Haematology Flashcards
Most common type of autoimmune haemolytic anaemia?
Warm
Antibody in warm haemolytic anaemia?
Where does haemolysis tends to occur?
IgG
Extravascularly (e.g. spleen)
Antibody in cold haemolytic anaemia?
Where does haemolysis tends to occur?
IgM
Intravascualrly
Drug that can cause warm AI haemolytic anaemia?
Methyldopa
Drugs that can cause aplastic anaemia?
Cytotoxic agents
Phenytoin
Gold
Sulphonamides
Feature of autoimmune haemolytic anaemia (not just haemolytic)?
Positive direct Coomb’s test
Management of hereditary spherocytosis?
Folate supplementation
Splenectomy
Cholecystectomy if gallstones are problematic
Inheritance of spherocytosis/ellipotocytosis?
Autosomal dominant
Most common inherited haemolytic anaemia (Northern Europeans)?
Spherocytosis
Inheritance of G6PD deficiency?
X-linked recessive
Blood film in G6PD deficiency?
Heinz bodies
Bite and blister cells
Diagnosis of G6PD deficiency?
G6PD enzyme assay
Triggers of crisis in G6PD deficiency?
Fava beans
Anti-malarials (primaquine)
Sulphonlyureas, Sulphasalazine, other sulphonamides
Ciprofloxacin
What are patients with paroxysmal nocturnal haemaglobinuira predisposed to?
VTE - PE, DVT, hepatic vein thrombosis
Muscle dystonia - oesophageal dysmotility, erectile dysfunction
Causes of microangiopathic haemolytic anaemia?
Haemolytic Uraemic Syndrime
Disseminated Intravascular Coagulopathy
Thrombotic thrombocytopenia purpura
Systemic Lupus Erythematous
Cancer
Hyposplenism features?
(biochemical)
Howell-Jolly bodies
Siderocytes
Target cells
Pappenhiemer bodies
Acanthocytes
Criteria for diagnosis of acute chest syndrome in sickle cell anaemia?
- Fever or respiratory symptoms
- New infiltrates seen on CXR
Definitive diagnosis of sickle cell anaemia?
Haemaglobin electrophoresis
Vaccines in sickle cell anaemia?
Should receive pneumococcal vaccine every 5 years
Haemophillia biochemistry?
Prolonged APTT
Prothrombin and thrombin time normal
Bleeding time normal
Inheritance of thalassaemia?
Autosomal recessive
Features of thalassaemia major?
Severe microcytic anaemia
Bone deformities
Splenomegaly
Complication in thalassaemia (due to lots of transfusions)?
Iron overload
Require iron chelation
Iron chellating agent?
Desferrioxamine
FBC in beta thalassaemia trait?
Microcytosis is characteristically disproportionate to the anaemia
Most common cause of thrombophilia?
Factor V Leiden (activated protein C resistance)
Evan’s syndrome ?
Immune thrombocytopenia with associated autoimmune haemolytic anaemia
Management of immune thrombocytopenia (adults)?
Prednisolone (oral)
Deficiency in thrombotic thrombocytopenia Purpura?
ADAMTS13 protein
(this protein inactivates vWF)
Diagnosis of myelodysplastic syndrome?
Bone marrow aspiration and biposy
Blood film for myelodysplastic syndrome?
May be blasts
Progression of myelodysplastic syndrome?
Increased risk of acute myeloid leukaemia
Philadelphia chromosome translocation association?
Acute Lymphoid Leukaemia
(30% adults, 3-5% children)
9 to 22 translocation = CML
Blood film in CLL?
Smudge or smear cells
Richeter’s transformation?
CLL to high grade (non-Hodgkin) lymphoma
Patient become very suddenly unwell
Complication of CLL?
Warm autoimmune haemolytic anaemia
Phases of CML?
Chronic: 5 years, usually asymptomatic
Accelerated: 10-20% blast cells, symptomatic
Blast: >30% blast cells, sever symptoms, can be fatal
CML features?
Increased granulocytosis at different stages of maturation
Thrombocytosis
Decreased leukocyte alkaline phosphatase
Blood film AML?
Blast cells with Auer rods (rods in cytoplasm)
Side effects of Erythropoietin ?
Flu-like symptoms
Bone aches
Rashes
Tumour Lysis Syndrome scoring system?
Cairo-Bishop
2 or more of the following (within 3 days before and 7 days post chemotherapy):
1. Uric acid > 475 or 25% increase
2. Potassium > 6 or 25% increase
3. Phosphate > 1.125 or 25% increase
4. Calcium < 1.75 or 25% decrease
Following initial investigations where myeloma is suspected?
BLIP
Bence-Jones protein (urine electrophoresis)
Serum free Light chain assay
Serum Immunoglobulins
Serum Protein electrophoresis
Bone marrow biopsy is necessary to confirm diagnosis of myeloma
Initial investigation is myeloma is suspected?
FBC (low WCC)
Calcium (raised)
ESR (raised)
Plasma viscosity (raised)
if any of these are positive must urgent serum electrophoresis and urine Bence-Jones protein
Which symptoms = a poor prognosis of in lymphoma?
B symptoms
Mutation in polycythaemia vera?
JAK2
Management of polycythaemia vera?
Aspirin (75mg daily)
Venesection to maintain haematocrit < 0.45
Management if INR is between 5-8 (patient is not bleeding)?
Withold 1 or 2 doses of Warfarin
Richter’s transformation?
CLL to non-hodgkin lymphoma
Features of thrombotic thrombocytopenic purpura? (pentad)
- Fever
- Neuro signs
- Throbocytopenia
- Haemolytic anaemia
- Renal failure
What are haptoglobin?
Binds to haemoglobin, decrease in HUS
When should vaccines be given to a patient going for elective splenectomy?
2 weeks prior to surgery
1st line management in warm acute haemolytic anaemia ?
Steroids +/- rituximab
Why is there increased risk of VTE in factor V Leiden ?
Activated factor V is inactivated more slowly by Protein C
Description of Reed-Sternberg cell?
Mirror image nuclei
Management of antiphospholipid syndrome after first VTE?
Life long warfarin
Warfarin PT and APTT?
Prolonged PT
Normal APTT
Most common type of lymphoma in the UK?
Diffuse large B cell lymphoma
Which transfusion product has the highest risk of bacterial contamination compared to other types of blood products?
Platelets
Blood film in DIC?
Schistocytes
What needs careful monitoring in use of unfractioned heparin ?
APTT
Unpredictable anticoagulant effect
Which heparin is high risk for HIT?
Unfractioned
Monitoring in LMWH?
Anti Xa assay
Which pathway does warfarin mostly affect?
Extrinsic (PT)
What is raised in beta thalassaemia major?
HbA2
DIC typical blood picture?
Low platelets
Low fibrinogen
Increased PT and APTT
Increased fibrinogen degradation products
Blood film in autoimmune haemolytic anaemia?
Spherocytes can be found in AIHA as well as hereditary spherocytosis
Management of KIT positive tumours (GI stromal tumours/CML)?
Imatinib
What forms the major criteria for cryoprecipitate use?
Fibrinogen level (low)
When is irradiated blood required ?
Severe immunodeficiency
History of Hodgkin lymphoma
Exposure to certain drugs (bendamustine)
Following stem cell transplant (allogenic for longer)
Starry sky appearance on lymph node biopsy?
Burrkit’s lymphoma
C-myc gene translocation?
Burkitt’s lymphoma
Blood film iron deficiency anaemia ?
Target clue
Pencil poikilocytes
Myelofibrosis bloodfilm?
Tear drop piokilocytes
Intravascular haemolysis blood film?
Schistocytes
Megalobiastic anaemia blood films?
Hyper segmented neutrophils
Diagnosis of hereditary spherosytosis?
EMA binding
Drugs causing haemolysis in G6PD deficiency?
Ciprofloxacin
Sulphon drugs
Anti malarial drugs
Type of haemolysis in G6PD deficiency?
Intravascular
Type of haemolysis in spherocytosis?
Extravascular
Blood film in lead poisoning?
Microcytic anaemia and basophilic stippling
Most common type of Hodgkin lymphoma?
Nodular sclerosing
Best prognosis in Hodgkin ?
Lymphocyte predominant