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
Ann arbour staging of lymphona?
stage 1: 1 lymph node = 1 splodge
stage 2: 2 areas on same side of diaphragm = 2 splodges
stage 3: areas on both sides of diaphragm = 3 splodges (OG lymph node and both sides of diaphragm)
stage 4: mets outside lymph system = 4 splodges
What is polycythaemia?
having a high concentration of red blood cells in your blood. This makes the blood thicker and less able to travel through blood vessels and organs
What causes hypersegmented neutrophils
Cant rememebr
What causes hypersegmented neutrophils
Cant rememebr
What is an importnat test to do if have an unexplained thrombocytopenia?
HIV
When does Ritcher’s transformation occur?
when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma. Patients often become unwell very suddenly.
What is aplastic crises caused by?
Infection with parvovirus
Haemoglobin and reticulocyte count in an aplastic crises?
sudden fall in haemoglobin
bone marrow suppression causes a reduced reticulocyte count
Features of acute haemolytic reaction?
Fever, abdominal pain, hypotension
What is myelofibrosis?
myeloproliferative disorder
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)
Mnemonic for transfusion reactions:
G raft vs. Host disease
O verload
T hrombocytopaenia
A lloimmunization
B lood pressure unstable
A cute haemolytic reaction
D elayed haemolytic reaction
U rticaria
N eutrophilia
I nfection
T ransfusion associated lung injury
Antiphospholipid syndrome in pregnancy:
Aspirin and LMWH
What is sideroblastic anaemia?
disorder where the body produces enough iron but is unable to put it into the haemoglobin
How to remeber that shistocytes are seen in DIC?
‘schistocyte’ is a fragmented red blood cell, from the Greek word schistos for ‘divided’ - this is where we get the word ‘schism’
just in case that helps anyone remember
haemolytic anaemia –> schism in the cell
Bone marrow aspirate in myeloma?
confirms the diagnosis if the number of plasma cells is significantly raised
What drug should be prescribed to reduce risk of tumour lysis syndrome?
Allopurinol
Most common hodgkin’s lymphoma?
Nodular sclerosis
Best prognsosis hodgkins lymphoma?
Lymphocyte predominant
Worst progrnosis hodgkins lymphoma?
Lymphocyte depleted
What does haptoglobin do?
Binds to free haemoglobin
What should be given in DIC after resus
fresh frozen plasma
Transfusion Associated Circularory Overload signs
Hypertension, raised jugular venous pulse, afebrile, S3 present.
Transfusion Related Acute Lung Injury signs?
Hypotension, pyrexia, normal/unchanged JVP
What is immune thrombocytopenia purpura?
immune-mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.
Management of ITP?
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 an exchange transfusion?
Way of reducing the number of sickle red cells and increasing the number of normal red cells, in order to improve oxygenation
What should be used in patients who are found to have iron deficiency anaemia prior to surgery if oral iron either can’t be tolerated or the time interval is too short?
IV iron
What is a deficinecy in factor VIII called?
Haemophilia a
What is PT a test of?
Extrinsic pathway- VII
What is aptt a test of?
Intrinsic pathway- VIII, IX, XI and XII
What is haemophilia B a deficinecy in?- Christmas disease
Factor IX
What is haemophilia C?
Deficinecy in factor XI
Features of vitamin B12 deficiency
macrocytic anaemia
sore tongue and mouth
neurological symptoms
the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia
neuropsychiatric symptoms: e.g. mood disturbances
First line in CML?
imatinib
inhibitor of the tyrosine kinase associated with the BCR-ABL defect
What is myelofibrosis?
Bone marrow is replaced with connective tissue- Fibrosis. Interferes with cell production
Hypersegmented neutrophils on the blood film indicate what?
Macrocytic anaemia
What reverses rivoroxaban and apixaban?
Andexanet alfa
What reverses dabigatran?
Idarucizumab
Peripheral blood film in myeloma?
rouleaux formation
Antibiotic therapy for neutropneic sepsis?
piperacillin with tazobactam (Tazocin)
What are target cells associated with?
Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease
What are spherocytes associated with?
Hereditary spherocytosis
Autoimmune hemolytic anaemia
What is basophilic stripping associated with?
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
What are howell-jolly bodies associated with?
Hyposplenism
What are Heinz bodies associated with?
G6PD deficiency
Alpha-thalassaemia
What are shistocytes assoictaed with?
Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation
What are ‘Pencil’ poikilocytes associated with?
Iron deficinecy anaemia
What are Burr cells (echinocytes) associated with?
Uraemia
Pyruvate kinase deficiency
What are Acanthocytes associated with?
Abetalipoproteinemia
Treatment of tranfusion-associated circulatory overload?
IV loop diuretic
What is Burkitt lymphoma?
rapidly proliferating B cell tumour.
What does Burkitt lymphoma show on lymph node biopsy?
Starry sky appearance
Some drugs causing haemolysis
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
G6PD deficiency drugs?
sulph- drugs: sulphonamides, sulphasalazine and sulfonylureas can trigger haemolysis
What are thymomas associated with?
myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH
First line treatment of polycythemia vera?
Venesection
What is dabigatran?
Direct thrombin inhibitor
Features of lead poisoning?
abdominal pain
peripheral neuropathy (mainly motor)
neuropsychiatric features
fatigue
constipation
blue lines on gum margin (only 20% of adult patients, very rare in children)
Sickle cell patients should be started on what to reduce the incidence of complications and acute crisis?
hydroxycarbamide
What is acute intermittent porphyria?
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)
What is factor V leiden?
Resistant to activated protein C
Investigation for multiple myeloma?
Whole body MRI
When might hypersegmented neutrophils be seen?
vitamin B12 or folic acid deficiency
Drugs causing aplastic anaemia?
: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
What is Waldenstrom’s macroglobulinaemia
lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein
most common inherited thrombophilia
Activated protein C resistance (Factor V Leiden)
To diagnose G6PD deficiency what investigation should be done?
enzyme assay should be repeated around 3 months after an acute episode of haemolysis
Clinical tumor lysis syndrome diagnostic criteria?
laboratory tumour lysis syndrome plus one or more of the following:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure
What immunoglobulin deficinecy increases risk of anaphylaxis in blood transfusion?
IgA
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
