Haematology Flashcards
VTE length of warfarin treatment?
Provoked- 3 months
Unprovoked- 6 months
Why must all Hodgkin’s lymphoma patients be given irradiated blood products?
To prevent transfusion-associated graft versus host disease (taGVHD), for the rest of their life. Irradiated blood destroys all nucleated cells (such as leucocytes), therefore, eliminating the risk of taGVHD.
Features of CLL?
often none: may be picked up by an incidental finding of lymphocytosis
constitutional: anorexia, weight loss
bleeding, infections
lymphadenopathy more marked than chronic myeloid leukaemia
Blood film of CLL?
Smudge cells
Features on blood films in G6PD?
Heinz bodies on blood films. Bite and blister cells may also be seen
What is multiple myeloma?
Haematological malignancy characterised by plasma cell proliferation. It arises due to genetic mutations which occur as B-lymphocytes differentiate into mature plasma cells.
Mneumonic for features of multiple myeloma?
CRABBI
Hypercalcaemia
Monoclonal production of immunoglobulins results in light chain deposition within renal tubules
Bone marrow crowding suppresss erythropoiesis leading to anaemia
Also causes thrombocytopenia which puts patient at risk of bleeding and brusiing
bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity creates lytic bone lesions
a reduction in the production of normal immunoglobulins results in increased susceptibility to infection
What is polycythaemia ruba vera?
myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets
Management of neutropenic sepsis?
empirical antibiotic therapy with piperacillin with tazobactam (Tazocin) immediately
Steps given by Passmed man on knowing if CML, AML, CLL, ALL
- Low Lymphocytes -> the answer should be AML or CML
- WBC > 100 -> Chronic causes so Consider CML (rule out AML)
- Presence of bands cell -> confirm CML
Pathophysiology of G6PD deficinecy?
↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress
Blood film features of G6PD deficinecy?
Heinz bodies on blood films. Bite and blister cells may also be seen
Some drugs causing haemolysis
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
Some drugs causing haemolysis
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
Reversal agent for dabigatran?
Idarucizumab
How are thrombotic crisises diagnosed?
Clinically
Features of hyposplenism?
Howell-Jolly bodies
siderocytes
Blood films in hyposplenism?
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
Blood films in iron deficinecy anaemia?
target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cellstarget cells
Most common cause of thrombophilia?
Factor V leiden
What is idiopathic thrombocytopenia purpura?
immune mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb-IIIa or Ib complex
Blood transfusion prodict that has the highest risk of bacterial contamination?
Plateelts
First line for CML?
Imatinib
What is first line for cancer patients with a VTE?
DOAC
Management of warm autoimmune haemolytic anaemia?
treatment of any underlying disorder
steroids (+/- rituximab) are generally used first-line
What is Methaemoglobinaemia
haemoglobin which has been oxidised from Fe2+ to Fe3+. This is normally regulated by NADH methaemoglobin reductase, which transfers electrons from NADH to methaemoglobin resulting in the reduction of methaemoglobin to haemoglobin. There is tissue hypoxia as Fe3+ cannot bind oxygen, and hence the oxidation dissociation curve is moved to the left
Diagnostic test of hereditary spherocytosis?
EMA binding test
Investigations for hodgkins lymphoma?
normocytic anaemia- may be multifactorial e.g. hypersplenism, bone marrow replacement by HL, Coombs-positive haemolytic anaemia etc
eosinophilia- caused by the production of cytokines e.g. IL-5
LDH raised
lymph node biopsy- Reed-Sternberg cells are diagnostic: these are large cells that are either multinucleated or have a bilobed nucleus with prominent eosinophilic inclusion-like nucleoli (thus giving an ‘owl’s eye’ appearance)
What is an aplastic crisis?
caused by infection with parvovirus
sudden fall in haemoglobin
bone marrow suppression causes a reduced reticulocyte count
Patients with beta thalassaemia-major need regular transfusions from birth. As a result, they are at a high risk of iron overload from transfusions. What should be given?
Iron chelation therapy, such as desferrioxamine is important to reduce the risk of complications.
Patients with beta thalassaemia-major need regular transfusions from birth. As a result, they are at a high risk of iron overload from transfusions. What should be given?
Iron chelation therapy, such as desferrioxamine is important to reduce the risk of complications.
Lab findings of myelofibrosis?
anaemia
high WBC and platelet count early in the disease
‘tear-drop’ poikilocytes on blood film
unobtainable bone marrow biopsy - ‘dry tap’ therefore trephine biopsy needed
high urate and LDH (reflect increased cell turnover)
most common inherited thrombophilia,
Factor V Leiden (activated protein C resistance)
Recombinant human granulocyte-colony stimulating factors are used to do what? What are some examples?
increase neutrophil counts in patients who are neutropenic secondary to chemotherapy or other factors.
Examples include:
filgrastim
perfilgrastim
What is give to patient’s with polycythaemia vera to reduce the risk of thrombotic events
Aspirin
Prognosis of polycythemia vera?
thrombotic events are a significant cause of morbidity and mortality
5-15% of patients progress to myelofibrosis
5-15% of patients progress to acute leukaemia (risk increased with chemotherapy treatment)
if an US scan is negative but the D-dimer is positiv what should be done?
stop interim therapeutic anticoagulation
offer a repeat proximal leg vein ultrasound scan 6 to 8 days later
most common form of lymphoma in the UK
Diffuse large B cell lymphoma
First line for immune thrombocytopenia in adults?
first-line treatment for ITP is oral prednisolone
pooled normal human immunoglobulin (IVIG) may also be used
it raises the platelet count quicker than steroids, therefore may be used if active bleeding or an urgent invasive procedure is required
splenectomy is now less commonly used
What is autoimmune intermittent poryphoria?
rare autosomal dominant condition caused by a defect in porphobilinogen deaminase, an enzyme involved in the biosynthesis of haem. The results in the toxic accumulation of delta aminolaevulinic acid and porphobilinogen. It characteristically presents with abdominal and neuropsychiatric symptoms in 20-40-year-olds. AIP is more common in females (5:1)
Complications of CLL?
anaemia
hypogammaglobulinaemia leading to recurrent infections
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)
Mnumonic for cyoperitate?
CryoperitEIGHT
Factor VIII is the major consitutiet
What does cryoperitate contain?
contains concentrated Factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin, produced by further processing of Fresh Frozen Plasma (FFP). Clinically it is most commonly used to replace fibrinogen
Burkitt’s lymphoma is associated with what gene translocation?
c-myc gene translocation, usually t(8:14).
What is desmopressin?
synthetic analogue of vasopressin a.k.a antidiuretic hormone (ADH). It stimulates the release of Von Willebrand factor from Weibel-Palade bodies in endothelial cells.
What is a sequestration crisis?
sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
associated with an increased reticulocyte count
Features of acute promyelotic leukemia?
associated with t(15;17)
fusion of PML and RAR-alpha genes
presents younger than other types of AML (average = 25 years old)
Auer rods (seen with myeloperoxidase stain)
DIC or thrombocytopenia often at presentation
good prognosis
