Haematology

Question 1

Idiopathic Thrombocytopenia (ITP)

Answer 1

• Usually 1-5 years
• Platelets <20x10⁹ with acute onset bruising/petechiae, epistaxis following recent viral illness
• No atypical features (absence of systemic symptoms)
• Bone marrow aspirate indicated in presence of atypical features or failure to spontaneously resolve
• Complications include intracranial haemorrhage
• Treatment
  
  • Careful observation and limit high impact activities
  • Platelet transfusion
  • IVIG
  • Consider steroids
  • Consider splenectomy

Question 2

Henoch Schonlein Purpura (HSP)

Answer 2

• Vasculitis - due to deposition of IgA containing immune complexes
• Purpura (extensor aspects of lowe limbs and buttocks), non-erosive arthritis (ankles, knees, elbows), colicky abdominal pain, N+V, blood and mucus on PR, haematuria, proteinuria and HTN
• No diagnostic test - deposition of IgA on biopsy strongly supportive
• Supportive management

Question 3

IDA

Answer 3

• Affects 10-30% of those at high risk (preterm, LBW, multiple births, exclusive breastfeeding >6 months, delayed weaning, excessive cow’s ilk, asolescent females, social deprivation, strict vegans)
• Also seen in malabsorption
• Listlessness, irritability, reduced cognitive and psychomotor performance
• Treatwith iron supplementation and iron rich diet

Question 4

Leukaemia

Answer 4

• ALL most common in children
• Bone marow failure (anaemia, low WCC, increased infections, low platelets - easy bruising/bleeding), bone pain/limp, lymphadenopathy, hepatosplenomegaly, testicular enlargement, cranial nerve palsies and meningism
• FBC/blood film/BM aspirate/LP/coag/U&Es/LDH/CXR for diagnosis
• Prognosis good but poor prognostic indicatior include:
  
  • Age <1 or >10 years
  • Male
  • T cell lineage
  • WCC at presentation >50
  • Chromosomal abnormalities
  • Failure to rapidly respond to induction chemotherapy
• Treatment involves multi-drug chemotherapy

Question 5

Haemophilia

Answer 5

• Haemophilia A = factor VIII deficiency
• Haemophilia B = Factor IX deficiency
• Spontaneous joint/muscle bleed or due to minor trauma
• Diagnosis from isolated prolonged APTT, specific factor deficiency
• Treat with recombinant factor administration IV
• Complications include progressive arthropathy, allergic reactions, risk of infections with IVs

Question 6

von Wilebrand Disease (vWD)

Answer 6

• Majority AD, some AR
• Deficiency/abnormality with Factor VIII:vWF - decreased platelet aggregation
• Diagnosis is prolonged APTT, reduced factor VIII:vWF levels
• Present with mucosal bleeding from GI, gums, epistaxis, menorrhagia
• Treat with DDAVP prophylaxis or recombinant factor VIII

Question 7

Lymphadenopathy

Answer 7

• Features suggestive of malignancy
  
  • Site - supraclavicular, epitrochlear
  • Associated systemic symptoms
  • Hepatosplenomegaly
  • Other palpable masses
  • Signs of bone marrow infiltration
• Malignant causes
  
  • AML/ALL
  • Lymphoma
  • Hodgkin’s disease
  • Neuroblastoma
  • Rhabdomyosarcoma
• Infectious causes
  
  • Bacterial lymphadenitis
  • Viral infection
  • Cat scratch disease
  • TB
  • Atypical mycobacterium
• Autoimmune causes
  
  • Kawasaki’s disease
  • JIA
  • SLE
  • Sarcoidosis
  • Drug reactions