Haematology Flashcards
How can ITP be identified?
- Bleeding, purpuric rash
- Thrombocytopenia on bloods
- Large platelets, no schistocytes, normal D dimer
What test does ITP require to be excluded from leukemia?
Bone marrow biopsy
- Megakaryocytes seen
Antibodies are directed against what in ITP?
Glycoprotein IIb-IIIa or Ib complex
What blood transfusion reaction is thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage?
- Seen in RBC and platelet transfusions (more common in platelet )
- Features include fever and chills
Non-hemolytic febrile reaction
How is non-hemolytic febrile reaction managed?
Slow or stop transfusion
- Paracetamol
- Monitor
How are Minor allergic reactions to blood transfusions treated?
- Present with: Pruritus, urticaria
Temporarily stop transfusion
- Antihistamine and monitor
What blood transfusion reaction is caused by foreign plasma proteins?
Minor allergic reaction
What blood transfusion reaction can be caused by patients with IgA deficiency who have anti-IgA antibodies?
Anaphylaxis
What type of blood transfusion reaction has angioedema as a feature?
Anaphylaxis
What type of blood transfusion reaction involves ABO-incompatible blood e.g. secondary to human error?
Acute hemolytic reaction
What are the features of an acute hemolytic reaction (blood transfusion reaction)?
- Fever
- Abdo pain
-Hypotension
How can Transfusion-associated circulatory overload (TACO) and Transfusion-related acute lung injury (TRALI) be differentiated?
- TACO = hypertension
- TRALI = Hypotension, fever
How is TRALI treated?
Stop transfusion
- O2 and supportive care
How is TACO treated?
Slow or stop transfusion
- Consider intravenous loop diuretic (e.g. furosemide) and oxygen
What condition may be caused by chemotherapy/radiotherapy and present with pancytopenia
Myelodysplastic syndrome
What can myelodysplastic and myelofibrosis progress into?
AML
What can Polycythemia ruba vera progress to (5-15% of the time)?
- Myelofibrosis
- AML
What can myelofibrosis present with to differentiate it from myelodysplasia?
- Hepatoslpenomegaly
- B symptoms (classic cancer signs)
CLL can transform into what kind of cancer? (1)
What is this transformation called? (2)
- High grade Non-hodgkin lymphoma
- Richter transformation
What is given in antiphospholipid syndrome?
Aspirin and LMWH
1 unit of RBCs should be transfused over the course of how long?
90 - 120 mins
How many Ann Arbor stages? (1)
Describe them (2)
4 stages
Von Willebrand’s disease investigation findings (5)
- Prolonged bleeding time
- APTT may be prolonged
- PT normal
- Factor VIII levels may be moderately reduced
- Defective platelet aggregation with ristocetin
What to do with children and young people with petechiae or hepatosplenomegaly
Immediate specialist assessment
Target cells, Howell-Jolly bodies and acanthocytes (spikey RBCs) are seen i what patients?
Asplenics
Bite cells (Degmacytes), blister cells, heinz bodies seen in what condition
G6PD deficiency
- remember fava BEANS can cause flare up - bite out of heinz beins, holly jolly heinz beans
Imaging for multiple myeloma
whole-body MRI
What investigation confirms diagnosis of MM?
- What will be seen? (2)
Bone marrow aspiration
- Plasma cells significantly raised (>30% diagnostic, 10-30%, minor criteria)
What will be seen on protein electrophoresis in MM?
Raised concentrations of monoclonal IgA/IgG proteins will be present in the serum
In MM raised concentrations of monoclonal IgA/IgG proteins in urine are known as what?
Bence Jones proteins
Beta thalassemia treatment
- Repeat transfusions
- Desferrioxamine (iron chelation)
What do G6PD deficiency flare ups cause?
Hemolysis
What drugs may cause G6PD deficiency flare ups?
Sulpha drugs, malaria prophylaxis, fava beans
Acute intermittent porphyria is inherited how?
Autosomal Dominant
Features of Acute Intermittent Porphyria flare up
- Abdo pain, ?vomiting
- Polyneuropathy ?motor
- Psychiatric features ?depression
- HTN, tachycardia common
- Port wine urine!
Acute intermittent porphyria is a mutation/deficiency in what enzyme?
Porphobilinogen deaminase
How can Acute intermittent porphyria be treated?
- IV haematin / haem arginate (or IV glucose)
- Avoid triggers
Haemarthrosis without trauma, raised APTT and normal PT and BT most likely to be what?
Haemophilia A or B
MM X-ray head finding?
Rain-drop skull
How can AML and CML be differentiated in terms of what is seen on film?
- CML: All stages of granulocyte maturation (?also splenomegaly)
- AML: Blast / immature cells predominate
Polycythemia Vera treated with what?
- Venesection
- Aspirin
- ? chemo -> Hydroxyurea, phosphorus-32
Auer rods seen in what leukemia?
AML
Tear drop poikilocytes seen in what condition?
Myelofibrosis
How can aplastic and sequestration crises be differentiated in terms of bloods?
- Aplastic = Low reticulocytes
- Sequestration = High reticulocytes
What kind of drug is enoxaparin?
LMWH
What kind of drug is dabigatran?
Direct thrombin inhibitor
What is the reversal agent for dabigatran?
Idarucizumab
What is the reversal agent for riveroxiban and apixaban?
Andexanet alfa
Scan for DVT -ve, d-dimer positive, what to do?
Stop anticoag -> repeat scan in 1 week
Tamoxifen increases the risk of what? (2)
- VTE
- Endometrial cancer
Pencil poikilocytes and target cells on blood film is indicative of what condition?
Iron deficiency anemia
Autoimmune hemolytic anemia treatment
Steroids
Irradiated blood products are depleted of T-lymphocytes and used to avoid what condition?
Graft vs Host disease (caused by engraftment of viable donor T lymphocytes)
CMV negative bloods are required in what situations?
- Granulocyte transfusions
- Intra-uterine transfusions
- Neonates up to 28 days post expected date of delivery
- Pregnancy: Elective transfusions during pregnancy (not during labour or delivery)
2 main types of Burkitt’s lymphoma
What are they and where are they common and what organs affected in each?
- Endemic (African) form: typically involves maxilla or mandible. EBV associated
- Sporadic form: abdominal (e.g. ileo-caecal) tumours are the most common form. More common in patients with HIV
What translocation is found Burkitt’s lymphoma?
C-myc gene translocation, usually t(8:14)
Drug used to prevent tumour lysis syndrome in Burkitt’s lymphoma
Rasburicase (recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin)
Gastric MALT lymphoma is associated with what condition? (1)
Bonus point info (2)
H pylori (1)
- If low grade then 80% respond to H. pylori eradication (2)
Gastric MALT lymphoma can have what sign on bloods?
Paraproteinaemia (presence of excessive amounts of myeloma protein or monoclonal gamma globulin in the blood, seen in protein electrophoresis)
NH Lymphoma has many different treatments (chemo, radiotherapy, watch/wait), what monoclonal antibody may be used?
Rituximab
What is the staging system used in NH Lymphoma? (1)
Explain how it works
Lugano staging
- Stage 1-4: Involvement (single node, diaphragm, diffuse)
- Other classification: A/B (symptoms), E (extranodal disease), S (spleen involved) and X (bulky tumor mass) also used
Malaria may be linked to what lymphoma?
Burkitt’s
autoimmune hemolytic anemia treatment
steroids
Waldenstrom’s macroglobulinaemia, how to differentiate from MM?
- Lymphoplasmacytic cells (immature) in bone marrow
- Only IgM associated
- Organomegaly more common
- Hyperviscosity syndrome e.g. visual disturbance
- Cryoglobulinaemia (Raynaud’s)
- Less associated with hypercalcemia and kidney failure
Common Multiple myeloma gene translocation?
11;14
Transfusion threshold with RBCs (1)
- Target after transfusion (2)
70g/L (80 in ACS)
- Target after transfusion +20 (e.g 70-90 or 80-90 in ACS)
When is Prothrombin complex concentrate used usually?
Emergency reversal of anticoagulation in patients with either severe bleeding or a head injury with suspected intracerebral haemorrhage
Give examples of when Cryoprecipitate may be used?
(this is used in smaller amounts and contains contains concentrated Factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin)
- Clinically significant w/o major haemorrhage fibrinogen concentration < 1.5 g/L (prophylactic pre surgery <1 g/L)
- Disseminated intravascular coagulation, liver failure and hypofibrinogenaemia secondary to massive transfusion. It may also be used in an emergency situation for haemophiliacs (when specific factors not available) and in von Willebrand disease
Fresh frozen plasma (FFP) used when?
‘Clinically significant’ but without ‘major haemorrhage’ in patients with a prothrombin time (PT) ratio or activated partial thromboplastin time (APTT) ratio > 1.5
- Also prophylaxis pre major surgery w. heavy bleeding
At what platelet count is a platelet transfusion offered, when there is clinically significant bleeding
< 30 x 109
- higher (maximum < 100 x 109) for patients with severe bleeding
In what conditions are platelet transfusions contraindicated? (4)
- Chronic bone marrow failure
- Autoimmune thrombocytopenia
- Heparin-induced thrombocytopenia, or
- Thrombotic thrombocytopenic purpura.
What are the platelet targets pre-surgery? (depends on the surgery) (3)
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
> 100×109/L if surgery at critical site
