Spleen Introduction Functions Types Complications And Treatment Splenectomy Thalassaemia Notes

Spleen Introduction

Diseases of the spleen and causes of enlargement of the spleen are a great concern for physicians. Dozens of differential diagnoses of splenomegaly are being taught in medicine. However, a surgeon’s role in the treatment of an enlarged spleen is minimal.

Read And Learn More: Gastrointestinal Surgery Notes

However, it is he who does this ‘magic’ of removing the spleen within minutes and saves the life of a patient with a ruptured spleen. A surgeon plays a major role by treating hypersplenism with splenectomy. In this chapter, only topics related to the surgeon’s interest are dealt with. Other topics are mentioned briefly.

Describe the applied anatomy of the spleen.

The Spleen Is An Anatomically Small Organ, hidden underneath the 9th to 11th ribs (dull note on percussion) measuring 1 × 3 × 5 inches and weighing about 7 oz (80–300 g). It lies in intimate contact with the undersurface of the diaphragm.

This explains why a splenic abscess can rupture through the diaphragm causing empyema. When blood collects due to splenic injury, it irritates the diaphragm causing referred pain to the shoulder tip (kehr’s sign).

• The anterior border is notched and is in contact with the stomach. It is enclosed by the gastrocolic omentum. The surgical importance of this is that when traction is applied on the stomach during vagotomy, a splenic capsular tear can occur, resulting in bleeding.
• The two leaves of gastrocolic omentum pass backwards in front of the kidney forming the lienorenal ligament. This ligament is responsible for some part of the posterior fixation of the spleen. When this ligament is divided, the spleen can be brought to the surface of the wound during splenectomy.
• The inferior part of the hilum of the spleen is (just) in contact with the tail of the pancreas. Often, during an emergency splenectomy, the tail of the pancreas can get injured, resulting in a pancreatic fistula.

Spleen Splenic Parenchyma

• It is highly vascular.
• Splenic pulp has red pulp and white pulp.
• Red pulp is a honeycombed vascular space which is made up of cords of reticular cells and sinuses.
• White pulp consists of lymphatic tissue and lymphoid follicles containing lymphocytes, plasma cells and macrophages.

Spleen Blood Supply

• Arterial supply: The splenic artery is a branch of the celiac artery. At the hilum of the spleen, it divides into 4–5 trabecular arteries and then branches into central arteries. Central arteries pass through white pulp and give radial branches to periphery including distal red pulp. Anatomical knowledge of these branches helps when performing partial splenectomy for a ruptured spleen. (The blood flow rate through the spleen is about 300 ml/min.)
  • Spleen has 3–7 segments and each has its own vascular supply. The splenic artery divides into larger superior artery and smaller inferior artery. Lacerations of the spleen parallel to segmental vessels result in minimal haemorrhage. Lacerations perpendicular to the segments result in significant haemorrhage.
  • In children, splenic capsule is thick and more tolerant to damage. Hence majority of the children with blunt abdominal trauma to the spleen are managed by conservative/non-operative treatment.
• Venous drainage: Four to five branches of the vein join and form the splenic vein. This forms the portal vein by joining with the superior mesenteric vein behind the 2nd part of the duodenum.

Spleen Lymphatic Drainage: The lymph drains into hilar nodes, then into retropancreatic nodes and finally into celiac nodes. Since it has intimate contact with the fundus of the stomach and tail of the pancreas, the spleen also used to be removed (nowadays not done) along with the stomach and tail of the pancreas during radical gastrectomy and distal pancreatectomy.

Functions Of The Spleen

1. Destruction: The process of removal of abnormally aged, damaged red blood cells is called culling. Culling, the Greek word, implies sorting a collection of two groups: One that will be kept and the other that will be rejected. For example, spherocytes, in hereditary, spherocytosis are broken down in the spleen. This happens in red pulp.

2. Production: Production of lymphocytes, monocytes and plasma cells. Lymphopoiesis continues throughout life. Lymphocytes take part in the immune response of the body.

3. Antibody production: Spleen synthesises antibodies in its white pulp. It removes antibody-coated bacteria and antibody-coated blood cells via blood and lymph node circulation.

4. Immune response: White pulp is composed of Malpighian corpuscles. They are composed of B lymphocytes and T lymphocytes—which are in periarteriolar lymphoid sheaths (PALS). Thus white pulp plays a major role in active immune response through humoral and all mediated pathways.

5. Reservoir of platelets: It is a storehouse of platelets, which are released into circulation in an emergency situation. Thus when the spleen is enlarged, 80% of the platelets are sequestrated resulting in thrombocytopenia. Splenomegaly typically involves the expansion of red pulp.

6. Immune function: It is the major site for the synthesis of tuftsin, a peptide that stimulates the phagocytic activity of leucocytes. Opsonins are antibodies produced by the spleen against bacteria and fungi.

7. Removal: Heinz bodies, Howell-Jolly bodies, etc. are removed from RBCs. These cells are non-deformable intracellular substances.

8. Erythrocyte production: During foetal development, white and red blood cell production occur till the 5th month of gestation. In adults, in the case of myelofibrosis, erythropoiesis can occur.

Congenital Abnormalities

1. Accessory spleens: Splenunculi: They are found in about 10–20% of the population.

• They can be found near the hilum of the spleen, tail of the pancreas, splenic ligaments or in the mesocolon.
• In diseases of the spleen (haemolytic anaemia, thrombocytopenic purpura), all these accessory spleens have to be removed along with the spleen.

2. Splenic agenesis is rare.

3. Polysplenia is rare. It occurs due to the failure of splenic fusion.

4. Wandering spleen is due to loose ligaments. It is more vulnerable for torsion.

5. Hamartoma: Rare, small masses of 1 cm in diameter. It may be in lymphoid tissue also (Hamartoma = misfire).

Rupture Of The Spleen Causes

1. Blunt injury abdomen

• Injury to the left side of the chest, left lower rib fractures, due to fall from a tree, and road traffic accidents can be associated with splenic rupture.
• Retroperitoneal haemorrhage, fractured spine and renal injuries may be associated with splenic injury.

2. Penetrating injuries to the abdomen may cause a rupture of spleen.

3. Spontaneous rupture of the spleen is seen in malaria and infectious mononucleosis, rarely in sarcoidosis, haemolytic anaemia and leukaemia.

4. Iatrogenic: The splenic capsule may be torn during surgical procedures such as vagotomy or gastrectomy due to traction on the stomach.

Types Of  Spleen Injuries

Most of these injuries are diagnosed by CT scan today (CT classification is given).

1. Subcapsular haematoma: A capsular tear or parenchymal tear results in subcapsular haematoma.
2. Clean (incised wounds): This can happen in sharp injuries, Staal injuries (not common) or operative injuries.
3. Lacerated wounds: Often they are multiple as in blunt injuries abdomen.
4. Hilar injuries with pedicle avulsion are the most serious injuries. Need urgent laparotomy ligation of bleeders and splenectomy. Otherwise, death will occur.
5. Splenic injury with other organs. Tail of pancreas, diaphragm, left kidney, left colon.

Splenic Injury CT Scan Grading

Grade 1:

• Nonexpanding subcapsular haematoma <10% surface area.
• Nonbleeding capsular laceration with <2 cm deep parenchymal involvement.

Grade 2:

• Nonexpanding subcapsular haematoma 10–50% surface area.
• Nonexpanding intraparenchymal haematoma <2 cm diameter
• Bleeding capsular tear or parenchymal laceration 1–3 cm deep

Grade 3:

• Expanding subcapsular haematoma or intraparenchymal.
• Bleeding subcapsular. Haematoma or subcapsular haematoma >50% surface area
• Intraparenchymal haematoma >2 cm
• Parenchymal laceration >3 cm deep

Grade 4:

• Ruptured intraparenchymal haematoma with active bleeding.
• Laceration involving segmental or renal vessel >25% spleen volume devascularised.

Grade 5:

• Completely shattered or avulsed spleen.
• Hilar laceration which devascularises the entire spleen

Spleen Clinical Presentation

It can be divided into three groups:

1. Tearing Of Splenic Vessels: Avulsion Of Splenic Pedicle Can Result In Severe Haemorrhage And Shock. Death Can Occur Within A Few Minutes. Even In The Best Of The Situations, Death Cannot Be Prevented Often.
2. A slow-developing haemorrhagic shock followed by recovery. Examination reveals evidence of intraperitoneal bleeding. This can occur due to a capsular tear or injury to the splenic parenchyma. Its clinical features are as follows:
   • Anaemia—pallor
   • Pulse—tachycardia, more than 100/min
   • BP—low/hypotension
   • Cold, clammy extremities
   • Abdominal distension
   • Paralytic ileus develops slowly
   • Guarding and rigidity
   • Kehr’s sign is positive. Irritation of the undersurface of the diaphragm by the blood causes irritation of the phrenic nerve (C3, 4). Thus, this pain is referred to as the shoulder region (supraclavicular nerve C4). Kehr’s sign can be elicited by elevation of the foot end of bed for about 10 minutes.
   • Ballance’s sign: Blood in the vicinity of the spleen is fresh blood which is coagulated and blood in the periphery is not coagulated. Hence, there will not be shifting dullness on the left side of the abdomen but can be present on the right side.
   • Saegesser’s splenic point of tenderness: An area of tenderness on the left side between the sternomastoid and scalenus medius (rare sign).
3. Initial features of haemorrhagic shock, recovery and sudden haemorrhagic shock after a few hours to a few days. It is due to the following reasons:
   • Greater omentum seals off a tear which gets reopened after some time. The time period is called the “latent period of Randet”.
   • A subcapsular haematoma which had developed, ruptures after some time.
   • Associated injury to the tail of the pancreas causes release of enzymes which digest the tissues at a later date. This variety is called the “delayed type of shock” or delayed rupture of the spleen.

Complications Of Splenic Injuries

Haemorrhagic shock—if not treated—multiorgan failure results.

1. Disseminated intravascular coagulation due to massive bleeding and blood transfusion.
2. Injuries to the tail of pancreas—manifesting later as pancreatic ascites, pancreatic fistula or pseudocyst of pancreas.
3. Splenic artery pseudoaneurysm.
4. Splenic arteriovenous fistula.

Splenic Injuries Investigations

1. Hb% estimation, PCV (packed cell volume) estimation (however, in appropriate cases, it should be repeated at frequent intervals to detect continuing haemorrhage).
2. Four quadrant tap/aspiration by using a fine needle demonstrates blood either fresh or old. However, diagnostic peritoneal lavage (DPL) is more reliable (vide infra).
3. Emergency USG can reveal a splenic tear, or a subcapsular haematoma and can rule out other injuries. It is the most important investigation in suspected cases of splenic injury.
4. CT scan is more reliable. It should be used in cases of doubtful diagnosis and stable patients.1 It also rules out hollow viscus perforation, pancreatic injuries, etc.
5. Diagnostic peritoneal lavage (DPL): It is indicated in blunt injury abdomen where there are equivocal signs or doubtful signs of peritonitis.
   • Diagnostic peritoneal lavage Indications:
     • Unconscious patient with polytrauma with signs of abdominal injury.
     • Unexplained shock
     • Associated spinal cord injury
   • Diagnostic peritoneal lavage Procedure:
     • A 1 cm incision is made in the sub umbilical region under local anaesthesia (LA) after emptying the bladder. The peritoneum is opened and a 12 or 14 Fr peritoneal dialysis catheter is introduced into the peritoneal cavity.
     • The skin is closed and 1000 ml of normal saline is allowed to flow over 30 minutes.
     • The patient is turned to the right and left sides and fluid is allowed to flow out.
   • DPL is positive when:
     1. The fresh blood of more than 20 ml is removed immediately after inserting the dialysis catheter and it does not clot.
     2. RBC count 1,00,000/mm³ or WBC >500/mm³.
     3. Amylase level of over 175 units/dl.
     4. Bile, food or any other fresh contents.
     5. Gram stain is positive in the contents of lavage.
   • Diagnostic accuracy is around 95%. DPL is useful to rule out intestinal injury in addition to intraperitoneal bleeding.
6. Plain X-ray abdomen should not be done in an emergency situation when patients are in shock. Findings of splenic trauma in plain X-ray abdomen are given here.
   • The splenic outline may not be seen clearly.
   • Fundic air bubbles may be indented by the haematoma.
   • Psoas shadow is obliterated (KUB X-ray).
   • Evidence of fracture of lower ribs.
   • Evidence of fluid in the peritoneal cavity—‘ground glass’ appearance
   • Elevation of left dome of the diaphragm.

Spleen Treatment

Treatment—4 Headings

1. Emergency splenectomy – Massive bleeding and unstable
2. Splenorrhaphy – Stable
3. Partial splenectomy – Stable
4. Nonoperative – Stable patient

1. Emergency Splenectomy: Haemodynamic instability is the main indication for emergency splenectomy. Blood pressure less than 90 mmHg and heart rate more than 130 per minute is a clear indication for surgery. When there is active bleeding with hypotension due to large lacerations, emergency splenectomy is the treatment of choice since it is quick, easy to perform and can be life-saving.

The splenic artery is ligated first at the upper border of the pancreas followed by the splenic vein. In desperate situations, the spleen is mobilised by incising the lienorenal ligament, a large arterial clamp is applied at the splenic hilum and splenectomy is done.

2. Partial Splenectomy

• It can be done because the splenic artery gives an upper polar branch and a lower polar branch which again divides into 2 branches. Hence, when one of the branches of the splenic artery is ligated, the bleeding stops and a partial splenectomy can be done comfortably.
• After splenectomy, the spleen can be cut into multiple pieces and can be implanted within the greater omentum. The spleen survives because of neovascularisation and functions like a spleen in the production of antibodies. This can be done in children.

3. Splenorrhaphy: It is a method of preservation of the spleen when the general condition of the patient is reasonably good. In this, a small tear is sutured by using chromic catgut and can be wrapped using greater omentum.

4. Nonoperative or Conservative Treatment: It is safe in selected adult and paediatric patients with isolated splenic injuries. The small risk of delayed rupture should be explained to the patient.

• Patients are admitted in the intensive care unit. Serial monitoring of haemoglobin and total WBC counts are done. Abdominal girth and blood pressure are monitored. If there is any doubt of hypotension, exploration is done.
• While managing the following patients, utmost care should be given with the non-operative treatment
  1. Older patients above the age of 50
  2. Grade 3 and 4 injury
  3. More than 500 ml of blood detected by the CT scan
  4. Other solid organ injury (liver)
  5. Systemic vascular abnormalities, such as pseudoaneurysm or AV fistula, etc.

Early Complications Of Splenectomy

1. Pancreatic injury (0–6%): Treated by drain placement and measuring drain amylase. Usually, it is a minor discharge and generally settles over a few days.

2. Vascular injury (2–3%): Slippage of ligature (splenic artery), hilar dissection, capsular tear during retraction. Needs to be identified and sutured coagulated or clamped.

3. Injury to the bowel: Colon, stomach. These can be avoided with careful dissection techniques. During ligation of short gastric arteries too close to the stomach side, injury to the stomach can occur. It is repaired in two layers. Colonic injury can occur during dissection to separate the lower pole. It is also repaired in a similar manner.

4. Diaphragmatic injury: This can happen when adhesions are present between the spleen and diaphragm due to perisplenitis or recurrent infarctions. Simple suturing is enough if recognised.

5. Splenosis: This refers to multiple small implants of splenic tissue on the peritoneal surface following a traumatic rupture of the spleen. They can give rise to adhesions.

6. Pulmonary: These are common complications. Generally they go unnoticed. It can range from minor cough to atelectasis to pneumonia and pleural effusion. Basal atelectasis is one of the common causes of postoperative fever.

7. Paralytic ileus: Treated by drip and suction.

8. Haematemesis can occur due to congestion of the gastric mucosa as a result of ligation of the short gastric vessels.

9. Subphrenic abscess: Not common. If it occurs, percutaneous drainage and antibiotics.

10. Wound problems: Haematomas, seromas and wound infection.

11. Thrombocytosis and thrombotic complications: Sometimes it can be dangerous. They can present as abdominal pain and vomiting which is confused for incision pain, gastritis, etc. Ultrasound examination is a must. One of our patients had portal vein thrombosis which was managed by anticoagulants. Platelet count after splenectomy was 8 lakh/mm3.

Indications For Splenectomy

Indications for splenectomy Always indicated

1. Primary splenic tumour
2. Hereditary spherocytosis

Indications for splenectomy Usually indicated

1. Primary hypersplenism
2. Chronic ITP
3. Splenic vein thrombosis
4. Splenic abscess

Indications for splenectomy Sometimes indicated

1. Splenic injury
2. Autoimmune haemolytic disease
3. Elliptocytosis haemolysis
4. Nonspherocytic congenital haemolytic anaemia
5. Hodgkin’s disease with anaemia
6. Thrombotic thrombocytopenic purpura
7. Idiopathic myelofibrosis
8. Splenic artery aneurysm
9. Wiskott-Aldrich syndrome
   • X-linked disorder predominantly presents as features of thrombocytopenia such as epistaxis, bloody diarrhoea, and petechiae in young age
   • Combined ‘B’ and ‘T’ cell immunodeficiency causes eczema
   • More incidence of malignancies
   • Spleen destroys platelets count as low—20,000 or 40,000 cells/microlitre
   • The best treatment is human leukocytic antigen (HLA) matched sibling bone marrow transplantation
   • Otherwise—splenectomy and antibiotics are the treatment of choice
10. Gaucher’s disease

Indications for splenectomy Rarely indicated

1. Chronic leukaemia
2. Thalassaemia major
3. Sickle cell anaemia
4. Felty’s syndrome
5. Hairy cell leukaemia

Haematological Indications For Splenectomy

Idiopathic Thrombocytopenic Purpura (ITP)

• This condition occurs due to the development of autoantibodies against the patient’s own platelets.
• Normal levels of platelets are between 1,50,000 and 4,00,000 cells per mm³.
• The spleen is probably responsible for the sequestration of platelets and for the production of antibodies.

Idiopathic Thrombocytopenic Purpura Types

1. Type 1: Acute—children: Follows an acute infection and resolves spontaneously in about two months.
2. Type 2: Chronic—adults: Longer than six months. No cause is identified.

Idiopathic Thrombocytopenic Purpura Other Causes

• ↓ Production: Cytotoxic drugs, aplastic anaemia.
• ↑ Consumption of platelets: DIC as in septicaemia.
• ↑ Destruction: SLE, infectious mononucleosis.
• ↑ Capillary fragility: Steroid-induced or HenochSchönlein purpura.

Idiopathic Thrombocytopenic Purpura Clinical Features

• Condition is common in females, F: M:: 3: 1.
• Purpuric patches (ecchymosis refers to skin discolouration due to extravasation of blood) are found on the buttocks and petechial haemorrhages (spots) are found on the limbs. These are dependent areas having high intravascular pressure.
• All types of haemorrhages are common and can be mild or moderate.
• Intracranial haemorrhage is found in 1–2% of patients but it may be the cause of death.
• In 25% of cases, the spleen is just palpable (small size).
• Hess tourniquet test: More than 20 petechiae in a circle of 3 cm diameter in the cubital fossa suggests purpura.

Idiopathic Thrombocytopenic Purpura Common Types of Haemorrhages

• Epistaxis
• Menorrhagia
• Haematemesis
• Bleeding gums
• Haemarthrosis
• Haematuria

Idiopathic Thrombocytopenic Purpura Investigations

• Bleeding time is prolonged in purpura. Clotting and prothrombin times are normal.
• Platelet count is reduced.
• Bone marrow biopsy: Precursors of platelets (megakaryocytes) are increased.

Idiopathic Thrombocytopenic Purpura Treatment

• In Children
  • Spontaneous regression occurs in the majority of cases.
  • Short course of corticosteroids is beneficial. Tablet prednisolone in the dose of 10 mg/day over a period of 6 weeks is given.
• In Adults: Splenectomy is indicated in the following situations:
  • When ITP has presented for more than 6–9 months.
  • When ITP has relapsed in spite of steroids, the patient is given a trial of prednisolone, 1 mg/kg/day. Platelet count rises within 7 days of starting steroids after which splenectomy can be done.
  • Also indicated, if a patient has two relapses on steroid therapy or if the platelet count is less.

Idiopathic Thrombocytopenic Purpura Results: 70–80% of patients respond permanently and do not require any further treatment. Counts rise above 1,00,000/mm3 in 7 days. Even if counts are not raised, recurrent bleeding rarely occurs.

Idiopathic Thrombocytopenic Purpura Surgery

• Can be done even when platelets are as low as 10,000 cells/mm³.
• Diathermy is used to open layers of the abdomen.
• Ligate the splenic artery first at the upper border of the pancreas after opening the lesser sac.
• Splenectomy is not difficult. Accessory spleen (15–30%), if present, should be removed.
• Platelet transfusion may be required postoperatively if there is bleeding.
• Two-thirds of patients will be cured by surgery.
• 15% will show improvement.
• The remaining will not benefit.

Idiopathic Thrombocytopenic Purpura Haemolytic Anaemia: This type of anaemia results from increased red cell destruction which occurs in the spleen. The lifespan of red blood cells is also shortened. Hence, irrespective of the cause of haemolytic anaemia, these cases can benefit from splenectomy. Anaemia, jaundice and splenomegaly are the triads of haemolytic anaemia.

• ITP Causes of Haemolytic Anaemia
  • Hereditary spherocytosis
  • Acquired autoimmune haemolytic anaemia
  • Thalassaemia
  • Sickle cell disease

Hereditary Spherocytosis

This hereditary disorder is transmitted by either parent as Mendelian autosomal dominant. The disease is characterised by a defect in cell wall protein of the red cell, namely spectrin, which increases the reflux of sodium into the cell. This causes the biconcave red cell to swell and become spherical. Hence, the name spherocytosis.

Consequences of Spherocytes in Circulation

1. The spherocyte is already weak. Adding onto this, there is a greater loss of membrane phospholipid which results in a delicate fragile spherocyte.
2. Because of the altered shape, oxygen and energy requirements of RBCs increase which cannot be met by the spleen. In the spleen, they are destroyed and release excessive haemoglobin which is converted into bilirubin.
   • The amount of bilirubin produced is increased and most of it is unconjugated. It gets attached to albumin as it is lipid soluble and, therefore, cannot be excreted in the urine. Hence, this type of jaundice is also known as acholuric jaundice.

Hereditary Spherocytosis Clinical Features

• Commonly seen in young children but can manifest in adults too.
• Seen equally in both sexes.
• A history of recurrent attacks of jaundice can be elicited from childhood.
• Jaundice is mild, never deep, and not associated with itching or bradycardia.
• Pallor is an important feature due to the destruction of the red cells.
• Acute haemolytic crisis: It is precipitated by an acute infection or stress. Abdominal pain, nausea, vomiting, pyrexia and pallor are the features. The condition can be confused for an abdominal emergency. In severe cases, anaemia, thrombocytopenia, leukopenia can occur.
• The spleen is moderately enlarged. The liver is also palpable in a few cases.
• In adults, gallstone colic and obstructive jaundice due to CBD stone can also complicate the disease process. The incidence of gallstone disease is around 50%. It is common after the age of 10.
• Leg ulcers may occur as a result of anaemia.

Hereditary Spherocytosis Investigations

1. Peripheral smear: Spherocytes are present.
2. Reticulocyte count is increased (15–25%): These are immature red cells which are discharged by the bone marrow due to loss of red cells.
3. Coombs’ test is negative: Serum bilirubin is mildly elevated and most of it is unconjugated.
4. Fragility test: Normal red cell haemolysis occurs in 0.47% saline solution. Here, it occurs at 0.6% or even in a weaker solution.
5. Ultrasound abdomen is done to rule out gallstones.

Hereditary Spherocytosis Treatment: Splenectomy is a treatment including the removal of accessory spleen.

• Ideally, it should be done from the age of 6 to 10 years. Why after 6 years? The incidence of post-splenectomy sepsis is more when splenectomy is done within 6 years of age. Why before 10 years? After 10 years of age, the incidence of gallstones increases.
• Splenectomy helps in the following ways
  1. By decreasing erythrocyte destruction, anaemia improves, thus avoiding blood transfusions.
  2. Erythrocytes achieve a normal lifespan. Hence, jaundice also disappears.
  3. Leg ulcers heal quickly.
     • If gallstones are also found in these cases, laparoscopic splenectomy and cholecystectomy can be done in the same sitting.

Acquired Autoimmune Haemolytic Anaemia

Due to an autoimmune reaction, the red cell surface is damaged and it gets destroyed in the splenic red pulp. Antibodies develop against red cell antigens. In 50% of cases, the cause is not known. The following are the probable causes:

• SLE, lymphoma, chronic lymphocytic leukaemia
• Drugs: Methyldopa, mefenamic acid
• Exposure to chemicals or infections

Acquired Autoimmune Haemolytic Anaemia Clinical Features

• More common in females after 50 years of age.
• Splenomegaly, moderate in size.
• Gallstones are found in around 25% of the cases.

Acquired Autoimmune Haemolytic Anaemia Investigations

• Anaemia is present.
• Spherocytes may be present because of damage to the red cell surface.
• Coombs’ test is positive.

Acquired Autoimmune Haemolytic Anaemia Treatment

• It is a self-limiting disorder. Reassurance plays an important role.
• Corticosteroids: A short course of prednisolone 60 mg/day is given.
• Splenectomy is done when steroid response is not satisfactory. 80% of patients respond to splenectomy.

Thalassaemia

It is also called Cooley’s anaemia, Mediterranean anaemia, and Heinz body haemolytic anaemia.

Thalassaemia Aetiopathogenesis

• It is a hereditary disorder, transmitted as a dominant trait. It is characterised by a defect in haemoglobin peptide chain synthesis. Depending on the type of peptide chain involvement, it is classified into α, β and γ. However, β-thalassaemia is more common. There is a decrease in haemoglobin and red cells will be destroyed prematurely due to intracellular precipitates (Heinz bodies).
• When it is a heterozygous disorder, it is called beta-thalassaemia minor and when homozygous, it is called beta-thalassaemia major (Cooley’s anaemia). The abnormal gene is inherited from either or both parents.

Thalassaemia Clinical Features

• Severe anaemia resulting in weakness, lethargy, leg ulcers, etc.
• Splenomegaly is mild to moderate in size. Sometimes, it is massive resulting in abdominal discomfort or may cause abdominal distension.
• Jaundice due to haemolysis. The liver can also be enlarged.
• To compensate for anaemia, bone marrow hyperplasia occurs resulting in bossing of the skull and prominent malar bones. Thalassaemia major usually manifests in the first year of life.

Thalassaemia Complications

• Haemosiderosis (deposition of iron in tissues) of the pancreas results in chronic pancreatitis and diabetes.
• Hepatic cirrhosis due to liver haemosiderosis.
• Aplastic crisis with severe life-threatening infections.
• Gallstones occur in about 20% of cases.

Thalassaemia Investigations

• Peripheral smear demonstrates microcytic hypochromic anaemia. Red cells are small, thin and are resistant to osmotic lysis.
• Haemoglobin electrophoresis reveals reduction or absent levels of haemoglobin A (HbA). There is a compensatory increase in foetal haemoglobin (HbF).

Thalassaemia Treatment

• Blood transfusion: Multiple repeated transfusions may be necessary.
• Surgical: Splenectomy is indicated in a few cases that require multiple transfusions and patients with gross splenomegaly.

Sickle Cell Anaemia Or Sickle Cell Disease

This is a hereditary haemolytic disorder in which haemoglobin A is replaced by haemoglobin S (HbS). This results in crescent-shaped erythrocytes. This HbS molecule crystallises if there is hypoxia, strenuous work or due to dehydration. As a result of this, red cells are distorted and elongated. Therefore, blood viscosity is increased, causing obstruction in the splenic circulation.

Functional Abnormalities in Sickle Cell Anaemia

• The spleen sometimes acts as a large reservoir for red cells and the sickle cells are destroyed.
• Antibody production is decreased.
• Spleen’s ability to filter Streptococcus pneumoniae is reduced.

Effect of HbS Blockage

• Splenic microinfarcts
• Splenomegaly or auto splenectomy due to repeated infarcts.

Sickle Cell Anaemia Clinical Features 

• The disease is very common in Africans. Most of the patients in India have high HbF levels which protect HbS. Hence, symptoms are not seen in the first few weeks.
• Abdominal pain is due to recurrent infarcts or gallstones in a few patients.
• Skin ulcers occur due to anaemia or hypoxia.

Sickle Cell Anaemia Investigations

• Peripheral smear demonstrates sickle cell.
• Haemoglobin electrophoresis to identify the type of haemoglobin.

Sickle Cell Anaemia Treatment

• Folic acid supplements
• Splenectomy has a doubtful value. It is indicated only when excessive numbers of RBCs are sequestrated causing anaemia.

Sickle Cell Anaemia Complications

• Infarct of cerebrum resulting in hemiplegia.
• Mesenteric infarction resulting in gangrene of the bowel.
• Pulmonary infarction resulting in chest pain.

Splenectomy For Other Conditions Hypersplenism

It is defined as pancytopenia in the presence of normal or hypercellular bone marrow. The following are the causes of hypersplenism: It usually involves red pulp expansion. It must be remembered that in cirrhosis of the liver, 15% of patients develop hypersplenism. Anaemia and thrombocytopenia are mild. Rarely requires splenectomy (a few causes are given below):

• Tropical splenomegaly due to malaria, kala-azar or schistosomiasis, etc. Since these diseases are endemic in tropical countries, it is called tropical splenomegaly. Gross enlargement of the spleen and hypersplenism are indications for splenectomy in such cases.
• Myeloproliferative disorders: Splenectomy reduces the need for transfusion and may relieve abdominal discomfort.
• Portal hypertension: Maximum benefit is in segmental portal hypertension with oesophagogastric varices.
• Genetic: Gaucher’s diseases.
• Neoplastic infiltration.
• Inflammatory disease: Sarcoidosis, lupus erythematosus.
  • Chronic infections, such as tuberculosis, brucellosis and malaria.
  • Chronic haemolytic diseases—spherocytosis, thalassaemia, glucose-6-phosphate dehydrogenase deficiency, elliptocytosis.

Cysts Of The Spleen

Parasitic cyst is rare. Typically, it is caused by echinococcal disease (hydatid disease).

• Traumatic cysts are due to a haematoma giving rise to liquefaction. These are false cysts of the spleen.
• Congenital cysts can be due to a dermoid cyst, haemangioma or lymphangioma.
• Better save the spleen by deroofing the cyst wall.

Tumours Of The Spleen Or Neoplasms

Among neoplasms, lymphoma is the most common cause of enlargement of spleen. Spleen used to be removed as a part of a staging laparotomy. Now, it is very rarely removed. Patients with large spleen of chronic myeloid leukaemia, Gaucher’s disease and hairy cell leukaemia (details later) will also benefit from splenectomy.

• Fibrosarcoma and angiosarcoma are rare malignant tumours.
• Haemangioma is the most common benign tumour of the spleen. It may turn into haemangiosarcoma.
• Spleen is removed in oesophagogastrectomy or upper carcinoma stomach—only when it is directly infiltrated. Sometimes it happens in the carcinoma tail of the pancreas/splenic carcinoma.

Other Indications For Splenectomy

A splenic abscess can occur due to infected septic emboli from otitis, typhoid fever or due to thrombosis of the splenic vein causing infarction followed later by infection. Tubercular abscess of the spleen is rare. It is usually affected secondary to abdominal or pulmonary tuberculosis.

Felty’s syndrome refers to splenomegaly, neutropenia with rheumatoid arthritis. After splenectomy, leg ulcers heal quickly and neutropenia also improves. The patient may respond to steroids better and the incidence of recurrent infection becomes less.

Splenic Artery Aneurysm

It is an uncommon condition. More common in women, and some of them occur during pregnancy.

Causes of Splenic Abscess

• Salmonella infection
• Paratyphi infection
• Labour—puerperal sepsis
• Ear infection—otitis
• Necrosis of pancreas
• Infection of bone—osteomyelitis
• Chronic disease—tuberculosis

Remember as SPLENIC

Splenic Artery Causes

• Atherosclerosis: Commonly seen in elderly patients.
  
• Congenital: Young patients. These patients may present for the first time during pregnancy with enlargement or rupture.
  
• Acute pancreatitis: Inflammatory process may give rise to pseudoaneurysm especially because of pancreatic necrosis.
  

Splenic Artery Clinical Features

• Pain and vomiting—haematemesis
• Thrill or bruit
• Calcification in a routine X-ray
• Rupture and features of haemorrhagic shock.

Splenic Artery Investigations

• Ultrasound/CT scan
• Angiography

Splenic Artery Treatment

• Non-operative: Embolisation of splenic artery
  
• Operative: Ligation of the splenic artery at the upper border of the pancreas after opening lesser sac followed by splenectomy.
  
• Rupture: Carries a high mortality rate.

Hairy Cell Leukaemia (HCL)

The cells on a blood smear have an irregular outline due to the presence of filament-like cytoplasmic projections. Hence, the name hairy cells. HCL is a clonal proliferation of abnormal B cells (very rarely T cells).

Hairy Cell Leukaemia Clinical Features

• Anaemia
• Recurrent infection
• Massive enlargement of spleen.

Hairy Cell Leukaemia Investigations

• Blood counts are low. White cell count may be raised with circulating hairy cells.
• Bone marrow has increased cellularity with characteristic infiltration by hairy cells.

Hairy Cell Leukaemia Treatment

• Drug: 2-Chloradenosine acetate (2-CDA) has been beneficial. It can induce remission.
• Splenectomy is indicated in cases of diagnostic difficulties and in very large spleens.

Spleen Multiple Choice Question And Answers

Question 1. In splenic injury, Kehr’s sign refers to:

1. Absence of shifting dullness on the left side of the abdomen
2. Tenderness on the left side between the sternocleidomastoid and scalenus medius muscle
3. Tender left iliac fossa
4. Shoulder pain due to irritation of the under-surface of the diaphragm

Answer: 4. Shoulder pain due to irritation of the under-surface of the diaphragm

Question 2. Spontaneous rupture of the spleen is seen in the following except:

1. Idiopathic thrombocytopenic purpura
2. Malaria
3. Infectious mononucleosis
4. Leukaemia

Answer: 1. Idiopathic thrombocytopenic purpura

Question 3. Diagnostic peritoneal lavage is said to be positive in all of the following except:

• Fresh blood >20 ml is aspirated after inserting the dialysis catheter
• Gram stain is positive in the contents of the lavage
• RBC count >100,000 cells/cu mm
• Amylase level of 75 units/dl

Answer: 4. Amylase level of 75 units/dl

Question 4. The following is not true about nonoperative treatment in splenic trauma:

1. Hemodynamically stable patient
2. Hollow viscus injury
3. Close monitoring is available
4. Hospitalisation is required

Answer: 2. Hollow viscus injury

Question 5. Big spleen is not a feature of:

1. Malaria
2. Idiopathic thrombocytopenic purpura
3. Portal hypertension
4. Leukaemia

Answer: 2. Idiopathic thrombocytopenic purpura

Question 6. The following is true about purpura:

1. Bleeding time is prolonged
2. Clotting time is prolonged
3. Prothrombin time is prolonged
4. Activated partial thromboplastin time is prolonged

Answer: 1. Bleeding time is prolonged

Question 7. Which of the following vaccinations has to be given after splenectomy to prevent overwhelming post-splenectomy infection?

1. Measles vaccine
2. Pneumococcal vaccine
3. Hepatitis vaccine
4. Varicella vaccine

Answer: 2. Pneumococcal vaccine

Question 8. The following is true about splenectomy in idiopathic thrombocytopenic purpura (ITP):

1. Is required in all cases of ITP
2. Must be done when the platelet count is >100,000 cells/cu mm
3. Accessory spleens, if present must be left behind
4. Two-thirds of patients will be cured by surgery

Answer: 4. Two-thirds of patients will be cured by surgery

Question 9. The following is true about splenectomy for hereditary spherocytosis:

1. Splenectomy should be done as a last resort
2. Splenectomy must be done either before 6 years or after ten years of age
3. Accessory spleen, if present must be left behind
4. Jaundice disappears after splenectomy

Answer: 4. Jaundice disappears after splenectomy

Question 10. The most common benign tumour of the spleen is:

1. Haemangioma
2. Lymphoma
3. Tuberculoma
4. Lipoma

Answer: 1. Haemangioma