Haematology Flashcards
Transmission of which type of infection is most common following platelet transfusion?
Bacterial
Which drugs can reduce the risk of tumour lysis syndrome
Allopurinol or Rasburicase
Which haematological malignancy is most strongly associated with the mutation (t(9:22))
CML
(Philadelphia chromosome)
- Also found in 30% of ALL cases
How often should sickle cell patients receive the pneumococcal vaccine?
every 5 years
When does heparin induced thrombocytopenia typically occur
5-14 days after heparin exposure
Blood findings in DIC
Decreased Hb
Decreased Platelets
Decreased Fibrinogen
Increased APTT
Increased FDP
Which blood products have the highest risk of bacterial infection
Platelets
Transfusion of platelets is contraindicated in which conditions?
Chronic bone marrow failure
TTP
Autoimmune thrombocytopenia
Heparin induced thrombocytopenia
MOA of aspirin
irreversibly inhibits COX to reduce the production of thromboxane from arachidonic acid, therefore reducing platelet aggregation
MOA of Apixaban
Directly inhibits factor Xa preventing the formation of thrombin from prothrombin
Complications of polycythemia vera
AML
Myelofibrosis
Thrombotic events:
Stroke
Splenic infarct –> Ruptured spleen (rare)
Kidney stones (due to high uric acid)
Metabolic abnormalities in tumor lysis syndrome
Hyperkalemia
Hypophosphatemia
Hypocalcemia
Labatory findings in beta thalassemis major
Increased HbA2
Increased HbF
Absent HbA
Classic skull Xray findings in myeloma and difference from hyperparathyroidism and pagers disease
Patchy bone reabsorption
Rain-drop skull - random pattern of dark spots.
Hyperparathyroidism gives ‘Pepperpot skull’ (random dark and light spots)
Pagets disease gives a thickened vault + osteoporosis circumscripta.
Tear drop poikilocytes are seen in which condition
Myelofibrosis
First line treatment for ITP
Oral prednisolone
Metabolic differences between Myeloma, Bony metastasis, Pagets disease, Osteomalacia + Hyperparathyroidism
Myeloma = Normal ALP, Raised Calcium & Raised phosphate
Bony mets = Raised ALP, Calcium & Phosphate
Osteomalacia = Normal ALP, Decreased calcium, phosphate & Vit D.
Pagets disease = Isolated ALP rise
hyperparathyroid = Raised ALP, Raised calcium, Decreased phosphate
Management of non-haemolytic febrile transfusion reaction
Paracetamol
Treatment of neutropenic sepsis
Tazocin
Sideroblastic anemia investigation findings
Hypochromic microcytic anemia
Bloods = High ferritin, High Transferrin, normal iron
Basophilic stippling of RBCs
Causes of sideroblastic anaemia
Anti-TB meds
Myelodysplasia
Alcohol
Lead
Which type of Hodgkins lymphoma carries the best prognosis
Lymphocyte predominant
What type of transfusion reaction is more common in patients with IgA deficiency
Anaphylaxis
Which drugs need to be avoided in G6PD deficiency (due to risk of hemolysis)
Sulph drugs
Sulphonamide, Sulphonylureas (E.g Glipizide) + Sulfalazine
Causes of Microcytic anaemia
Thalassemia
Anemia of chronic disease
Iron deficiency anemia
Lead poisoning
Sideroblastic anemia
Causes of normocytic anemia
Acute blood loss
Aplastic anemia
Anemia of chronic disease
Haemolytic anemia
Hypothyroidism
Causes of microcytic anemia
Normoblastic = Reticulocytosis + Alcohol + Liver disease + Azathioprine
Megaloblastic = Pernicious or Folate deficiency
Which medication can cause Megaloblastic microcytic anemia
Methotrexate as it causes a folate deficiency
Causes of iron deficiency
Pregnancy
Iron losses - GI bleed, Cancer, Menorrhagia
Malabsorption - PPIs, Coeliac, Crohns
Blood findings in iron deficiency anemia
High TIBC + Low transferrin
Management of IDA
Refer for investigations if high risk (OGD/Colonoscopy).
Oral ferrous sulfate 200mg TDS
IV cosmofer if malabsorption (anaphylaxis risk)
Investigations for pernicious anemia
Bloods: Megaloblastic microcytic anemia
IF antibody
Gastric parietal cell antibody
Hereditary causes of haemolytic anemia
Hereditary spherocytosis
Hereditary elliptocytosis
G6PD deficiency
Thalassemia
Sickle cell
Acquired causes of haemolytic anemia
Warm/cold AHA
Alloimmune
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anemia
Prosthetic heart valves
Sx and Invx for spherocytosis
Sx = Jaundice + Gallstones + Splenomegaly + Aplastic crisis (during parvovirus) + Fhx
Invx;
- blood films shows spherocytes
- Diagnosis = EMA binding test
G6PD deficiency
X linked recessive mutation resulting in a defect in the G6PD enzyme. Makes RBCs more prone to oxidative stress.
Triggers of haemolysis in G6PD deficiency
Infection
Drugs - sulph drugs + antimalarials
Flava beans
Blood film findings in G6PD Deficiency
Heinz bodies, Bite cells + blister cells
Diagnosis of G6PD Deficiency
G6PD enzyme assay at presentation + 3 months after
Warm AHA
More common. Occurs at body temperature. Can be secondary too CLL.
Caused by IgG antibody
Features = extravascular haemolysis (splenomegaly)
Treatment of AHA
Steroids +/- Rituximab
Cold AHA
Occurs at cold temperatures (<10 degrees). Usually secondary to SLE/EBV/CMV/HIV/Lymphoma/Leukemia
Caused by IgM antibodies
Features = intravascular haemolysis + Raynaud’s
Paroxysmal nocturnal haemoglobinuria
Complement driven destruction of RBCs
Sx = red urine in the morning
Tx = Eculizumab (Inhibits C5)
Bone marrow transplant is curative
Causes of Microangiopathic haemolytic anemia
DIC
HUS
TTP
SLE
Cancer
A mutation on which chromosome causes sickle cell
Chromosome 11
What medication can be given to prevent the occurrence of sickle-cell crises
Hydroxycarbamide - increases production of HbF
Complications of sickle cell
Increased risk of infection
Stroke
Avascular necrosis of large joints
pulmonary HTN
Priapism
Acute chest syndrome
Sickle-cell crises
CKD
Splenic sequestration crisis presentation
Acutely enlarged + painful spleen
Severe anemia
Hypovolemia
Presentation of Aplastic crises (sickle cell)
Aplastic anemia
Commonly following slapped cheek/parvovirus
Beta-thalassemia cause
Recessive mutation on chromosome 11
Alpha thalassemia cause
Recessive mutation on chromosome 16
Thalassemia diagnosis
Hb Electrophoresis
Invx for sideroblastic anemia
FBC = microcytic hypo chromic anemia
Iron = raised ferritin + iron + transferrin
Blood film = basophilic stippling of RBCs
Causes of aplastic anemia
Idiopathic
Fanconis anemia
Cytotoxics, chloramphenicol, sulphur drugs, gold
Benzene
Parvovirus B19 & Hepatitis
Acute myeloid leukaemia
Most common adult acute leukemia
Raised WCC + Thrombocytopenia + reduced reticulocytes
Blood film shows Auer rods + blast cells
Acute Lymphoid leukaemia
Most common paediatric leukaemia (age 2-4yrs)
RF = Downs syndrome / Philadelphia chromosome
Chronic myeloid leukaemia
Massive splenomegaly!
RF = philadelphia chromosome
Increase in granulocytes at different stages + occasionally thrombocytosis
(likely to have raised everything)
% Blasts in blood/marrow >30%.
Tx = imatinib
CLL
Common in adults >55yrs
RF = TP53 Mutations / Trisomy 12
Lymphadenopathy main feature
Blood film = smudge/smear cells
Complications of CLL
Richter’s transformation / Warm AHA / Hypoglobulinemia (recurrent infections)
Hodgkins lymphoma
Characterised by presence of Reed-Sternberg cells
Classically presents with painful lymphadenopathy after drinking
Most common type of Hodgkins lymphoma
Nodular sclerosing
Types of non-hodgkins lymphoma
Diffuse large B cell - most common. Rapidly growing mass in pt >65yrs
Burkitts lymphoma = starry sky appearance. associated with EBV. Tumor lysis syndrome
MALT lymphoma - in stomach. associated with H.pylori
T cell lymphoma in intestines - Coeliac disease
Staging system for lymphoma
Ann Arbor
Features of myeloma
Calcium high
Renal failure
Anemia
Bone disease
Hyperviscosity / Infections
Blood film findings in myeloma
Rouleux formation
Urinary marker of myeloma
Bence jones proteins
Chemotherapy for myeloma
Bortezomib + thalidomide + dexamethasone
Features of polycythemia vera
Conjunctival plethora
Facial plethora (ruddy complex)
Splenomegaly
Raised Hb
Intense itch after hot water
JAK 2 mutation
Features of essential thrombocythemia
Raised platelets
Characteristic burning sensation in hands
Investigations for myeloproliferative disorder
Blood film = Tear drop poikilocytes
Bone marrow aspiration = Dry tap
JAK2, MPL & CALR gene testing
Immune Thrombocytopenia purpura (ITP)
Thrombocytopenia + normal bone marrow
Cause = autoantibodies against Glycoprotein Ib-V-IX complex
Treatment of ITP
Can observe in children
Prednisolone +/- IV immunoglobulin if acute
Presentation of TTP
Pentad: Microangiopathic haemolytic anemia + Purpura + Renal insufficiency + Neurological symptoms + Fever
Von Willebrand disease
Most common inherited bleeding disorder
Caused by autosomal dominant mutation resulting in vwf deficiency
Difference in presentation between Von-willebrand and haemophilia
VWD behaves like a platelet disorder so causes epistaxis + menorrhagia whereas haemophilia causes spontaneous bleeding such as hemarthrosis or intracerebral bleeds.
Also VWD can occur in females whereas haemophilia is X linked
Haemophilia A
Deficiency of factor VIII
90% of haemophilia
Haemophilia B
Deficiency of factor IX
Types of Thrombophilia
Inherited;
- prothrombin gene mutation
- Factor V leiden (most common)
- Protein C deficiency
- Protein S deficiency
- Antithrombin III deficiency
Acquired;
- Antiphospholipid syndrome
- COCP
Which type of inherited thrombophilia causes the greatest increased risk of VTE
Antithrombin III deficiency
Features or Antiphospholipid syndrome
Recurrent thrombosis + miscarriage
Bleomycin
MOA = Cytotoxic antibiotic. Works on G2 phase + mitosis
SE = Pulmonary fibrosis (lower zone)
Methotrexate
Antimetabolite - Folic acid antagonist.
Works by inhibiting dihydrofolate reductase.
SE = Myelosupression, Lung fibrosis, Liver damage.
Requires monitoring of LFTs, U&Es, FBC etc
Doxarubicin
Anthracycline
MOA = Inhibits topoisomerase II therefore preventing transcription
SE = Cardiomyopathy
Cisplatin
Platinum compound.
MOA = Creates cross-links between guanine molecules which blocks replication + transcription
Uses = mainly ovarian / lung cancer / colon cancer
SE = Ototoxicity + Nephrotoxicity
Cyclophosphamide
Aklylating agent
moa = prevents DNA replication + blocks DNA repair
Use = Leukemia / lymphoma
SE = Haemorrhagic cystitis
Vincristine / Vinblastine
Vinca alkaloids
MOA = binds to tubulin + inhibits microtubule formation during mitosis thus preventing cell division. (M phase arrest of mitosis)
SE = Peripheral neuropathy + myelosuppression
