Haematology Flashcards
What is Immune thrombocytopenia?
This is either an acute or chronic condition.
- Acute - follows an URTI
- IgG antibodies against GPIIb/IIIa
Symptoms:
- epitaxis
- menorrhagia
- petechiae bruising
- *normal spleen size
Labs:
- low platelets
- antibody screen positive
- Megakaryocytes seen on bone marrow
Treatment:
- prednisolone
- Immunoglobulin therapy
- Rituximab
Emergency:
- platelet transfusion
- Splenoectomy
What is:
Thrombotic Thrombocytopenic Purpura?
- TTP
A type of microangiopathic haemolytic anaemia.
- acquired or congenital (ADAMTIS13 deficiency, allowing vWBF to promote platelet aggregation)
*causes multisystem thrombotic occulsions due to activation of the platelets
Most common in females following an URTI
Symtpoms:
Pentad:
- Anaemia
- Fever
- thrombocytopenia
- renal failure
- CNS involement
Labs:
- reduced platelets
- LDH increased
- Haptoglobin decreased
- Clotting studies - normal
- Blood smear - schiztosites
**test for shigella toxins as this may be haemolytic uraemic syndrome caused by E.Coli 0157
Treatment:
Haematological emergancy:
- plasma infusion
- plasma exhange
- steroids
- rituximab
Beta thalassamias:
Point mutations on chromosome 11. defects in beta globulin chain.
Beta thalasemia trait:
carrier state with one chain missing.
- usually symptomatic
Beta thalasemia Mjor
both globulin chain defects.
- 1st year of life
- severe anaemia
- failure to thrive
- skull bossing (extra medullary haematopoesis)
- splenomegaly
Blood film:
- HbA2
HbF
*no HbA
Treatment:
promote healthy lifestyle
folate supplements
2-4 weekly tranfusions *Iron overload is a problem often causes endocrine issues (hypothyroidism, hypocalaemia)
- splenoectomy
What will 1 unit of packed RBCs equate to in blood volume?
1 unit = 350ml
1 unit = ~ 1g/L Hb in 70kg adult
How long a life span do platelets have in the bag?
5 days
What temperture must FFP be kept at?
how long does it take to thaw and when does it need to be used by?
-18Celius
20mins to thaw
2 hours
What drug can be used in sickle cell disease to help promote production of HbF?
Hydroxycarbamide
What drug can stimulate production of Von Williebrand in people who have Von Williebrand disease?
Desmopressin
What gene is associatted with Burkits lymphoma?
MYC gene with 8;14 translocation
List some causes of Haemolytic anaemias:
Acquired:
Autoimmune Haemolytic Anaemia:
- Warm IgG
- Cold IgM
Drug Induced
- RBCs autoantibodies
Microangio haemolytic anaemias:
- Haemolytic uraemic syndrome - E.Coli
- TTP - ADAMST13
Infection:
- Malaria
Paraoxysmal Notctual Haemoglobinuria:
Heridatiry:
Glucose - 6 - phosphate deficiency
Pyruvate deficiency
Heridatory spherocytosis
Sickle cell disease
Thalassaemias
What is Cryoprecipitate and when is it used?
Preperaed plasma contianign:
- fibrinogen
- von williebrand
- factor VIII
- Factor XIII
Medical emergancies for massive bleeding. Can be used in DIC
also used for haemophilae
What infective transmission may occur in blood transfusion and what steps have been put in place to reudce its spread?
vCJD
> 1999 donation have White cells removed
>1999 plasma sourced from abroad
>2004 recipitants of blood donation are excluded form giving blood
What anticoagulant can be used in pregnancy?
LMWH
If a male has explained Hb <110g/L what should be done?
Urgent endoscopy and colonoscopy
<2 weeks
Which DOAC can be used in the prevention of thromboembolism from AF?
Dabigatran
What are the risk factors for devleoping ALL?
Ionizing radiation
- especially during pregnancy
Down’s syndrome
Being young
- most common cancer in childhood
What is the classfication system used and how is the prognosis worked out in ALL?
Chromosomal and molecular analysis
Immuno-Phenotyping
What are some differentials for petechia bleeding?
*Leukaemia
*Vasculitis - Henock Scholien
*Microangio Haemylytic syndroes
- TTP
- E.Coli
*Menigitis
- N. menigitis
What investigations should be done in suspected leukaemia?
- FBC
- this should be done with 48 hours of suspected leukaemia.
*note young adults or children with ptechiae should be referred to hospital immediatley
- will usually see a high WCC (although they won’t be functioning WCs)
- Blood film
- looking at abnormal cells
- LDH
- this will show increased cellular turnover
- Bone Marrow Biopsy
- with phenotyping. this is definitive diagnosis
- Chest x-ray
- looking for mediastinal masses
- CT/ PET CT
- LP
- if suspected CNS involvement
What is the treatment of ALL?
Support:
- Blood transfusions
- IV fluids
Allopurinol - tumour lysis syndrome
**insert hickman line
Prophylatic antibotics and antifungals
Neutropenic regieme
- hygeine
- high calorie intake
- full barrier treatment
- daily checks
Chemotherpay + steroids
- remission induction
- consolidation
- Maintance
Marrow transplant
- young adults
What are some symptoms seen in AML that help to distinguish it?
Anaemia
Bruising
Infections
Symptoms that help differentiate:
- DIC
- Gum hypertrophy / bleeding
*these infilatrate
What are the symptoms of CML?
Mostly chronic insideous symptoms.
weight loss
tiredness
fever
night sweats
Splenic enlargemnt
- abdominal discomfort
**often the spleen is massive in CML
What is the natural history of CML?
- *Chronic phase**
- last years
- *Accelerated phase:**
- increase in symptoms
- increase in splenic size
- *Blast Crisis:**
- AML tranformation
- features of leukaemia. death is likely
What is the most common leukaemia over all? and how does it often present?
CLL
Often CLL is asymptomatic and is found out by incidental finding.
Patient may be prone to:
- infections
- bleeding
- large rubbery non tender lymph nodes.
What are some complications of CLL?
- Autoimmune haemolysis
- over production of B cells leads to production of antibodies against RBCs.
* - Warm IgG antibody anaemia* - Hypogammaglobulinaemia
- Marrow failure
- Richter’s transformation
What is the natural history of CLL?
1/3 never progress
- or even regress
1/3 progress slowly
1/3 Progress rapidly into aggressive lymphoma called:
- RIchter’s lymhoma
*majority die from infection or transformation
When is a blood transfusion indicated pre-operatively?
if a patient is unable to take oral Iron, then how should they be managed pre-operatively?
Hb: <8g/dL
IV Iron - ferric carboxymaltase 1g/ weekly
What is the most common type of Hodgkins lymphoma?
Nodular sclerosing
Any supcision of leukaemia in a young person should be managed how?
FBC within 48hours
What is the most important factor in deciding whether cyroprecipitate should be given?
Low fibrinogen level
What are the symptoms of lead poisoning?
Muscle weakness
Neuropychiatric symptoms
Anaemia
Blue gums
- mainly seen in children
Adbominal pain/ constipation
Outwith a high MVC, what other feature may pointi n the direction of megoblastic anaemia?
Hyper - segmented neutrophil polymorphs
Glucose 6 phosphate defiency is inherited by which means?
X linked
What can cause haemolysis Glucose - 6 - phosphate dehydrogenase deficiency?
Infection
Fava beans
Drugs - nitrofurantoin
Acidosis
What disease is associated with stary sky appearance on H&E stain
Burkits lymphoma
Why is parvovirus worrying in sicklecell patients?
It can cause aplastic crisis, which is a poor response of the bone marrow to respond to the drop in Hb
What is the blood tranfusion cut off for patients without ACS and those who present with ACS?
Non ACS - <7g/dL
ACS - <8g/dL
If a patient presents with a painful swollen leg and meets the criteria of Well Score >2, what should the next course of action be?
Carry out leg ultrasound scan within 4 hours.
if the scan is negative D Dimers should be done. If these are positive the LMWH should be started
What type of infection is most likely to transmitted via platelets?
Bacterial
- stored at room temperture making them more likely to devleop bacterial infections
What investigation should be done to estbalish polycaemthemia ruba vera?
JAK 2 mutation screen
At what level should platelets be transfused?
<30
What is the most common thrombophilia inherited condition?
Factor V Leiden
- Activated protein C resistance
prothrombin Gene mutation is second most common
What is required to diagnose tumour lysis syndrome?
to diagnose tumour lysis syndrome you need both a clinical and laboratory result.
Lab results:
- *Urate**
- high
- *K+
- ** High
- *Phosphate**
- high
- *Ca2+**
- low
Clinically:
- *Cardiac arrythmias**
- K+ ^^
- *Rasied creatinine**
- renal failure
Seizure
What cancer is CLL most likely to transform into?
Richter’s lymphoma
*not AML
Name a medication used in cancer than increases risk of VTE?
Tamoxifen
Which Drug should be avoided in people with G6PD deficiency?
anti- malarial prophylaxis
- primaquine
Which haematological cancer often causes tumour lysis syndrome? and what are the symptoms of tumour lysis syndrome?
Burkitt’s lymphoma
Muscle cramps, confusion
release of:
- uric acid
- phosphate
- K+
What is the most common type of lymphoma?
Diffuse large B cell
Name some causes of Thrombocytosis:
Bleeding
infection
Malignancy
Chronic Inflammation
Trauma
Iron deficiency
Post surgery
Name some causes of thrombocytopenia:
Chemotherapy
Radiation
Bone marrow failire
- leukaemia
- myeloma
- Myelofibrosis
Autoimmune - ITP
DIC
TTP
Splenomegaly
Dilutional effect
- massive transfusion
What are the investigations that should be done into sickle cell disease?
- *FBC**
- low stable Hb
- *Blood films**
- show’s evidence of hyposplenism
Sickle Solubility test
- *Hb Electrophresis
- ** Hb SS present
- no HbA will be present if sickle disease
What is the general management of sickle cell:
Prevention of triggering factors of sickling
- *During attack:**
- High O2
- opoid medication
- Transfusion
Propholyatic antibiotics
- *Hydroxycarbamide**
- increase HbF
Stem cell transplant
What may cause thrombocytosis:
Splenectomy
Infection
Essential thrombocytosis
Inflammatory diseases
- RA
- IBD
Malignancy
recent surgery
If a young person presents with signs of leukamia, when should they be referred to a specialist?
Immediately. This is the most urgent referral
From the different types of transfusions, which is most likely to pass on a bacterial infection?
Platelets
What is a metabolic risk of giving high amounts of RBCs?
Hyperkalamia
What is the universal donor of FFP?
AB
**remember it is FFP that will contain antibodies towards the RBCs.
thus AB will have no antibody already produced against AB on the patient
What are some causes to B12 deficiency?
Percinous anaemia
- intrinsic factor destruction
Reduced Gastric acid
- PPIs
Terminal ileum disruption
- Crohn’s disease
Reduced intake
- Vegan diet
What labatory findings may be seen with reduced vitamin B12?
Increased homocystiene
>MVC
Pancytopenia
Hypersegmented neurtrophils
What is the most common type of bleeding disoder, how is it transmitted, what will labatory results show and how is it treated?
Von Williebrand disease
Autosomonial dominant
Labs:
- prolonged bleeding time
- Prolonged aPTT
- Normal platelet count
- light transission agrregratory will be low
Treatment:
- desmopressin
- Transmexic acid
- factor VIII
What is the diagnostic criteria for Myeloma?
and how is it treated?
Diagnostic criteria:
*>10% blast cells
*Evidence of End organ damage (CRAB symptoms)
*Evidence of paraprotein/ bence jones proteins
Treatment:
Cancer:
- chemo- induction therapy
- stem cell transplant
Bones:
- analgesia
- Bisphosphonates
- orthopedic procedures (vertoplasty)
- localised radiotherpy
Anaemia:
- EPO
- Blood transfusion
Infections:
- Immunoglobulins
- antibiotics
How is HUS investigaed and treated?
Investigated:
- FBC (Anaemia, Thrombocytothenia)
- Blood film (fragmented RBCs)
- U&Es (AKI)
- Stool culture
Treatment:
- Fluids
- Plasma exhange
- Dialysis
What are the bedside procedures for safe blood transfusion?
Taking blood for pre-transfusion testing
- positvely idenitify the patient
- Lable the sample tube and lable form at bedside
*do not write any forms or lables in advance
Administering Blood:
- Positively Identify Patient at beside
- Ensure identification matches the blood pack
- Check ABO and RhD groups
- Check the bag of blood (damage)
Record keeping:
- record in patients notes when giving etc
Observations:
- Blood pressure, temp and pulse mesured 15mins
What are the types of Haemopoietic stem cell transplantation?
Allogenic
- HLA matched
Autogenic
- self stem cells
Umbilical cord
What investigations should be done into DIC and how is it treated?
Investgiation:
- FBC - Platelets
- Coagulation studies - PT high, aPTT, Fibrinogen loss
- D-dimers (increased)
Blood film - Schistocyte
Treatment:
- treat cause
- Cryopercipitate or FFP
If a patient has a unrpovoked P.E or DVT, following initial management, what additional tests should be done afterwards?
Cancer screening as this is a big risk factor for a clot. Investgiatiosn include:
- Physcial examination
- CXR
- Blood test
- Urine Analysis
*>40 it is recommened to go for a CT scan
What drug is used in the treatment of a DVT?
LMWH
What non- reactive (Haemolytic, alergic) side effects can come about from blood transfusion?
Infection
- vCJD
- HIV
- Hep B
Tranfusion Associated Circulatory overload
- Hyertensive
- Pulmonary oedema
**HTN differentiates it from Acute Lung injury
What test should be performed into polycythemia Ruba vera?
FBC
JAK2 Mutation
Serum Ferritin
Renal and LFTs
How is Haemophilia A treated?
what are some complications?
Desmopressin - stimulated Von Williebrand Factor and increases levels of Factor VIII.
In needed times recombinant Factor VIII can be used.
Complications:
- Desmopressin - Fluid overload/ hyponatrarmia
Factor recominant VIII - Autoimmune auto-antibodies towards the factor.
Infection risk vCJD
Which blood group physiologically has lower amounts of Von Williebrand factor?
Group O.
the antigens A and B are expressed on von williebrand reducing it from being proteolysed
What are the types of MDS?
Primary
- idiopathy
Secondary
- Radioation
- chemotherpay
What are the reversal agents for dabigatran?
Idarucizumab
In haemolytic anaemia - what would you expect to find in the urine?
Increased urobilinogen
- due to increased reabsorption from the gut
What is a potential finding on the blood smear in haemolytic anaemia? and why is this?
leucoerythroblastic blood film
- immature red and white blood cells
this occurs because there can be such a stimulus to produce new cells that not only are the RBCs increased but so is the granulocytes. - both of which can be immature as they are pumped out into the circulation.
List some causes of intravacular haemolysis, and what may be seen in the urine?
ABO incompatibility
Malaria
Clostridum perfringens
Trauma - heart vales
oxidative stres
- drugs. dapsone
- *Haemoglobinuria**
- can present as black urine (as seen in fulminant malaria)
- *Haemosiderinuria**
- in smaller amoutns of free haemoglobin the blood, the tubular cells can absorb it and store it. when these slough off the release their contents.
Name a non-vascular related complication of increased Haemolytic anaemia:
Pigmented gallstones
foalte deficiency
Management of splenectomy:
Pneumococcal vaccination every 5 years.
life long penicillin V propholyaxis
Education
Card to alert professionals to the overwhelming sepsis risk
When is the indirect coomb’s test carried out?
Prior to blood transfusion
Pregnancy - Rhesus status to assess if maternal blood as developed antibodies against the fetus blood
How is IgG haemolytic anaemia diagnosed and what is the treatment?
Direct Coomb’s test
Remove the causative agent
Steroids
Splenoectomy or Azathiprine (not preferred in children)
What typical abnormal lab results do you get in tumour lysis syndrome? and what drug is used propholyatic for it?
Hyperkalamia
hyperphosphotaemia
Hypocalcaemia
Allopurinol
What is the management of a sickling crisis, and what is the management of long term sickle cell disease?
Crisis:
- oxygen
- IV fluids
- analgesia
- Tranfusions
- especially if aplastic anaemia is present
- Antibiotics
Chronic:
- Folic Acid
- Pneumoccoal vaccination
- Seasonal Influenza vaccination
- hydroxycarbamide
- Stem cell transplant
What is the most important immunological compatibility needed for platelet/ FFP and Cryoprecipitate use?
RhD compatibility
ABO is not as strictly needed
What are the broad classes of pancytopenia?
Bone Marrow Failure:
- Aplastic anaemia
- Idiopathic
- Drugs (chemo, radiation)
Infiltrative causes:
- Leukaemia
- Myeloma
- lymphoma
- MDS
Infective causes:
- HIV
- EBV
- CMV
Ineffective causes:
- B12
- Folate
Peripheral Blood Pooling
- Hypersplenism
- SLE
What is the bone marrow of leukaemia usually like?
Hypercellular with excessive blast formation
What is the general treatment regieme of Acute leukamia
Specific treatment (chemotherapy regiemes) \*RCHOP is usally used for ALL - need prior preperation for this.
Specific treatment involves:
- induction therapy
- Consolidation therapy
- Remission maintaince
*this can relaspe at any stage.
Stem cell transplant may be used following this.
(usually reserved for <60 years)
Outwith FBC, blood smear and immunophenotyping, what other Investigations should be done into CLL?
Direct Coombs test
- risk of autoimmune haemolytic
Reticulocyte count
*typically Bone marrow is not needed for diagnosis
What are the high grade lymphomas? and low grade:
High grade
Burkitt’s
Diffuse large B cell
Mantle
High Grade:
Small lymphocytic
Follicular
Marginal zone
What investigations are done into Hodgekin’s lymphoma and how is it treated?
- *Bloods**
- FBC (normocytic anaemia)
- LFTs (infiltration)
- LDH (raised)
- ESR (rasied)
- *CXR**
- mediastinal mass
Biopsy:
- done under radiological guiadance/ surgical removal of lymph node
Treatment:
1A - 2- radiation
>2 - Chemotherapy:
- ABVD (Doxirubicin, bleomycin, vinblastine, Dacarbazine)
Review:
every 3 months for 1-2 years
6 months 3-5 years
Annual influenza
Breast screening programme
What are the investigations and treatments for Non-hodgekins Lymphoma?
- *Bloods**
- FBC (normocytic anaemia)
- LFTs (infiltration)
- LDH (raised)
- ESR (rasied)
- HIV testing
- *CXR**
- mediastinal mass
- *Biopsy:**
- done under radiological guiadance/ surgical removal of lymph node
- Important to establish cytogenetics for B or T cell
Bone marrow Aspiration + Biopsy (trephine biopsy)
Treatment:
Low grade:
- radiotherapy
- rituxumab
High grade:
- RCHOP
What is done during a bone marrow biopsy and where are they typically done?
Bone marrow aspiration
- cellular film that can be analysed under microscope
Bone Marrow aspiration - Trephine
- looks at archietecture, infilatration and bone marrow cellularity
Procedure on: Posterior of iliac Crest
What score can be used to predict prognosis in neutropenic sepsis?
Multinational assessment for supportive care in cancer
- MASCC
>21 is good
What investigations should be done into Macrocytic anaemia?
FBC
Blood film
- macrocytic, hyerpsegemented neutrophils
B12/ folate levels
LFTs
- can cause macrocytic anaemia
Bone marrow
What is the severe complication of Vitamin B12 deficiency?
What other “classical” neurological signs may be seen?
Subacute combined degeneration of spinal cord
- dorsal leminicus pathway degereration
- corticospinal tract defects.
Classical signs:
- up going plantars (UMN)
- absent knee jerk (LMN)
- Absent Achiels jerk (LMN)
What are the clinical findings in ALL?
Petechia brusing
Epitaxis
Hepatomegaly
Splenomegaly
Testicular swelling
Bloods:
- neutropenia
- Anaemia
- Thrombocytopenia
What affect can Iron Deficiency and Megoblastic anaemia have on Hb1Ac scores?
It can exaggerate them - over estimating
What are some causes of Low vitamin B12?
Dietary
Pernicious anaemia
Small bowel pathology
- Ileum segmentation
What other cells outwith RBCs will demonstrate Macrocytosis in B12 defiency?
All proliferating cells will.
ones of obvious appearance are:
- buccal mucosa
- tongue
- small intestines
- cervix
- vagina
What are the clinical features and signs of megablastic anaemia?
Symptoms:
- malaise
- breathlessness
- Poor memory
- paraesthesiae
Signs:
- smooth large beefy tongue
- Skin pigmentation
- Pyrexia
Why is the LDH often markedely increased in megoblastic anaemia?
Cells during development in the bone marrow arrest and die, which induces a hypercellular marrow. This dying releases LDH and bilirubin.
*there will be no rise in reticulocytes like often seen in other haemolytic anaemias which also have similar biochemical findings
If someone is seen to have a low folate level and you decide to supplement their folate, what else must you give?
Vitamin B12 because if this is also low it can worsen subacute combined spinal cord degeneration if
