Haematology/Oncology Flashcards
When does neutropenic sepsis most commonly occur
7-14 days after chemotherapy
How is G6PD deficiency inherited
x link recessive pattern
what drugs can precipitate haemolysis in G6PD deficiency
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
what side effects is cisplatin commonly associated with
Ototoxicity, peripheral neuropathy, hypomagnesaemia
what is often given prior to chemo in Burkittss lymphoma to prevent tumour lysis syndrome
Rasburicase (a recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin*) is often given before the chemotherapy to reduce the risk of this occurring
what is wiskott aldrich syndrome
Wiskott-Aldrich syndrome causes primary immunodeficiency due to a combined B- and T-cell dysfunction. It is inherited in a X-linked recessive fashion and is thought to be caused by mutation in the WASP gene.
what is the most common inherited thrombophilia
factor V leiden
how is von willebrnd inheritied
autosomal dominant
translocation in Burkitts
T8:14
what virus is associated with burkitss lymphoma
EBV
what is the best screening test for hereditary angioedema
use C4 between attacks (low)
how does desmopressin help in von willebrand disease
raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
what is a thymoma associated with
myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH
give causes of intravascular haemolysis
G6PD
cold autoimmune haemolytic anaemia
mismatched bloods transfusion
PNH
what is the most common type of lymphoma
nodular sclerosing
how is hereditary spherocytosis diagnosed
the British Journal of Haematology (BJH) guidelines state that ‘patients with a family history of HS, typical clinical features and laboratory investigations (spherocytes, raised mean corpuscular haemoglobin concentration[MCHC], increase in reticulocytes) do not require any additional tests
if the diagnosis is equivocal the BJH recommend the cryohaemolysis test and EMA binding
what is the most common microorganism causign neutropenic sepsis
usually gram positve cocci
staph epidermidis most commonly
what is the diagnostic test for PNH
flow cytometry of blood to detect low levels of CD59 and CD55
what are ppeopl with PNH more at risk of
thrombosis
they should be anticoagulated
what is the pathogenesis of TTP
in TTP there is a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor
what is the pentad of symptoms in TTP
fever, neurological dysfunction, evidence of haemolysis (blood film), renal injury and thrombocytopenia.
what is filgrastin
G-CSF
can be used to trreat neutropenia
what is bombesin a tumour marker for
small cell lung cancers
recurrent bacterial infections (e.g. Chest)
eczema
thrombocytopaenia
low IgM levels
whats the diagnosis
Wiskott-Aldrich syndrome
what treatment is given for CLL
some patients can just be monitord with regular FBCs but if treatment given
fludarabine, cyclophosphamide and rituximab (FCR) has now emerged as the initial treatment of choice for the majority of patients
what is a prognostic marker in myeloma
B2-microglobulin is a useful marker of prognosis - raised levels imply poor prognosis. Low levels of albumin are also associated with a poor prognosis
reed sternberg cells
hodgkins lymphoma
which type of hodgkins lymphoma carries the best prognosis
lymphocyte predominant
what inhertied thrombophilia classically causes skin necrosis on commencing warfarin
protein c deficiency
what can be given to try reduce the likelihood of developing haemorhhagic cystitis from cyclophosphamide
mesna
what can polycythaemia rubra vera progress to
CML
myelofibrosis
tear drop poikolocytes
myelofibrosis
basophilic stippling
lead poisoning
aflatoxin increases risk of which cancer
hepatocellular
tumour marker for primary peritoneal cancer
ca 125
give acquired causes of sideroblastic anaemia
myelodysplasia
alcohol
lead
anti-TB medications
what is li-fraumeni
Autosomal dominant
Consists of germline mutations to p53 tumour suppressor gene
High incidence of malignancies particularly sarcomas and leukaemias
how does cyclophosphamide work
inhibition of IL2
