SPLEENOMEGALY &

HYPERSPLENISM
ETIOLOGY PATHOGENESIS AND
SURGICAL MANAGEMENT
By
Dr Aravind
 Spleen is the largest lymphoid organ of
the body
It plays important role in Red blood cells
sequestration and immunity
Store house of platelets
Produces RBC and WBC in fetus during
gestation period and some times in
adults
Anatomy of Spleen
Develops from
mesenchymal cells
in the dorsal
mesogastrium
during the fifth
week of gestation.
 Located between
9th and 11th ribs on
left side
It is about 14cms
in length and 7 7cm

cms in breadth
Weighs 150
-200gms 14 cm

Accessory spleens
called splenunculi
Ligaments
Gastrosplenic
ligament
Lienorenal ligament

Lineophrenic

ligament
Splenocolic ligament
Blood supply
Artery
Splenic artery
Short gastric
arteries
Veins
Splenic vein
 Red pulp(90%)
Cords and sinuses
Phagocytosis
Open circulation
White pulp
Periarticular
lymphatic sheets
Immunoglobulins
Functions of spleen
Cellular Immunological
Pitting Synthesis of Ig M
Culling Lymphocytes
Storage of Tuftsin, opsonin,
platelet properdin,
Phagocytosis interferon
Iron reutilisation
Hyperspleenism
Hypersplenism is a condition in which the
spleen becomes increasingly active and
then rapidly removes the blood cells
Splenomegaly,
Pancytopenia or a reduction in the number
of one or more types of blood cells
Maturation arrest
decreased red blood cells survival
decreased platelet survival.
Spleenomegaly
Normally spleen not palpable
Size 2 to 3 times the size spleen is
palpable
Weight 400-500 gms
Spleen size is not a reliable indicator of
spleen function
Clinical features
Mass in left hypochondrium
Notch felt
Moves with respiration
Dull on percussion
Directed toward Rt iliac fossa
Hook sign
Can not insinuate fingers under Lt costal
margin
Causes
Based on pathological mechanism
divided
Increased function
Abnormal blood flow
Infiltration
Increased function
Removal of defective RBCs
spherocytosis
thalassemia
hemoglobinopathies
nutritional anemias
early sickle cell anemia
 Immune hyperplasia
Response to infection (viral, bacterial, fungal, parasitic)
mononucleosis, AIDS, viral hepatitis
subacute bacteria endocarditis, bacterial septicemia
splenic abscess, typhoid fever
brucellosis, leptospirosis, tuberculosis
histoplasmosis
malaria, leishmaniasis, trypanosomiasis
ehrlichiosis
Disordered immunoregulation
rheumatoid arthritis
Systemic lupus erythematosus
serum sickness
autoimmune hemolytic anemia
sarcoidosis
drug reactions
Extramedullary hematopoiesis
myelofibrosis

marrow infiltration by tumors, leukemias

marrow damage by radiation, toxins

Abnormal blood flow
Organ Failure
cirrhosis
Vascular
hepatic vein obstruction
portal vein obstruction
BuddChiari syndrome
splenic vein obstruction
Infections
hepatic schistosomiasis
hepatic echinococcosis
Infiltration
Metabolic diseases
Gauchers disease
NiemannPick disease
alpha-mannosidosis
Hurler syndrome and other
mucopolysaccharidoses
amyloidosis
Tangier disease
 Benign and malignant infiltrations
leukemias (acute, chronic, lymphoid, and myeloid)
lymphomas (Hodgkins and non-Hodgkins)
myeloproliferative disease
metastatic tumors (commonly melanoma)
histiocytosis X
hemangioma, lymphangioma
splenic cysts
hamartomas
eosinophilic granuloma
littoral cell angioma
Causes of massive spleenomegaly

visceral leishmaniasis (kala-azar)

chronic myelogenous leukemia
myelofibrosis
malaria
primary lymphoma of spleen
Hereditary spherocytosis

Autosomal dominant inheritance

Most common congenital hemolytic anemia

Red cell membrane lacks the necessary protein assembly.

(spectrin &ankyrin)
decrease cellular plasticity with membrane loss

RBCs small,dense, deformed

hemolysis(in the spleen )

Clinical features
hemolytic anemia,

splenomegaly allmost always

jaundice .

Periodic exacerbation (follow viral infections )

Pigmented gall stones,CBD stones, Cholangitis

 Investigations
Fragility test - increased
Increased serum bilurubin
Increase dreticulocyte count
Increased feca lurobilonogen
Pheripheral smear spherocytes
Ultrasound of abdomen
Treatment
Splenectomy is the sole treatment

Associated gall stones -

Cholecystectomy
Splenectomy should be delayed in

children till they reach 7 years

Auto Immune Hemolytic anaemia

Production of IgG and IgM autoantibodies specific

for cell membrane proteins on erythrocytes
Classified to
Common warm antibodies (40-50% of cases )

Due to Ig G antibodies

Associated with CLL

Less Common antibodies

Due to Ig M antibodies

The hemolysis occur intravascularly & not in

within the spleen

Clinical features
Usually after 50 years

female to male 2;1

acute onset

Anemia,

Jaundice

Splenomegaly in 50% in patients

gall stones in 25%

 Treatment
Corticosteroids produce remission in
75%
Splenectomy is indicated in warm
antibodies anemia who fail to
respond to 4-6 weeks of high dose
corticosteroids
Thalassamias
Thalassemia major (mediterranean
anemia,Coolys Anemia )
Dominant autosomal inheritance
Deficit in synthesis of peptide chain .(alpha, beta,
gamma)leading to decrese in Hb-A
Manifest at first year of life
Failure to thrive
Severe chronic anemia
Large head, splenomegaly
Leg ulcers
Succeptiblity to infection
Pigment gall stones 25% of patients
 Investigations
Electrophoresis low Hb-A
Persistance of Hb-F (fetal)
Treatment
Iron chelation
Blood Transfusion
Splenectomy may reduce the need for
transfusion

Thassemia minor - most patients are

asymptomatic ,may have mild anemia
Sickle Cell anemia
Replacement of normal hemoglobin A
(Hb-A) by sickle hemoglobin Hb-S
Crescent shaped RBC more prone for
trapping in spleen
Spleenic micro infracts are common
Initially splenomegaly and latter auto
spleenectomy
 Clinical features
Anemia
Pain abdomen
Leg ulcers
Cerebral pulmonary and mesenteric infracts
Diagnosis by electrophoresis
Treatment
Sodium cyanate
Partial exchange transfusion
Antibiotics
Spleenectomy has limeted role
Idiopathic Thrombocytopenic purpura
(ITP)

Results from destruction of platelets by

circulating IgG antiplatelets factors
originating from spleen
Common in females
Acute common in children
Spontaneous remission
Platelets below 50000/cc cause bleeding
Regular follow up
 Clinical fetures
Purpuric patches over skin and mucus
membrane
Epistaxis
Heamaturia Hemarthrosis
GIT bleeding
Intracranial bleed
Hess test
 Investigation
Bleeding time increased
Clotting and prothrombin time normal
Platelet count decreased
Bone marrow increased megakaryocytes
Anemia and neutropenia not present
Spleenomegaly
 Treatment
Methly prednislone IV for 3-5 days
Oral prednisolone 6-12 weeks
IV immuniglobin 0.4- 1 gm/kg for 5 days
Vincristin 2 mg/week 6 weeks
Danazol 200mg tid
Anti RhD antibodies
Azathiprine
Splenectomy
FFP, platelets and whole blood transfusions
 Indications for Splenectomy in ITP
Relapse
Girls reaching menarche
Refractory to treatment
Pregnancy with bleeding problems
Thrombotic thrombocytopenic purpura
(TTP)

Arterioles and capillaries of micro

circulation involved
Anemia
Thrombocytopenia
Altered mental functions neurological
deficits
Plasmaphoresis
spleenectomy
Feltys syndrome
Rheumatiod arthritis
Mild lekopenia
Spleenomegaly
Spleenectomy Indications
Absolute

Bleeding varices due to splenic vein

thrombosis
Hereditary spherocytosis
Massive splenic trauma
Primary splenic malignancy
 Relative
Autoimmune hemolytic anemia
Hypersplenism due to portal HTN
Idiopathic thrombocytopenic purpura (ITP)
Leukemia (chronic myloid leukemia )
Lymphoma
Primary hypersplenism
Myelofibrosis
Sickle-cell disease
Splenic abscess
Staging for hodgkins lymphoma
Thalassemia
Thrombotic thrombocytopenic purpura
Radical gasterctomy involving removal of spleen
Preoperative preparation
Blood grouping and typing
Cross matched blood
Platelets should not be administered
preoperatively in patient with idiopathic
thrombocytopenic purpura
In myeloproliferative disorders administer low-
dose heparin, 5000 units twice daily, and aspirin
on the day before surgery and to continue this
regimen for 5 days postoperatively
Vaccines against Streptococcus pneumonia,
Haemophilus influenzae type B, and Neisseria
meningitides are administered 14 days before
operation
Types of surgery
Open
Laparoscopic
Thoracoabdominal approach
(Abandoned)
Open Spleenectomy
Anesthesia General
Position - Supine
Incision Left sub costal
Midline in case of trauma
 Mobilization of the
spleen to the
midline by division
of the lateral and
superior pole
attachments
The splenocolic and
splenorenal
ligaments at the
lower pole are
divided
 short gastric
vessels are
divided between
ligatures
Splenic vessels
are isolated
 Tail of pancreas is
displaced
medially to avoid
injury
Splenic hilum is
held enboc
between three
clamps and
divided
 Search for
accesory spleens
should be done in
elective cases
Complications for open spleenectomy

Bleeding
Left lower lobe atelectasis
Subphrenic abcess
Thrombosis of the splenic vein
Injury to the tail of the pancreas
Laproscopic Spleenectomy
Most of cases
laproscopic
speenectomy can
be done
Patient Rt
decubitus position
 First abdomen searched for accessory
spleens
All Ligaments examined first
gastrosplenic ligament is opened to see
the tail of pancreas
Splenocolic ligament is divided spleen
retracted
Short gastric vessels divided tail of
pancres and vascular bundle are
visualised
Splenic vessels divided by various
 Spleen is held by
lineophrenic
ligament only
A nylon bag is used
as retrieval bag
Brought near
epigastric or
supraumblical port
and its open spleen
morcellated
removed piece meal
Complications
Injury to Diaphragm which is rare in
open spleenectomy
Common complications of both methods

Postsplenectomy sepsis
(increasesd incidence of pneumonia ,
septicemia,meningitis )

Overwhelming post splenctomy

sepsis(0,8 % in adult,high in children)

Thrombocytosis

Splenosis
Post Splenectomy care
Immunisation
 Antibiotic prophylaxis
Controversial
To prevent OPSI
Pencillins given for two years after
spleenectomy in children