Haem Onc Flashcards
In general what is the difference between leukaemia vs lymphoma?
Leukaemia = mainly affects bone marrow +/- circulation neoplastic cells in blood Lymphoma = predominantly nodal / organ based
What are some of the symptoms of Hypercalcaemia?
- Fatigue
- Abdominal pain
- N&V
- Polyuria
- Polydipsia
- Constipation
- Confusion
- Headaches
Which cancer are Auer rods found in?
Acute Myeloid Leukaemia
Which age group is ALL most common in?
Younger patients
Which multi-drug chemotherapies are available for ALL?
- BiTe
- CAR-T
Which age group is AML most common in?
Older patients
how does CML present?
- High WCC
- Splenomegaly
What is the gene transformation in CML?
Philadelphia chromosome (BCR-ABL)
Cytogenetic translocation of chromosomes (9:22)
How is CML treated?
Tyrosine kinase inhibitors e.g imatinib
How is AML treated?
- Intensive vs Non-intensive (if poorer QoL)
- standard of care vs trial
- Allogeneic stem cell transplant if poor risk
How does CLL present?
- Relapsing remitting course
- High numbers of B lymphocytes
- Can have nodal or splenic disease
How is CLL treated?
Chemotherapy or target immune treatments e.g. ibrutinib
only those who are symptomatic get treated others are on watch and wait pathway
What are some of the clinical features of essential thrombocytosis?
- Thrombosis (arterial or venous)
- haemorrhage - if platelet count >1,500
- Splenomegaly due to sequestration
What transformations can occur in essential thrombocytosis?
- Transformation to myelofibrosis
- Leukaemia transformation to AML
Which mutation is commonly seen in Polycythaemia (rubra) vera?
JAK 2
What is the definition of Polycythaemia (rubra) vera?
Uncontrolled production of red cells in bone marrow despite erythropoietin production being switched off
What are some of the symptoms related to viscosity seen in Polycythaemia (rubra) vera?
- Headaches
- Visual disturbances (transient)
- Drowsiness
- Pruritis especially after a warm bath
- Skin - plethoric complexion (flushed face)
What are some of the secondary causes of increased platelets (not malignancy related)?
- Bleeding
- Iron deficiency
- Inflammation
- Infection
- Post surgery
- Splenectomy
What are some of the secondary causes of increased red cells (non malignancy related)?
- Chronic hypoxia
- Altitude
- Smoking and alcohol
- OSA
- Renal/ hepatic tumours
- Congenital heart disease
- EPO supplement overload
What is myelofibrosis?
A CLONAL disorder of haematopoietic stem cells
What mutation is seen in myelofibrosis?
JAK 2
How is myelofibrosis treated?
- supportive care (transfusion)
- cytoreduction drugs
- JAK2 inhibitors
- Splenectomy (rare now)
- Bone marrow transplant (often risk outweighs benefit though)
What is myelodysplasia?
A diagnosis of exclusion, a strider of ineffective haematopoeisis
What is the definition of lymphoma?
A CLONAL proliferation of lymphoid cells
What are the two main subtypes of lymphoma?
- Hodgkin
- Non-Hodgkin
What are the two main subtypes of Non-Hodgkin lymphoma?
Low grade and High grade
What are two types of high grade non-Hodgkin lymphoma?
- Diffuse large B cell
- Burkitt lymphoma
What are two types of low grade non-Hodkin lymphoma?
- Follicular
- Mantle cell lymphoma
What are the specific translocations seen in two types of low grade non-Hodkin lymphoma?
- Follicular = t(14;18) prevents normal switching off of BCL-2
- Mantle cell lymphomas = t(11;14) causes over expression of cyclin D1
What are some of the disease sites of lymphoma?
- Gastric MALT lymphoma
- Splenic marginal zone lymphoma
- Cutaneous lymphoma
- Hepatosplenic T cell lymphoma
What is the definition of myeloma?
-Clonal population of abnormal plasma cells (B lymphocytes)
What are the solid plasma cell tumours called?
Plasmacytoma
How may myeloma present?
- Bones: osteopenia, pathological fractures
- Hypercalcaemia: polyuria, polydipsia, confusion, abdominal pain, constipation
- Bone marrow infiltration: anaemia, thrombocytopenia, neutropenia
- Infections
- Hyperviscocity syndromes: visual disturbance, headache
- Neuropathy
- Bleed diathesis (affects clotting factors)
What are myeloma defining events?
SliM
S- Sixty % bone marrow plasmaytosis
Li- Light chain ration >100
M- >1 focal lesion on MRI >5mm
What 4 things show end organ damage in myeloma?
- Calcium elevation >2.75
- Renal impairment (Cr >177/CrCL <40)
- Anaemia (Hb <100)
- Bone lesions (1 + on XR, CT, PET)
Must be accompanied by >10% clonal population of BM plasma cells
What does the serum free light chain show? What result would you see in myeloma
Serum free light chains shows both kappa and lambda chains, normal ratio is 2:1 kappa:lambda
If all kappa high = high ratio and likely to be clonal = myeloma
If both kappa and lambda are high = polyclonal = not myeloma
What type of bone lesions do you see in myeloma
Lytic e.g. pepper pot skull
What defines smouldering/ asymptomatic myeloma?
- Absence of any myeloma defining events or amyloidosis
- Paraprotein/ M protein >30g/L
- Clonal bone marrow plasma cells 10-60%
What is MGUS and how is defined clinically?
Monoclonal Gammopathy of undetermined significance
- Serum paraprotein. <30g/L
- Clinical bone marrow plasma cells <10%
- Absence of CRAB or amyloidosis
What is amyloidosis?
Generic term for extracellular tissue deposition of fibrils (beta-pleated sheet)
What is immunoglobulin light chain amyloidosis?
Amyloidosis in which fibrils are composed of fragments of monoclonal light chains
What staining is used in amyloidosis?
Congo red
