8. haematological malignancies

Question 1

classification of heamatological malignancies

Answer 1

1. Myeloid

• myeloproliferative disorders
• myelodysplasia

2. Lymphoid

• Lymphomas
• immunosecretory disorders

Question 2

types of myeloid disorders

Answer 2

• Acute Myeloid Leukaemia (AML)
• Chronic Myeloid Leukaemia (CML)
• Myeloproliferative Disorders (MPD)
  
  • Essential Thrombocythaemia (ET)
  • Polycythaemia Rubra Vera (PRV)
  • Myelofibrosis (MF)
  • Idiopathic Hypereosinophilic Syndrome (IHES)
  • Systemic Mastocytosis (SM)
• Myelodysplastic Syndromes

Question 3

types of lymphoid disorders

Answer 3

• Acute Lymphoblastic Leukaemia (ALL)
• Chronic Lymphocytic Leukaemia (CLL)
• Plasma Cell Disorders e.g. Myeloma
• Non Hodgkins Lymphomas e.g.
  
  • Hodgkins Disease
  • Diffuse Large B NHL (DLCL)
  • Follicular Lymphoma (FL)
  • Mantle Cell Lymphoma (MCL)
  • Marginal Zone Lymphoma (MZL)
  • Burkitts Lymphoma (BL)
• T cell lymphomas

Question 4

acute vs chronic myeloid leukaemias

Answer 4

acute

• very rapid cell growth
• bone marrow failure (anemia, neutropenia)
• high WBC not always present
• bone marrow may fill before spilling out
• immediate chemo

chronic

• slow growth
• no bone marrow failure
• high WBC
• splenomegaly, praipism
• due to Ph chromosome
• protein specific inhibition

Question 5

acute vs chronic lymphoblastic leukaemia

Answer 5

cancer of immature lymphocytes

acute

• Common in children
• Present with cytopenia (mature blood cells) or chest mass
• Higher relapse in older children and boys
• 85% cures, 90% into remission with induction chemotherapy
• Strong chemo and milder tablets (prevent relapse)

chronic:

• cancer of B lymphocytes
• common, increases with age
• patients die of unrelated conditions
• treatment only for symptoms, bone marrow failure

Question 6

what are B symptoms

Answer 6

• Weight loss> 10% in 6 months
• Night sweats- change nightclothes/ bed linen
• Fever >38

usually present in lymphomas

(NHL has lymphadenopathy too)

Question 7

how to recognise lymphoma

Answer 7

• swelling of lymph nodes
• Include it into differential
• B symptoms
• Scan or exam for lymphadenopathy (disease of lymph nodes)
• Biopsy must be used to confirm diagnosis
• CT to stage tumour extent and response to therapy

Question 8

conditions for myelomas

Answer 8

1. Plasma cells in marrow >10%
2. Detectable paraprotein in blood or urine
3. Lytic lesions on skeletal survey

2/3 must be met

Question 9

myelodysplasia vs myeloproliferative disorders

Answer 9

dyplasia:

• Disordered maturation of blood cells in bone marrow
• Easy to diagnose if chromosomal abnormality detected or severe disorganisation of marrow
• Hard to distinguish from reactive marrow changes (rheumatoid arthritis)

Can progress to acute leukaemia

proliferative:

• Excess of mature cells in blood
• Polycythaemia Rubra Vera (PRV) = excess red cells
• Essential Thrombocythaemia (ET)= excess platelets
• Can progress to myelofibrosis or acute leukaemia

Myelofibrosis (MF) = excess bone marrow scarring due to abnormal megakaryocyte leading to bone marrow failure