Haem + onc Flashcards
Which organisms most commonly cause neutropenic sepsis?
Coagulase -ve gram +ve bacteria
particularly staph epidermis
What are auer rods? what conditions are they seen in?
Large, pink / red needle shaped structures in cytoplasm of myeloid cells
Seen in AML
Also in APML, high grade myelodysplastic syndromes and myeloproliferative disorders
What are some features of APML?
Presents younger than other forms of AML (avg 25yrs)
DIC / Thrombocytopaenia at presentation
Good prognosis
Genetic cause of APML
t(15;17) translocation - fusion of PML and RAR-alpha genes –> blocks the maturation of myeloid cells and leads to an accumulation of promyelocytes.
APML mx?
Treated with all-trans retinoic acid (ATRA) -> forces immature granulocytes into maturation to resolve blast criseses prior to definitive chemo
Bloods in DIC? Blood film?
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia
How can you distinguish between Waldenstrom’s macroglobulinaemia and multiple myeloma?
Waldenstrom’s macroglobulinaemia - Organomegaly with no bone lesions
Multiple myeloma - Bone lesions with no organomegaly
What are some features of Waldenstroms macroglobulinaemia?
Mx?
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance (the pentameric configuration of IgM increases serum viscosity)
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s
Mx - rituximab based combination chemo
Ix findings for waldenstrom’s macroglobulinaemia?
Monoclonal IgM paraproteinaemia (M - Macroglobulinaemia)
BM biopsy - diagnostic - infiltration of BM with lymphoplasmacytoid lymphoma cells
What genetic abnormality is associated with chronic myeloid leukaemia (CML)?
Philidelphia chromosome - t(9;22)
seen in 95% of cases
NB also seen in 25% of adults with ALL - poor prognostic feature in these cases
What genetic abnormality is seen in Burkitt’s lymphoma?
t (8;14) seen in Burkitt’s lymphoma
MYC oncogene is translocated to an immunoglobulin gene
What genetic abnormality is seen in Mantle cell lymphoma?
t(11;14) is seen in mantle cell lymphoma
deregulation of BCL-1 gene
What genetic abnormality is seen follicular lymphoma?
t(14;18) - increased BCL-2 transcription
What are some intravascular causes of haemolytic anaemia?
mismatched blood transfusion
G6PD deficiency*
red cell fragmentation: heart valves, TTP, DIC, HUS
paroxysmal nocturnal haemoglobinuria
cold autoimmune haemolytic anaemia
Extravascular causes of haemolysis
haemoglobinopathies: sickle cell, thalassaemia
hereditary spherocytosis
haemolytic disease of newborn
warm autoimmune haemolytic anaemia
What does haptoglobin do?
Binds to free Hb which is released in intravascular haemolysis - hence depleted in intravascular causes
What happens to free Hb once haptoglobin is saturated? how is this detected?
As haptoglobin becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by Schumm’s test).
Bloods in Warfarin?
PT - Prolonged
APTT - Normal
Bleeding time - Normal
Plts - Normal
Bloods in Aspirin use?
Bleeding time - prolonged
PT - Normal
APTT - Normal
Plts - Normal
Bloods in heparin use?
APTT - Prolonged
PT - Normal (usually) / prolonged
Bleeding time - normal
Plts - Normal
How does ITP present?
Commonly in older females - isolated thrombocytopaenia usually incidentally on bloods
If sx:
- petechiae, purpura, bleeding (epistaxis) -> catastrophic bleeding is not common
What is Evans syndrome?
When ITP is associated with AIHA
How can you distinguish between Ig in Cold and Warm AIHA?
Warm = Greece IgG
Cold = Moscow IgM
What can be seen in blood film in hereditary spherocytosis post surgery and why?
Howell-Jolly bodies - these are remnants of RBC nucleus normally removed by spleen
Hence visible post splenectomy
What causes raised MCHC?
Hereditary spherocytosis
AIHA - associated w/ spherocytosis
What causes reduced MCHC
Microcytic anaemia eg IDA
What is disproportionate microcytic anaemia? when is this seen?
This is when MCV is far more reduced than drop in Hb
Seen in B-thalassaemia trait
Blood film findings in hyposplenism? examples?
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
eg. coeliac / post-splenectomy
Bloods film features in IDA?
target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells
‘Tear drop’ poikilocytes are suggestive of which condition?
Myelofibrosis
What blood film feature is found in intravascular haemolysis?
Schistocytes
What are hypersegemented neutrophils suggestive of?
Megaloblastic anaemia
What are some examples of aromatase inhibitors? main adverse effects?
Anastrazole and Letrozole - used in ER +ve breast ca -> prevents androgen conversion into oestrogens (reducing peripheral oestrogen synthesis)
Adverse:
- Osteoporosis -> DEXA recommended when initiating
- Hot flushes
- Arthralgia + Myalgia
- Insomnia
Which medication should be used for chemo associated N+V?
Low risk - metoclopramide
High risk - 5HT3 antagonists eg ondansetron esp combined w dex
What genetic condtion is associated w young age tumours esp sarcomas + leukaemias? gene?
Li-Fraumeni Syndrome
p53 gene (sarcoma <45y & 1st degree relative any cancer + other family member any cancer <45y or sarcoma any age)
What is BRCA2 associated with in men?
Prostate Ca
Main symptoms of SVCO?
MOST COMMON - Dyspnoea
Swelling of face, neck and arms - ?oedema
Headaches - worse in AM
Visual disturbance
Pulseless JV distension
What is a leukaemoid reaction?
Presence of immature cells ef myeloblasts, promyelocytes and nucleated RBCs in peripheral blood
Due to infiltration of BM -> immature cells being pushed out
OR
Raised demand for new cells
What are some causes of leukaemoid reactions?
Severe infection
Severe haemolysis
Massive haemorrhage
Met Ca w/ BM infiltraion
How can you distinguish between leukaemoid reaction + myelofibrosis and CML?
Leucocyte Alkaline phosphatase (LAP) - raised in reactive response
CML - Low LAP
Leukaemoid reaction + Myelofibrosis - High LAP
What is HTLV1 and what ca is it associated with?
HTLV1 = human T cell lymphotropic virus
Associated w/ Adult T cell leukaemia
What ca is EBV associated with?
Hodgkins lymphoma
Burkitts lymphoma
Nasopharyngeal caricinoma