General revision Flashcards
Important info to remember
what are the criteria to say someone is “red flag sepsis”
News score of 7
News score of 5-6 plus any one of:
-lactate >2mmol/litre
-chemo in last 6weeks
-any other organ failure AKI
-patient looks extremely unwell
-patient is actively deteriorating
What is CML
Chronic Myeloid Leukaemia (CML)
All of myeloid line expanded, basophils prominent
Slower onset but can have accelerated phases
Philadelphia chromosome t(9;22)
What is AML
Acute Myeloid Leukaemia (AML)
Myeloid cells- ++ blasts (immature cells)
Commoner in older adults
Grows rapidly
What is CLL
Chronic lymphocytic leukaemia (CLL)
Lymphoid cells, Disease of older adults
Usually incidental finding and indolent course
Can get Autoimmune Haemolysis
What is ALL
Acute lymphoblastic leukaemia (ALL)
Lymphoid cells
Commonest malignancy in children
Grows rapidly
What is the most common malignancy in children?
Leukaemia: ALL
Which leukaemia is usually an incidental finding
Chronic lymphocytic leukaemia (CLL)
Lymphoid cells, Disease of older adults
Usually incidental finding and indolent course
Can get Autoimmune Haemolysis
Which leukaemia is related to the philadelphia chromosome
Chronic Myeloid Leukaemia (CML)
All of myeloid line expanded, basophils prominent
Slower onset but can have accelerated phases
Philadelphia chromosome t(9;22)
What is venesection used for
Polycythemia rubra vera (PRV)
High Hb and haematocrit
Treat with venesection
What is hydroxycarbamide used for
Essential thrombocythemia (ET)
High platelet count
Treat with cytoreduction (hydroxycarbamide)
Does Myelofibrosis (primary) have high or low cell socunts?
Myelofibrosis (MF)
Very stiff bone marrow- leucoerythroblastic blood film
Can have high or low counts
Constitutional symptoms
Treat with cytoreduction, JAK2 inhibitor or chemo and transplant
What is a JAK2 mutation associated with
Excess myelopoiesis in bone marrow
Typically carry JAK2 V617F mutation (or CALR, C-MPL) or other myeloid gene mutations
Risk of thrombotic events» ALL get Aspirin
Splenomegaly (especially MF)
what are the core elements of BM failure seen clinically
Symptoms and signs of bone marrow failure
Symptoms and signs of infection
Symptoms and signs of tissue infiltration by leukaemia cells
What is Horners syndrome
partial ptosis (drooping or falling of the upper eyelid)
miosis (constricted pupil)
facial anhidrosis (absence of sweating) due to a disruption in the sympathetic nerve supply.
what is myeloma
Malignant proliferation of plasma cells in the bone marrow
what are the risks of BM transplant
Not without risk: allogeneic HLA matched
Graft failure
GvHD- skin, gut, liver
Infection
-Atypical
-Viral reactivation
Fertility
Psychosocial
-Burden of follow up
-Burden of morbidity
Relapse
what is produced in multiple myeloma
The clonal (abnormal army) of plasma cells usually produce:
Monoclonal protein (paraprotein) ie the abnormal immunoglobulin
+/or monoclonal light chains
(very rarely myeloma is non-secretory)
What does CRAB stand for in multiple myeloma
HyperCalcaemia
Due to increased reabsorption of bone
Indicative of more advanced disease
Can present with confusion, dehydration, nausea, arrythmias
Renal Insufficency
Multi-factorial:
Hypercalcaemia
Infection
Dehydration
NSAIDS used for pain
Damage to tubules or glomeruli by the abnormal protein
Anaemia
Due to bone marrow infiltration (prevents normal function of BM)
Bone marrow infiltration can also cause reduced numbers or normal WBCs and lower normal Immunoglobulins and can therefore lead to recurrent infections
Bone lesions
From bone destruction causing pathological fractures or osteoporosis
Can cause neurological symptoms if spinal cord is compressed by collapsed vertebrae
What is a haem emergency that can cause arrythmias
Hypercalcaemia
How to manage hypercalcaemia
Risk of arrythmia
Bisphosphonate
iv fluids
+/- dexamethasone
Elucidate the diagnosis
what is haame emergency that can cause paralysis
Cord compression
How to manage cord compressions Haem emergency
Emergency: risk of neurological damage
MRI imaging modality of choice if suspected e.g. back pain
Bed rest pending scan
Steroids
Radiotherapy
Then chemo asap
Occasionally spinal surgery (if diagnosis unknown for tissue diagnosis and decompression)
how to manage mysleoma/MGUS no CRAB features
If no CRAB features but otherwise meets criteria for myeloma ie abnormal paraprotein or light chains + clonal (malignant rather then normal) plasma calls in bone marrow > 10% then patient is labelled “asymptomatic” or “smouldering” myeloma
If no CRAB features, but has raised LCs or paraprotein but plasma cells in bone marrow are < 10%: MGUS (Monoclonal gammopathy of uncertain significance)
Offer ‘active surveillance’ whereby we can monitor the paraprotein and light chains and FBC/renal/bone bloods
Benefit is ‘save’ treatment for when is really needed (and thus avoid toxicity of treatment, and avoid using up lines of treatment)
How to manage CRAB feature myeloma?
This is essentially symptomatic myeloma with “end organ damage” and patient will need anti-cancer therapy
Chemotherapy/antibody therapy: to kill the plasma cells producing the paraprotein/abnormal light chains (+/- autologous stem cell transplant if considered ‘fit’)
Radiotherapy: for cord compression or painful bone disease or if a ‘solitary lesion/plasmacytoma’
Experimental therapy/trials:
CAR-T vs standard of care, other immunotherapies
Relapse and progressive disease:
Further chemotherapy
Experimental therapies/early phase trials
what damage to the jaw can bisphosphonates cause
osteonecrosis of the jaw (check no dental disease)
what are the supportive care options in myeloma
Bisphosphonate for bone disease
Helps remodelling, decreases risk of myeloma bone disease
Risk of osteonecrosis of the jaw
Spinal support if vertebral disease
Hard brace, usually worn for 3 months
Infection prophylaxis- immuneparesis
Blood clot prevention
Psychological support
Physiotherapy/Occupational therapy
Palliative support/end of life care when appropriate
what are the CRAB features of myeloma
hyperCalcaemia
renal insuficiency
anaemia
bone leisions
most common lypmhoid neoplasms…
> 90% of lymphoid neoplasms are mature B cell
Most common sub-types are Diffuse Large B cell Lymphoma (DLBCL) and Follicular Lymphoma
Approx 4% of new cancers per year, frequency increasing
More common in developed countries
what are more common neoplasms (lymphoid) in asia/Caribbean/welsh/irish?
T cell and NK cell neoplasms uncommon
More common in Asia, Caribbean basin
Enteropathy associated T cell lymphomas
Welsh/Irish (related to coeliac disease genetics)
Which lymphoma is linked with EBV?
Burkitt’s lymphoma endemic in equatorial Africa, Papua new guinea
Jaw and facial bone site presentation 50%
100% with EBV
Sproadic BL, 30% EBV (seen throughout world) abdominal masses (ileocaecal)
Immunodeiciency BL e.g. HIV, AIDS manifestation EBV 25-40%
which malignant neoplasm is related to MYC gene at Ch 8q24 common mechanism
Occurrence of a neoplasm in many different populations and settings
Ultimately different and multiple environmental exposures converge in a common mechanism involving the MYC gene at Ch 8q24
Which lymphoma has “reed-sternberg cells”
Neoplastic tissues contain scattered large mononucleated (Hodgkin cells) and multinucleated (Reed-Sternberg Cells) scattered amongst inflammatory and accessory cells.
A patients blood has this multinucleated cell in it. what disease do they have
classical Hodgkin lymphoma:
multinucleated (Reed-Sternberg Cells) scattered amongst inflammatory and accessory cels
what are the classical features of lymphoma
Lumps or bumps in lymph node sites (lymphadenopathy- often firm, non tender)
“Nodal” – most common
“Extra-nodal”
Itching
Rash
Fatigue
Alcohol induced pain (Hodgkin’s)
“B symptoms”
Night sweats: drenching
Fevers: unexplained, >38°C
Weight loss: > 10% in 6 months prior to diagnosis
what is SVCO and why is it a haem emergency?
bulky lymph nodes from lymphoma obstruct superior vena cava headache, dizziness, facial plethora, oedema, stridor, dyspnoea, cough, dysphagia, prominent venous patterning.
URGENT CT avoid steroids if possible until after a biopsy.
