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Splenomegally

Section: Hepatobiliary Sub-section: Spleen Curriculum: Curriculum, page 92

Definition

Increased spleen size

Aetiology

Congestive - Pooled blood

Haematological - Increase in activity and subsequent hyperplasia

Autoimmune

Neoplastic - Invasion by cells foreign to splenic environment

Infectious

Enzyme Deficiencies

Other

Haematological

Hereditary Spherocytosis

  • Most common congenital haemolytic anaemia

Cause

  • Variants in genes that encode proteins of the red blood cell (RBC) membrane skeleton
    • Most common Spectrin (SPTA1 and SPTB genes)
    • Get spherical, rigid, fragile RBC,
  • Autosomal Dominant susceptibility in 75%
    • Triggered by viral illness
  • Clinical Presentation
    • Anaemia, jaundice, splenomegaly
  • Investigation
    • Coombs negative (not autoimmune, negative antibodies),
    • Get spherocytes & reticulocytes on blood film
  • Management
    • Splenectomy, should delay if possible till >6yrs age to allow immune system to mature
    • High incidence of gallstones due to haemolysis, consider cholecystectomy if present

Sickle cell anaemia

  • Autosomal recessive
  • Homozygous inheritance of HbS
    • Single amino acid substitution of valine for glutamic acid in 6th position of B-chain of HbA.
  • When oxygen tension low (deoxygenated);
    • HbS crystallises
      • cell becomes crescent
      • trapped
      • microinfarction.
  • Children get splenomegaly
  • Acute manifestations mainly relate to:
    • infections (due to functional asplenia)
    • Aneamia
    • Vaso-occultion
  • Treatment
    • Hydroxyurea
      • increases production of fetal hemoglobin that then interferes with the hemoglobin polymerisation as well as by reducing white blood cells that contribute to the general inflammatory state in sickle cell patients.
    • Transfusions
    • Splenectomy vaccinations
    • Indications for surgery include hypersplenism, sequestration crisis which lead to requirements for blood transfusion, development of splenic abscess, splenic infarction, pain.

Thalassaemia

  • Genetic abnormality (AD) leading to a quantative deficiency of either a or b chains of HbA (a2b2)
  • Normally
    • Two b-globin genes (one on each Chromosome 11)
    • Four a-globin genes (two on each chromosome)
  • b-thalassemia
  • a-thalassemia
  • Treatment
    • Mainstay of treatment is transfusion and chelation therapy
    • Stem cell transplant is playing greater role
    • Splenectomy rarely required

Acute Splenic Sequestration

  • Life threatening complication of haemoglobinopathies
    • Get hypersplenism
    • Can result in severe pain & anaemia.
  • Characterised by acute Hb drop
    • RBCs captured in spleen
    • Tends to occur in infants with Homozygous HbS, or beta thalassemia
  • 10-15% mortality rate
    • Fast, often die before transfusions can be given
  • Management – transfusion
  • Splenectomy to treat and prevent recurrence

Autoimmune

Immune thrombocytopenic purpura

  • Acquired thrombocytopenia caused by auto-antibodies against platelet antigens
    • Platelets destroyed by spleen secondary to IgG
    • Often triggered by viral URTI
  • Note different childhood variant
  • More severe, M=F
    • But 80% spontaneously resolve

Investigations

  • Low platelets, normal marrow function
  • Note spleen usually normal size
  • DDx
    • pre-eclampsia, drugs (heparin), viral, chronic liver disease, autoimmune, chronic DIC, lymphoproliferative

Management

  • initial management
  • Based on platelet count and symptoms
    • PLT > 50, asymptomatic
      • No Rx, bleeding unlikely even with surgery
    • PLT 30-50, asymptomatic
      • Follow
    • PLT <30, or PLT <50 and symptomatic
      • Prednisone 1mg/kg/d 1-3 weeks
    • PLT <20
      • Admit, IVIG 1g/kg/d for 2 days
      • ± transfuse platelets
      • IV dexamethasone
    • If critical bleeding
      • IVIG
      • Platelet transfusion
      • IV steroids
    • Splenectomy is the recommended second line treatment
      • Note spontaneous remission is possible, so usually attempt to delay splenectomy for 6-12 months to see if this will occur
  • First line
    • steroids
  • Second line
    • Rituximab
    • thrombopoietin receptor agonist (TPO-RA)
    • IV IG Operative Considerations in ITP
  • Indications
    • Refractory severe thrombocytopenia
    • Needing toxic doses of steroids for remission
    • Relapse
    • Pregnant and failing conservative measures
  • 80-90% curative
    • Predictors of success
      • Young, Platelets 150 d3, >500 d10
    • Most who will respond do so in 10 days
  • If failure to respond
    • Look for accessory spleen
    • Especially if absence of asplenic RBC morphology on film
    • Can do RBC scintigraphy (Tc99 TBC scan or Tc99 sulfur colloid scan
  • Pre-operative considerations
    • Immunisations
    • Optimise platelet counts - IVIG, steroids
      • Ideally plat count >50 but can still be done with platelets on standby

Thrombotic thrombocytopenic purpura

  • Acquired (or hereditary) condition
  • Caused by severely reduced activity of ADAM TS13
    • ADAMST13 is VW factor-cleaving protease
    • Platelets cannot be cleaved, so therefore clump
  • Characterised by small-vessel platelet rich thrombi, get
    • Thrombocytopaenia,
    • Microangiopathic haemolytic anaemia,
    • Organ damage
    • Disease fatal without prompt treatment
  • Mainstay is plasmaphoresis
  • Splenectomy previously used before plasmaphoresis

Autoimmune haemolytic anaemia

  • If caused by IgG may respond to splenectomy
  • Approx 50% of cases respond
  • Consider if
    • First line management to improve the haemoglobin fail to prevent haemolysis
    • Ongoing need for high dose steroids
    • Note IgM haemolytic anaemias are not splenic driven
      • Will not respond to splenectomy

Felty syndrome

  • Severe subset of seropositive rheumatoid arthritis complicated by granulocytopenia and splenomegaly
  • Splenectomy is indicated
    • Granulocytopenia and nonhealing leg ulcers
    • Recurrent or severe infections (pneumonia or septicemia)
    • Despite nonpharmacologic efforts to reduce risk of infection and use of medical therapies, including the conventional synthetic, biologic, and targeted synthetic disease-modifying antirheumatic drugs (DMARDs) and granulocyte colony-stimulating factor (G-CSF).
    • Patients with recurrent or severe infections usually have severe granulocytopenia (<1000 cells/microL)

Enzyme Deficiencies

Glucose-6-Phosphate Dehydrogenase Deficiency

  • Most common inherited red blood cell (RBC) enzyme abnormality
    • X-linked, Africa, middle-east, Mediterranean
    • Abnormal glucose metabolism = haemolysis
    • Usually after an antecedent stress
      • Medications, food (Fava beans), chemicals
  • Rarely warrants splenectomy

Pyruvate Kinase Deficiency

  • Autosomal recessive hemolytic anemia characterized by reduced activity of one of the two red blood cell (RBC) isoforms of the PK enzyme, which is encoded by the PKLR gene
    • Decreased RBC deformability
  • Splenectomy if transfusion-dependent anemia
    • Ideally, deferred until later childhood and preceded by indicated vaccinations
  • Cholecystectomy may be required for pigment gallstones.
  • Iron chelation may be needed to prevent or treat iron overload

Neoplastic

Lymphoma

Hodgkin’s Lymphoma

  • Get Reed-Sternberg cells – bi-lobed nucleus (owls eye)
  • Mostly young adults with asymptomatic lymphadenopathy
  • Splenectomy
    • Uncommon now, used to have a role in staging

Non-Hodgkin’s Lymphoma

  • Splenectomy occasionally for abdominal pain, pancytopenia
  • Spleen involved in 50-80%
    • <1% have disease confined to spleen and may benefit from splenectomy (ie: primary lymphoma)

Leukaemia

  • Splenectomy done if refractory to medical therapy
    • CLL & CML
      • Splenectomy for hypersplenism
      • If symptomatic or medically resistant

Myeloid Disease

  • Extra-medullary haematopoiesis
    • Spleen reactivated
  • Most of these diseases medically treated
  • Myelofibrosis
    • Marrow infiltration
    • Marrow damage
  • Generally only needed for massive splenectomy at the end stage of disease
    • But this adds risk to surgery
    • Bleeding, CHF etc

Primary splenic tumours

  • Rare
  • Benign
    • Haemangioma
      • Rare, usually < 2cm, often incidental
      • Excision indicated if symptomatic, or >5cm
    • Harmatoma
      • White or red pulp, nonencapsulated 2-4cm
    • Lymphangioma
      • Rare, slow growing
  • Malignant
    • Primary lymphoma
      • Usually non-Hodgkin’s
    • Angiosarcoma
      • Rare, grey nodules, early metastases, poor prognosis
  • Splenectomy for diagnosis, Rx or palliation

Metastatic

  • 7% of all cancer patients get splenic mets
  • Breast, lung, melanoma, ovary, choriocarcinoma
  • Splenectomy for palliation

Infectious

Splenic abscess

  • Uncommon
  • Unilocular (66%),
    • Usually healthy patients
    • MR 15-20%
  • Multilocular,
    • Immunocompromised
    • MR 80%
  • Risk factors: Cancer, polycythaemia, endocarditis, trauma, hemoglobinopathy, UTI, IVDU, AIDs
    • 70% hematogenous spread
      • Endocarditis, osteomyelitis, IVDU
    • Can also have direct spread
      • Colon, kidney, pancreas
  • Microorganisms
    • Staph, strep, enterococcus, mycobacterium, Actinomyces, candida
  • Management
    • Single– drain + Abx – success 75-90%
    • Multiple - splenectomy

Hydatid cysts

  • Hydatid cyst (Echinococcus)
    • Do serology
  • Spillage = anaphylaxis, need splenectomy
    • Sterilise with
      • Hypertonic saline
      • Alcohol or
      • Silver nitrate
  • Calcification on USS
  • Eosinophilia on bloods, + serology

Other

Splenic cysts

  • True cysts 10%
    • Asymptomatic unless >8cm or rupture, bleed or get infected. CA19-9 often elevated
    • Simple - Often multiple & peripheral, lined with cuboidal cells
    • Dermoid – rare – keratinising epithelium with skin appendages
    • Epidermoid – rare – squamous lining
  • Pseudocyst 70-80%
    • Due to trauma/ infarction
  • Parasitic
  • Management
    • <5cm & asymptomatic
      • Observe, unless suspicious feature (irregular wall) – follow with USS
    • Symptomatic or >5cm
      • Laparoscopic de-roofing/ marsupilise
        • Diathermy or harmonic & pack with omentum
        • Can recur
      • Splenectomy
      • Percutaneous drainage usually not recommended
        • High recurrence rate (even if inject alcohol)

Wandering spleen

  • Failure of formation of peritoneal attachments
    • Failure of fusion of dorsal mesograstrium
  • Can be aquired, causing long splenic pedicle
    • ED hormonal changes in pregnancy
  • Most are asymptomatic, but can cause splenic torsion
  • Management
    • Splenopexy
      • Procedure done by wrapping vicryl mesh around spleen and then tacking to abdominal wall
    • Splenectomy

Spontaneous splenic rupture

  • Rare
    • Malaria = commonest cause
    • Infectious mononucleosis – 2nd –
      • most frequent in 2nd - 4th week of disease
    • Other
      • Candidiasis (often associated with abscess)
      • Sarcoid
      • AML/CLL
      • Congestive splenomegaly
      • Polycythaemia

Isolated Splenic Vein Thrombosis

  • SV thrombosis without thrombosis of portal vein
  • Primary
    • Hypercoaguable/ thrombotic state
  • Secondary
  • Clinical
    • May present as UGIB due to isolated gastric varices (bypass)
  • Ix – CT, EUS, MRI
  • Mx
    • Most asymptomatic & require no Rx – Rx thrombotic condition
    • Splenectomy for bleeding varices

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