates of these functions are the

 White pulp – concerned with immune responses
 Red pulp – concerned with red cell filtration

 At the junction of white and red pulps is the Marginal Zone, which has its
own pathologic significance.
 Follicles Central
B-Cells arteriole
Marginal Zone
B-Cells
CD5-, CD10-,
Periarteriolar CD23-
Lymphoid sheath
(PALS)
T-Lymphocytes

Red Pulp
Sinuses
Red cells
Macrophages
 Blood Pooling

 Quality control of Red cells

 Immune Responses
 Red cells:

 Normal red cell content in spleen 30 -70 ml (<5% of total red cell mass)

 Splenomegaly – expansion of splenic vascular beds and ↑ red cell pooling

 e.g In myelofibrosis, up to 40% of total red cells may be pooled.

 Splenomegaly due to cellular infiltration: (e.g. in CML) the pool is less

prominent

 Splenomegaly due to congestion: Increased red cell pool is dominant

feature
 Granulocytes:

 30-50% of total granulocyte pool.

 Splenic granulocytes are in dynamic equilibrium with circulating

granulocyte pool.
 Splenic sequestration of granulocytes is thought to be
responsible for the neutropenia that often occurs in patients
with splenomegaly.
Platelets:

 In normal subjects, 20–40% of the total platelet mass is pooled in the spleen
and the platelets spend up to one-third of their lifespan there.

 The pool increases when the spleen is enlarged.

 This pooling and temporary sequestration must be distinguished from the

destruction of platelets in the spleen that occurs in many cases of
thrombocytopenia.
 The spleen is the body’s largest filter of blood.

 Sequestration: Temporary entrapment of RBCs in reticular

meshwork of spleen, which are then released back into
circulation.
 Phagocytosis: Permanently damaged cells are phagocytosed by
macrophages.
 Contain 25% of the T-lymphocyte pool and 10–15% of the B-
lymphocyte pool
 Abundant macrophages and dendritic cells
 Both innate and adaptive responses
 The marginal zone acts as the site for both of these processes,
whereas the white pulp is restricted to adaptive immunity.
 The marginal-zone B cells are a unique B-cell subset that bridges the
innate and adaptive immune responses. They are able to recognize
bacterial pathogens without the help of T cells and this T-independent
antibody response consists largely of low-affinity IgM.
 The T-cell-independent antibody response provide a first
line of immune defense against bacterial sepsis, especially
from Streptococcus pneumoniae, Haemophilus influenzae
and Neisseria meningitides.
 The spleen also appears to act as a defense against viral
infections and intraerythrocyte parasitic infections such as
Plasmodium and Babesia.
 The spleen is an important site of haemopoiesis in utero and
retains the ability to re-activate this process after birth.

 The mechanisms involved in this extramedullary haemopoiesis

are poorly understood. It is not clear if pluripotent stem cells are
present in the spleen or migrate from the bone marrow.
 Clinically appreciable when spleen size > 14cm
 Methods of Spleen size assessment:
 Physical Exam:
 False negative:
 Minor enlargement
 Obese patients
 False positive:
 Wandering spleen – lax phrenico-colic ligament, or ↓ tone of abd wall
 Obstructive airway disease – flattening of diaphragm, pushing down on spleen
 Radiographic assessment more reliable - US Abdomen, CT
Scan, MRI

 PET-CT: For splenic lymphoma

 Gamma camera scintigraphy (using Technetium labelled

autologous red cells):
 Functional assessment of spleen
 Detection of accessory spleen
 A clinical syndrome characterized by:
1. Enlargement of the spleen
2. Reduction in one or more of the cell lines in the peripheral
blood and
3. Normal or hyperplastic cellularity of the bone marrow
 Occurs in a range of medical conditions.
 Conditions where hyposplenism is a frequent feature are:
 Sickle Cell disease
 Gluten induced enteropathy
 Untreated AIDS

 In sickle cell anemia there is functional asplenia by 1 year of age and auto-
infarction leads to a state of anatomical asplenia after 6–8 years of age.
 Patients with functional hyposplenism have:
 Impaired immunity to blood-borne bacterial and protozoal infections, and

 Persistent thrombocytosis

 Characteristirisic changes in blood count – occur when spleen atrophies to

less than 20% of normal size.

 Howell–Jolly bodies, siderotic granules and target cells.
 Acanthocytes may also be seen
 Increase in number of reticulocytes
 No alteration in red cell survival
 Rise in the total leucocyte count

 Usually the total white cell count stabilizes at between 10 and

15 × 109/L

 In response to infection, splenectomized subjects produce

a much greater leucocytosis than persons with intact spleens.

 The thrombocytosis after splenectomy is usually
transitory and falls to normal or near-normal values
over the following 1–2 months.
 Occasional large and bizarre platelets may be seen in
splenectomized patients.
 A fall in the IgM fraction of the serum immunoglobulins is
commonly found after splenectomy. IgG levels do not change,
while IgA and IgE increase.
 First splenectomy for an enlarged spleen by Quittenbaum in 1826.

 The patient died after six hours of surgery, with no apparent cause of death.

 Till 1952 splenectomy was considered a relatively hazard-less procedure, as

the function of spleen weren’t properly known.

 In 1952 King and Schumacher report cases of severe sepsis in

splenectomized infants.
 Hematologic Diseases (Hereditary spherocytosis, AIHA,

splenic marginal zone lymphoma etc.)

 Trauma
 Splenic rupture (Infectious mononucleosis, tropical
splenomegaly pregnancy)
 Splenic Rupture

 Splenic abscess

 Neoplasm:

 As part of radical surgical oncological clearance; e.g. locally advanced gastric

carcinoma

 Primary

 Splenic artery aneurysm

 Red Cell Disorders:

 Hemolytic Anemias: HS and Autoimmune > Enzymopathies

 Hemoglobinopathies

 Platelet disorders
 ITP

 Others:
 Splenic marginal zone lymphoma
 Myelofibrosis
 CLL (for AIHA)
 Gaucher’s disease
 Spleen is the major site both for synthesis of antiplatelet antibodies and
for destruction of antibody-coated platelets.
 Splenectomy produces the highest cure rates for ITP patients compared to all
other therapies.
 Approximately 85 percent of patients with persistent or chronic ITP respond
well to splenectomy, and 60 to 66 percent of the patients remain in remission
after 5 years
 Because ITP can remit spontaneously, splenectomy should be postponed at
least 12 months after diagnosis
 Warm Type: After steroids +- rituximab

 Cold Type: steroids and splenectomy are usually ineffective

 Splenectomy is not recommended in patients

with
 CVID

 Chronic infections such as chronic hepatitis and HIV,

 Known thrombophilia
 Vaccination:

 Against Pneumococcus, Meningococcus and Haemophilus influenzae

 At-least 2 weeks prior to splenectomy

 Patient who have received Rituximab in the last 6 months may have a sub-

optimal antibody response following vaccination.

 PPV:
 polysaccharide
 23 serotypes
 Poor efficacy in hypo splenic patients

 PCV:
 Conjugate vaccine
 7 or 13 serotypes
 Efficacy good but serotype coverage narrow

 Schedule:
 PPV-23 is used in many countries
 Some authorities suggest PCV followed 2 months later by PPV
 Quadrivalent vaccine (MenACWY)
 Evidence for the role of meningococcal vaccine in asplenic patients is less
convincing than for pneumococcal
 Six serotypes (a-f), but serotype b is the most virulent.
 Hib vaccine: conjugate vaccine
 Single dose currently recommended
 Annual influenza vaccine is also recommended for asplenic

or hypo splenic patients, primarily as influenza may predispose

patients to secondary bacterial infection.
 Bleeding

 Thrombocytosis:

 Up to 600-1000 x 109/L

 Peak at around 7-12 day

 Antiplatelet therapy as long as thrombocytosis is present

 Overwhelming post-splenectomy sepsis (OPSS)
 Can be fatal in up to 50%

 Strep pneumonia is the most common cause

 Haemophilus influenzae, Neisseria meningitidis may also

be implicated.

 With effective vaccination, newer organisms (esp gram-negative rods like E.Coli) are

increasingly recognized as a cause of post-splenectomy sepsis.

 Children younger than 6 months (esp <2 months) are at

increased risk of OPSS.

 That is splenectomy is relatively contra-indicated in this age

group
 In up to 10 % of patients
 More common in spleno-portal circulation: e.g Portal vein thrombosis
 Can cause DVT and pulmonary embolism as well.
 Risk is at least partially dependent on the underlying disease
 High in cases of enlarged spleen (MPNs, hereditary hemolytic anemia)
 Low in cases of ITP and trauma

 In the long run, increased risk of atherosclerosis leading to increased

cardiovascular events and vascular dementia have also been reported.
 Anti-platelet therapy:
 If platelet count > 800

 Thromboembolic prophylaxis: as per routine guidelines


 Antibiotic therapy:
 Prophylactic:
 Inconclusive evidence regarding routine antibiotic prophylaxis when
vaccination is available.
 British Committee for Standards in Hematology advises prophylactic
antibiotic therapy for high risk groups:
 <16 yrs or >50 yrs
 Inadequate response to pneumococcal vaccine
 Hx of previous invasive pneumcoccal infections
 Splenectomy in cases of hematologic malignancies
 Suitable agents: Penicillin V 250mg BD or Erythromycin 250mg BD
 Antibiotic therapy:

 Pre-emptive:

 Antibiotics should be given to patients if they develop fever and cant reach

hospital within 2 hrs.

 E.g Amoxillin 500mg or Levofloxacin 750mg

 Empiric therapy on hospital arrival:

 Ceftriaxone with or without Vancomycin

 Suspect accessory spleen if hematologic features of

hyposplenism are not present. E.g. Howell–Jolly bodies and

increased pitting (

 Radionuclide scan (or CT if not available) for accessory spleen