Haematology Flashcards
What is Waldenstroms Macroglobulinaemia
IgM paraprotein !!!
Lymphoplasmacytoid Malignancy
How does Waldenstroms Macroglobulinaemia Present?
Systemic Upset - weight loss lethargy Hepatosplenomegally Raynauds Hypercoag state - Strokes etc NO lytic lesions
Waldenstroms vs Multiple Myeloma
Multiple Myeloma is IgG Bence Jones and presence of Lytic Bone Lesions
At what Hg level can you offer a transfusion in an otherwise healthy patient?
70
What target Hg is aimed for post transfusion in a healthy patient?
70-90
In a patient with ACS what level Hg is needed for a transfusion?
80
What is the Hg target in a patient with ACS receiving a transfusion?
80-100
What can be used to reveres Dabigatran?
Indarocuzimab
Side Effects of Heparin
Heparin Induced Thrombocytopenia - Bruise like rash itchy swollen
If someone cant tolerate Heparin what should be used?
DOAC
Commonest Hodgkins Lymphoma
Nodular Sclerosing
Good Prognosis
Lacunar Cells
Best Prognosis in Hodgkins Lymphoma.
Lymphocyte Predominant
Worst Prognosis in Hodgkins Lymphoma
Lymphocyte Depleted
Any tumour with B cell symptoms - fever night sweats weight loss etc
> 65 plus new iron deficiency anaemia
Referral for Colonoscopy +/- endoscopy
What does someone require post splenectomy?
Antibiotic prophylaxis - Penicillin V or Erithromycin
Pneumococcal Vaccine - Every 5 years
Influenza - Once a year
Haemophilus Influenza and Men C - Once off
Irradiated Blood Products protects against what?
Graft Versus Host
In immune Thrombocytopaenic Purpura what medication can be used in the treatment?
Oral Prednisilone
If you need quick results for example to allow a invasive surgery to occur. Immunoglobulin can be used as it has a quicker response.
INR >8 + Major Bleed
or Major bleed regardless of INR
Stop Warfarin
IV Vitamin K
Infusion of prothrombin complex or FFP
INR >8 + Minor Bleeding
Stop Warfarin
IV vitamin K
Repeat INR in 24hrs - <5.0 restart
INR >8
Stop warfarin Vitamin K oral
Check INR in 24 hours - <5.0 start again
INR 5.0 - 8.0
Withold 1 0r 2 doses and reduce maintenance dose
INR 5.0 - 8.0 + Minor Bleeding
Stop Warfarin + IV Vitamin K
Check INR restart after 24 hours in <5.0
Who is the universal Plasma donor?
AB blood donors as their plasma has no circulating antibodies
Beta thalassaemia major - blood results
Absent HbA - normal Adult Haemoglobin
Increased HbA2 and HbF - fetal haemaglobin and delta chain haemaglobin
ITP management
No treatment - platelets >30 and asymptomatic
Oral steroids - Symptomatic or platelets <30
Immunoglobulins can be used in more severe cases
resistant - splenectomy
Platelets only given in severe bleeding
What is elevated in tumour lysis syndrome?
Products of cell destruction
K+
PO4
Urea
Low Hb, MCV , MCH
Microcytic hypochromic
Target Cells
Extravascular haemolysis - jaundice and haemochromatosis
HbH disease
Alpha Thalassaemia 3 genes missing
Target Cells
Increased ferritin, Fe, Hbf and HbA2
Extravascular haemolysis - jaundice and haemochromatosis
Beta Thalassaemia
Describe the types of beta thalasaemia
The genes can be entirely deleted or altered so they are left effective.
Minor - 1 deleted gene
Intermedia - 2 genes affected
Major - 2 gene deleted
What is sideroblastic anaemia
Inability to properly convert Fe2+ into Haem
Sideroblastic anaemia - causes
Congenital - X linked ALAS2 mutation
Aquired - alcohol excess, deficiency in Vitamin B6, Lead poisoning
Pappenheimer bodies Basophilic stippling Haemochromatosis symptoms MCV is normal or low Increased Fe2+, Ferritin Decreased TIBC
Sideroblastic anaemia
Decreased serum iron and transferrin
Normal TIBC
Increased ferritin
Anaemia of chronic disease
Heinz body and bite cells
Decreased RBC and Haptoglobulin
Increased LDH Reticulocytes Bilirubin
Negative Coombs test
G6PD deficiency
Intravascular haemolysis due to oxidative stress
Common oxidative stressors in G6PD deficiency
Fava beans Aspirin NSAIDs Parvovirus Sulfonamides
What is the definitive diagnostic test in G6PD deficiency ?
Enzyme Assay
G6PD deficiency - demographic
X linked autosomal recessive disease - men
African and Mediterranean origin
Autoimmune Haemolytic Anaemia - Types
Warm - IgG extravascular, occurs at body temperature, mainly idiopathic
Cold - IgM extravascular occasionally intravascular (raynauds), occurs at cold temperatures
Direct Coombs test results in Haemolytic anaemia
Warm - IgG and C3D
Cold - C3D
Common causes of warm haemolytic anaemia
Viral SLE Lymphoma Leukamia Beta lactam antibiotics
Normocytic anaemia
Raised reticulocytes
Positive Coombs test
Haemolytic anaemia
Elevated 2,3 BGP
Negative Coombs test
Spiky dehydrated cells -echinocytes
Mainly extravascular haemolysis - raised bilirubin + Fe
Some intravascular - decreased haptoglobulin
Pyruvate Kinase deficiency
Extravascular haemolysis
Spherocytes
Reticulocytes
Hereditary Spherocytosis
What is use to diagnose Hereditary Spherocytosis
Osmotic fragility test
Hereditary Spherocytosis inheritance
75% AD
25% AR
Hodgkins Lymphoma - treatment
Adriamycin + Bleomycin + Vinblastine + Dacarbazine ABVD 2-6 cycles
+ Radiotherapy
Painless lymphadenopathy
Alcohol causes pain
Reed Sternberg Cells
Hodgkins Lymphoma
Non Hodgkins Lymphoma - Treatment
Rituximab - CD 20 Cyclophosphamides Hydroxydaunorubicis Oncovin Prednisolone
Nocturnal Haemoglobinuria
Phosphatidylinosite Glycan A defect
Symptoms of Nocturnal Haemoglobinuria
Anaemia Venous Thromboembolism Oesophageal spasm Erectile dysfunction Dark urine especially in the morning
Treatment for Paroxysmal Nocturnal Haemoglobinuria
Eculizumab
Sickle Cell Admission Criteria
Everyone is admitted apart from.
Well adult + mild/moderate pain + Temp <38*c
Well child + mild/moderate pain + No temp
Transfusion Reactions
Fever + Chills
Non haemolytic febrile reaction
Slow stop transfusion + Paracetamol
Transfusion Reactions
Pruritus + Urticaria
Minor allergic reaction
Stop + Antihistamine
Transfusion Reactions
Fever Abdominal Pain Hypotension
Acute Haemolytic Reaction
Stop + Confirm patient + Bloods to labs + Coombs test
Fluid Resus
Transfusion Reactions
Hypoxia + Infiltrates on chest xray
Acute Lung Injury
Stop + Supportive therapy
Transfusion Reactions
Pulmonary Oedema Hypertension
Circulatory Overload
Slow/Stop
Loop Diuretics
Common causes of aplastic anaemia?
Autoimmune destruction is commonest
Indomethacin, Propythiouracil, Chloramphenicol
EBV HIV
Common signs of Aplastic anaemia
Pancytopenia
Bone Marrow dry tap
Fanconis anaemia
Congenital aplastic anaemia
Short, microcephaly, cafe au lait, absent or small thumbs
If only RBC are absent or low but Platelets and WBC are normal what can be a cause?
Diamond Blackfan Anaemia
ITP - cause
IgG binds to GPIIb/IIIa which then allows the spleen to destroy platelets
ITP - Types
Acute - 2 weeks post virus - spontaneous resolution - generally children
Chronic - women of reproductive age - idiopathic or Hep C HIV Lupus
ITP - Diagnosis
Diagnosis of exclusion USS for splenomegaly FBC HIV Hep C screen
Thrombolitic Thrombocytopenic Purpura - presentation
Reduced platelets - bleeds, purpura
Neurological issues - confusion, speech, coma
TTP - cause
Von Willenbrand factor isn’t broken down so micrclots develop everywhere.
Aquired - autoimmune
Congenital - Autosomal recessive from birth
TTP - Treatment
Dialysis if AKI
Plasmapheresis or Rituximab
Von Willenbrand Disease - Cause
Reduced platelet adhesion +
VWF usually protects VIII from protein C and S
= Bleeds
Von Willenbrand Disease - Treatment
Minor bleed = Desmopressin analogues
Major Bleeds = Exogenus VWF and Factor VIII
Factor V leiden
Commonest Hyper coagulable state
Hodgkins lymphoma - Presentation
Nodal Disease
Painless cervical lymphadenopathy
Hodgkins Lymphoma - Cells and Immunology
B cells
CD15 and CD30
‘Owl eyes’ - reed-sternberg cells
Hodgkins Lymphoma - Nodular Lymphocytic predominant
Generally affects men
CD20 CD45
Lobulated popcorn cells
Rituximab can be used
Diagnosis of lymphomas
CT - staging
Lymph node biopsy
If there is mediastinal adenopathy what lymphoma is it likely to be?
Nodular Sclerosing Hodgkins Lymphoma
Non Hodgkins Lymphoma - Presentation
Nodal + Extranodal disease - hepatosplenomegally, mucosal tissues
Whats the commonest Non Hodgkins cell lymphoma ?
Diffuse Large B cel
Agressive course
What non Hodgkins cell lymphoma is considered indolent?
Follicular
Burkitt Lymphoma - histology
Starry Sky appearance
Burkitt Lymphoma - presentation
African - lymphoma affecting Jaw
European - Ileocaecal junction
Burkitt lymphoma - causes
HIV
EBV
Commonest T cell lymphoma
Adult T cell lymphoma
Caused by human T lymphotropic virus
Is MALT - Hodgkins or non hodgkins?
Non Hodgkins
CML - Immunology and cytology
Philadelphia Chromosome t(9,22)
BCR ABL - tyrosine kinase
CLL - Immunology and cytology
CD23 CD19 CD5
Smudge cells on blood film
What can be used to reverse a bleed in a patient using Rivaroxaban or Apixiban?
Andexanit Alfa
What can be used to reverse a bleed in a patient using heparin?
Protamine Sulfate
Multiple myeloma on skull xray
Raindrop skull
What is the commonest inherited bleeding disorder?
Von Willenbrand disease
Autosomal Dominant Disorder
Acts like a platelet disorder - less haemarthrosis
Sickle Cell Patient -
Dysopnoea Chest Pain Pulmonary Inflitrates on X-Ray
Acute Chest Syndrome # cause of death after childhood
Acute Chest syndrome management
Pain Relief
Respiratory support
Antibiotics
Transfusion
Sickle Cell -
Parvovirus infection
Reduced Hb and reticulocytes
Bone marrow suppression
Aplastic Crisis
Sickle Cell-
Worsening anaemia
Increased Reticulocyte
Hepatosplenomegally
Sequestration crisis
Sickle Cell Painful vasoocclusive Dehydrated Infection Deoxygenated
Thrombotic crisis
Recent heparin use
Prothrobotic state
+ve for heparin platelet factor 4 complex
Heparin induced thrombocytopenia
How long should a non urgent RBC transfusion take place over?
90-120 minutes
What affect can a packed RBC infusion have on a ECG?
Increased K+ leading to ECG changes related to this
Chemotherapy used in AML
Anthracycline
Cytarabin
What genetic conditions put you at an increased risk of acute leukaemia
Down syndrome
NF1
Klineflelters
Fanconis anaemia
What is one of the earliest signs in myelofibrosis
Isolated neutropenia
As spleen and liver can take up the slack in RBC and platelet production
Platelet transfusion
Theshold is <10
Unless bleeding or surgery is planned
Contraindications for a platelet transfusion
Autoimmune Thrombocytopenia
Heparin induced thrombocytopenia
TTP
Chronic Bone marrow failure - as body develops antibodies as platelets so rare
In a CLL patient presenting with acute onset B cell symptoms what is likely to have happened?
Richters transformation - non hodgkins lymphoma with a very aggressive course
Scott Coutts
Smile King
Elevated methaemoglobinaemia
Hypoxia not responding to oxygen therapy
Amyl Nitrate
Poppers usage
In post thrombotic syndrome what is the management?
Compression stockings
Polcythaemia rubra Vera - first line investigation
JAK2 mutation
Blood film
Increased Hb +/- neutrophil and platelet Pruritic after bath Splenomegaly Bleeds low ESR
PRV
EPO infusion side effects
Hypertensive encephalopathy Bone aches Flu like symptoms Urticaria Pure red cell aplasia Thrombosis Iron deficiency
Chemotherapy in CLL
Fludarabine
Cyclophosphamide
Rituximab
Management of antiphospholipid syndrome
Primary prevention - Aspirin
Secondary Prevention - Lifelong warfarin IN 2-3
- recurrent despite treatment - Aspirin + INR 3-4
- arterial thrombosis - INR 2-3
Only anticoagulant safe in pregnancy.
LMWH
If someone has an IgA deficiency what transfusion complication are they most at risk of?
Anaphylactic reaction
In a suspected G6PD defficiency when should enzyme assays take place?
At time or presentation
3 months
If someones INR is found to be below 2 what should be initiated?
LMWH cover and increase maintenance dose of warfarin
Age range for immediate same day referral for investigation if unexplained hepatosplenomegaly or petechia?
0-24 years old
If you have a Iron deficiency anaemia Iron study but the FBC shows an increased RDW on a background of crohns or coeliac what should you consider?
A mixed anaemia picture
RDW means there is an increased variation in size of RBC
Management of warm haemolytic anaemia
Steroids
+/- rituximab
If someone has a minor allergic reaction to a blood transfusion how is this managed?
Temporarily stop infusion
Antihistamine
Temperature rise shouldn’t be more than 2 degrees
What is the commonest primary antibody deficiency? And how does it present?
Selective IgA deficiency
Recurrent URTI and sinus infections
Linked to coeliac disease
Risk of severe anaphylaxis post blood transfusion
Recurrent Pneumonia and abscess
Staph Aureus and Fungal infections
Chronic Granulomatous Disease - Neutrophil disorder
Recurrent bacterial infections
No pus at sight of infection or wound
Desloughing of umbilical cord was delayed
Leucocyte adhesion
Child
Partial albinism
Peripheral neuropathy
Recurrent bacterial infections
Chediak Higashi syndrome - neutrophil deficiency
Hypogammaglobulinaemia
Risk of Autoimmune disorders
Risk of Lymphoma
Common variable immunodeficiency
X linked recessive condition
No B cells
Reduced immunoglobulins
Bruton X linked
Congenital heart disease - tetralogy of falot Learning difficulties Hypocalcaemia recurrent viral an fungal infections Cleft palate
DiGeorge syndrome - T cell deficiency
22q11.2 deletion
No thymus
Commonest x linked deficiency
Increased bacterial fungal and viral infections
Severe combined immunodeficiency
Stem cell is required
LMWH vs unfractionated heparin
LMWH - Antithrombin III and Xa inhibitor
- Monitored with Anti Xa assay - not routinely needed during surgery
- Prolonged duration - protamine sulphate isn’t as affective
- First line VTE and ACS
- Less risk of HIT and osteoporosis
Unfractionated Heparin - Antithrombin III Xa IXa XI and XIIa inhibitor
- Monitored with APTT required if used during surgery.
- Shorter duration of action - preferred if increased bleed risk and fully reversible.
When would you suspect neutropenic sepsis and how is this managed?
Cause of neutropenia identified + Temperature >38 + RR >20 + Presumed or confirmed infection
Give Piperacillin + Tazobactan (Tazocin) and send blood cultures - don’t wait for results to initiate sepsis 6.
Management of Polycythaemia Rubra Vera
Aspirin and venesection - first line
Hydroxyurea
Ruxotinib - JAK2 inhibitor
How long should a blood transfusion be transfused if the patient has HF?
3 hours
Inheritance of haemophilia
X linked
Fever Neurological symptoms Thrombocytopenia Haemolytic anaemia Renal failure
TTP
Auer rods
AML
Dabigatran MOA
Direct thrombin inhibitor
Apixiban or rivoroxaban
Direct Xa inhibitor
Ann Arbor staging for lymphoma
Grade 1 = Single nodal group
Grade 2 = 2 or more nodal groups
Grade 3 = Nodal groups on both sides of the diaphragm
Grade 4 = Extra lymphatic organs involved
A = Asymptomatic B =B symptoms X = bulky nodes S = splenic involvement E = extra nodal
