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PACES Abdominal Station > Splenomegaly > Flashcards

Splenomegaly Flashcards

1
Q

What are the causes of massive (>8cm) splenomegaly? (7, in 3 categories)

These can also be the causes of moderate and mild before they become massive

A
  1. Myeloproliferative
    - Chronic Myeloid Leukaemia
    - Myelofibrosis
    - Polycythemia
  2. Tropical infection
    - Chronic Malaria
    - Visceral leischmaniasis (kala-azar)
  3. Splenic marginal zone lymphoma
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2
Q

What are the causes of moderate (4-8cm) splenomegaly? (5)

A
  1. Gaucher’s
  2. Amyloid
  3. Lymphoma - enlarged lymph nodes
  4. Leukaemia
  5. Felty’s - signs of RA
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3
Q

What are the causes of mild (<4cm) splenomegaly? (3)

A
  1. Portal hypertension - look for signs of CLD
  2. Infections e.g. EBV, IE, Hepatitis - look for splinter haemorrhages, murmur
  3. Haemolytic anaemia
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4
Q

What are the investigations for splenomegaly? (7)

A
  1. Ultrasound abdomen
  2. Haematological causes:
    - FBC
    - Blood film
    - Bone marrow aspirate and trephine
    - Lymph node biopsy
  3. Infections
    - Thick and thin films for malaria
    - Viral serology
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5
Q

What are the indications for splenectomy? (4)

A
  1. Haemorrhage/rupture (trauma)
  2. Signficiant transfusion requirements in haemlytic disorders
  3. Signficiant syptoms
  4. Leucopenia or thrombocytopenia due to hypersplenism
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6
Q

What are the complications of splenectomy? (2)

A
  1. Thrombocytosis
  2. Overwhelming sepsis from encapsulated orfanism
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7
Q

How are splenectomy patients managed to prevent complication? (3)

A
  1. Vaccination against encapsulated bacteria
    - Pneumococcal
    - Meningococcus
    - H. Influenzae
  2. Prophylactic lifelong penicllin
  3. Medical alert bracelet
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8
Q

What are the clinical features of splenomegaly?

A
  1. Signs of anaemia
  2. Lymphadenopathy
  3. Purpura
  4. Left upper quadrant mass - estimate size
  5. Check for hepatomegaly
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9
Q

What is the genetic cause for hereditary spherocytosis?

A

Autosomal Dominant defect of 1 of 5 possible genes, usually chromosome 8

Codes for RBC membrane proteins causing sphere shaped red blood cells which are more prone to haemolysis

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10
Q

What are the clinical features of hereditary spherocytosis? (3)

A
  1. Anaemia (lethargy)
  2. Jaundice
  3. Splenomegaly
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11
Q

What are the complications of hereditary spherocytosis? (3)

A
  1. Anaemia
  2. Gallstones
  3. Aplastic crisis due to infection
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12
Q

What investigations should be done in hereditary spherocytosis? (7)

A
  1. FBC
    - Hb
    - MCH
    - Reticulocyte count
  2. Blood film
    - Haemolysis
    - Spherocytes
  3. LDH (raised)
  4. Haptoglobins (low)
  5. Unconjugated bilirubin
  6. EMA binding test
  7. Osmotic fragility test
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13
Q

What is the management for hereditary spherocytosis?

A
  1. Folic acid
  2. Transfusions for anaemia
  3. Splenectomy - eleminates haemolysis
    - Vaccines and prophylactic antibiotics
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14
Q

What are the causes of hepatosplenomegaly?

A
  1. Myeloproliferative
  2. Lymphoproliferative
  3. Cirrhosis with portal HTN
  4. CLL
  5. Lymphoma
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15
Q

What are the other (than splenomegaly) features of polycythemia rubra vera?

A
  1. Facial plethora
  2. Dusky cyanosis of the extremities
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16
Q

What is polycythemia?

A

Over activity of bone marrow - raised red cell count, keeps making red cells until the iron runs out so also has an iron deficiency picture

JAK2 mutation

17
Q

What are the investigations for polycythemia?

A
  1. FBC
  2. Iron studies
  3. JAK2 mutation genetic testing
18
Q

What are the causes of secondary polycythemia?

A
  1. Chronic hypoxia e.g. COPD, Pickwickian
  2. Smokers
  3. Cobalt poisoning
  4. Inappropriate EPO e.g. certain cancers - renal

PACES Abdominal Station (11 decks)

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