Haematological Malignancies Flashcards
What is essential thrombocythaemia?
A myloproliferative disorder- too many platelets
What is the name of a myloproliferative disorder that results in too many platelets?
Polycythaemia rubra Vera (PRV)
What causes myelofibrosis?
It is a myloproliferative disorder from abnormal megakaryocytes that release growth factors in bone marrow causing fibroblasts I’m bone marrow to multiply
Name two myloproliferative disorders involving mast cells and eosinophils
Idiopathic hypereosinophilic syndrome
Systemic mastocytosis
What lymphocytes are mostly effected by hodgekins lymphomas
B lymphocytes
What is underlying problem in ET and PRV?
JAK2 mutation
What 3 clinical features occur with bone marrow failure?
Anaemia- tiredness
Thrombocytopenia- bleeding and bruising
Neutropenia - infections
What is myelodysplastic syndrome?
Premature ageing of the bone marrow
How do you treat AML?
Strong IV chemotherapy in short, sharp, bursts. This is why young people do better
How do you treat ALL?
Mix of strong chemotherapy and persisting milder tablets to prevent replapse.
Why are lymphocytes hardest to kill off?
Because they want to last longer- memory B cells
What is the leak incidence of ALL?
4-5 years. May present with cytopenia or chest masses
What is the cure rate for ALL?
85%
Where are the sanctuary sites for ALL that can cause relapse?
Testes and CNS
What are the most common leucocytes found in CML?
Neutrophils and metamyelocytes
What is priapism?
Syrupy blood from too many WBCs disrupts blood flow in penis
What cell surface markers would you find in CLL?
Positive for CD5 and CD19
Negative for CD79b
Weak surface immunoglobulins
Negative for FMC7
What is cd5 a marker for?
Activated B cells
What is cd19 a marker for
B cells
What is cd79b a marker for?
Immature B cells
What is stage A of the Binet system for CLL?
Lymphocytosis only - 10 years
What is stage B of the Binet system?
Lymphocytosis and 3+ areas of lymphadenopathy - 5 years
Stage C of the Binet system
Lymphocytosis and LYmphodenopathy and hb
What are non hodgekins lymphomas?
Tumour growth of lymphoid cells
What percentage of NHL’s occur outside of lymph nodes ?
30%
What are b symptoms of lymphoma?
Weight loss >10% in 6 mts, night sweats? Fever above 37
All caused by cytokines
What causes hodgekins lymphoma?
EPV virus
Why do they think there are two age peaks in hodgekins? Teens and elderly?
Due to where the virus has integrated into the lymphocyte DNA- closer or further from Proto oncogenes
True or false: a bone marrow biopsy is diagnostic in lymphoma?
False
Low grade lymphoma
Slow and grumbling
What type of disorder is myeloma?
An immunosecretory disorder
What is MGUS?
Monoclonal gammopathy of uncertain significance - abnormal antibodies but nothing else
What is IgD normally expressed on?
B lymphocytes
What does IgM normally do?
Clears pathogens from circulation
What does IgG normally do?
Clears pathogens from tissue fluid
What does IgA normally do?
Prevents pathogens invading mucosal surfaces
What does IgE normally do?
Protects against parasites
True or false: IgG can switch to IgA and IgE
True
What is a paraproteins and how would I detect it?
Electrophoresis. Monoclonal antibody causing a spike in the normal globulin pattern
What is bence-Jones protein?
Isolated light chain
What criteria must be met for myeloma?
1) plasma cells in marrow>10%
2) detectable paraproteins in blood or urine
3) lytic lesions on Skeletal survey
What is a skeletal survey?
Series of plain X Ray’s - not like bone scan
Think of myeloma when you see elderly with what 5 conditions?
1) anaemia
2) high ESR
3) back pain
4) unexplained renal failure
5) unusually fractures
Is monoclonal IgM a sign of lymphoma?
No, IgM is expressed when lymphocytes are maturing not on plasma cells)
IgM paraproteins and marrow with lymphoplasmacytoid lymphocytes is found in what disease?
Waldenstroms
What is CHAD? What paraproteins is it associated with?
Cold haem agglutinin disease p. IgM
What is hyperviscosity syndrome?
Treacle like blood due to excess plasma proteins - fibrinogen, IgM and IgA
If a patient has anaemia due to hyperviscosity syndrome should u treat with blood transfusion?
No- dangerous. Anaemia reduces the viscosity
What cell lines can be affected by myelodysplasia?
Any or all! Eg pencytopenia- deficieny of red white and platelets
