Haematology Flashcards
What are reticulocytes?
Immature RBCs
What makes platelets?
Megakaryocytes
Where do B cells mature?
The bone marrow
Where do T cells mature?
The thymus
What is anisocytosis and what is it a feature of?
Anisocytosis is variation in the size of RBCs. Myelodysplasic syndrome
When are target cells seen on blood film?
In iron deficiency anaemia and post-splenectomy
When are heinz bodies seen on blood film?
G6PD and alpha thalassaemia
When are lots of reticulocytes seen on blood film?
Haemolytic anaemia
What are schictocytes?
fragments of RBCs
When are smudge cells seen on blood film?
In CLL
what are the 5 common causes of microcytic anaemia?
TAILS: T halassaemia A naemia of chronic disease I ron deficiency L ead poisoning S ideroblastic anaemia
What are the 5 common causes of normocytic anaemia?
3 As, 2Hs Acute blood loss Anaemia of chronic disease Aplastic anaemia Haemolytic anaemia Hypothyroidism
What are the causes of macrocytic anaemia?
Megaloblastic (due to deficiency of folate or B12)
Alcohol
Azathrioprine
Liver disease
What is Pica?
A symptom seen in iron deficiency anaemia. It is a dietary craving for things such as dirt
What causes pernicious anaemia?
Lack of vitamin B12
Where is intrinsic factor produced and what is its function?
Parietal cells in the gastric mucosa. It is essential for the absorption of vitamin B12
Why does pernicious anaemia cause a lack of vitamin B12?
It is an autoimmune condition where antibodies form against parietal cells or intrinsic factors
What are the symptoms of pernicious anaemia?
Peripheral neuropathy
Loss of vibration or proprioception
Visual changes
Mood or cognitive changes
How do you test for pernicious anaemia?
Intrinsic factor antibody
Gastric parietal cell antibody
How is pernicious anaemia treated?
Cyanocobalamin orally or IM hydroxycobalamin
What is the protocol of there is folate deficiency alongside B12 deficiency?
Treat B12 first. Treating B12 deficiency with folate too can lead to subacute combined degeneration of the cord
Name some inherited haemolytic anaemias
Hereditary spherocytosis Hereditary elliptocytosis Thalassaemia Sickle cell anaemia G6PD deficiency
What are the 3 features of haemolytic anaemia?
Anaemia
Jaundice
Splenomegaly
What investigations should be done if haemolytic anaemia is suspected?
FBC (normocytic anaemia)
Blood film (shows schistocytes)
Direct Coombs test (positive in haemolytic anaemia)
What inheritance pattern does hereditary spherocytosis have?
Autosomal dominant
How does hereditary spherocytosis present?
Jaundice, gallstones, splenomegaly and aplastic crisis
What is seen on blood film in hereditary spherocytosis?
Spherocytes
What is the treatment for hereditary spherocytosis?
Folate supplementation, splenectomy
What inheritance pattern is hereditary elliptocytosis?
Autosomal dominant
What inheritance pattern is G6PD deficiency?
X-linked recessive
What can trigger a G6PD deficiency crisis?
Infections, medications and fava beans
What does G6PD deficiency present with?
Jaundice (usually in the neonatal period), gallstones, anaemia, splenomegaly
What is seen on blood film in G6PD deficiency?
Heinz bodies
What is the diagnostic test for G6PD deficiency?
G6PD enzyme assay
Which drugs can precipitate a G6PD crisis?
Primaquine (antimalarial), ciprofloxacin, sulfonylureas, sulfasazine
What is the management of autoimmune haemolytic anaemia?
Blood transfusion
Prednisolone
Rituximab
Splenectomy
What is alloimmune haemolytic anaemia? When does this occur?
Foreign blood cells or antibodies are circulating in a patient’s blood which causes an immune reaction which leads to haemolysis. This can occur in transfusion reactions or haemolytic disease of the newborn
What is a haematological complication of prosthetic heart valves?
Prosthetic valve haemolysis
How is prosthetic valve haemolysis managed?
Monitoring
Oral iron
Blood transfusion
Revision surgery
What causes thalassaemia?
Genetic defect in protein chains which make up haemoglobin
What is haemoglobin made up of?
2 alpha and 2 beta globin chains
What is the inheritance pattern of thalassaemia?
Autosomal recessive
Why can people with thalssaemia have pronounce foreheads and malar eminences?
The bone marrow expands to produce extra RBCs for chronic anaemia
What are the signs and symptoms of thalassaemia?
Low MCV
Fatigue, pallor
Jaundice, splenomeagly and gallstones
Poor growth and development
What are the investigtions for thalassaemia and what do they show?
FBC shows microcytic anaemia
Haemoglobin electrophoresis shows globin abnormalities
DNA testing shows the abnormality
Why should people with thalassaemia have regular checks of ferritin levels?
To ensure they are not suffering with iron overload.
Iron overload occurs in thalassaemia as a result of faulty creation of red blood cells, recurrent transfusions and increased absorption of iron in response to the anaemia.
What is the management for alpha thalassaemia?
Monitoring FBC
Blood transfusions
Splenectomy
Bone marrow transplant
What are the signs of thalassaemia major?
Severe microcytic anaemia
Splenomegaly
Bone deformities
What are the 3 classes of beta thalassaemia?
Thalassaemia minor
thalassaemia intermedia
Thalassaemia major
What is the management of thalassaemia major?
Tranfusions
Iron chelation
Splenectomy
bone marrow transplant can be curative
What is the variant of haemoglobin which is present in sickle cell anaemia?
haemoglobin S
What is the inheritance pattern of sickle cell anaemia?
Autosomal recessive
What is the difference between sickle cell anaemia and sickle cell disease?
One copy of HbS codes for the sickle cell trait, 2 copies means sickle cell anaemia
What is the sickle cell gene protective against?
Malaria
When is sickle cell disease tested for?
On the newborn screening heel prick test at 5 days of age
What are some complications of sickle cell disease?
Anaemia Stroke Infection Avascular necrosis Pulmonary hypertension Priapism CKD Sickle cell crisis Acute chest syndrome
What is the management of sickle cell disease?
Antibiotic prophylaxis
Hydroxycarbamide (stimulates production of fetal haemoglobin)
Blood transfusion
Bone marrow transplant
What is the management for a sickle cell crisis?
Admit Keep warm Treat infection IV fluids Analgesia Aspirate penis in priapism
What are the features of vaso-occulsive crisis? (also known as painful crisis)
Distal ischaemia Dehydration Raised haematocrit Pain Fever
What is splenic sequestion crisis?
A complication of sickle cell disease. RBCs block blood flow in the spleen which leads to an enlarged and painful spleen. It can result in severe anaemia and circulatory collapse
What commonly triggers aplastic crisis?
Parvovirus B19
What are the diagnostic signs and symptoms of acute chest syndrome?
Fever or respiratory symptoms WITH new infiltrates seen on CXR
What is the management of acute chest syndrome?
Antibiotics
Blood transfusion
Incentive spirometry
Artificial ventilation
What is pancytopenia?
anaemia, leukopenia and thrombocytopenia
What ages do the 4 types of leukaemia affect?
Under 5 and Over 45= ALL
Over 55=CLL
Over 65= CML
Over 75= AML
What are the symptoms of leukaemia?
Non-specific
Fatigue, fever, pallor, failure to thrive, petechiae, abnormal bleeding, lymphadenopathy, hepatosplenomegaly
What are the main investigations for leukaemia?
Urgent FBC
Blood film
Bone marrow biopsy
What is the pathophysiology of ALL?
Acute proliferation of B lymphocytes. Excessive production of these cells causes them to replace other cells leading to pancytopenia
What is on a blood film in ALL?
Blast cells
Which other disease is ALL associated with?
Down’s syndrome
Which 2 types of leukaemia are associated with philadelphia chromosome translocation?
ALL and CML
What is it called when CLL transforms into high-grade lymphoma?
Richter’s transformation
What is seen on blood film in CLL?
Smear or smudge cells
What are the 3 phases of CML?
Chronic, accelerated and blast phase. Chronic lasts around 5 years and shows high WCC. Accelerated phase is when abnormal blast cells take up a high proportion of cells in the bone marrow. The blast phase is when blasts are >30% of blood and is often fatal
What is seen on a blood film in AML?
Auer rods
How is leukaemia generally managed?
Chemotherapy
Steriods
Bone marrow transplant
What is tumour lysis syndrome?
There is a release of uric acid from cells which are being destroyed by chemotherapy
What is the management of tumour lysis syndrome?
Allopurinol and rasburicase to reduce the high uric acid levels
Which ages does Hodgkin’s lymphoma affect?
Has a bimodal age distribution between 20 and 75 years
What are the risk factors for Hodgkin’s lymphoma?
HIV
EBV
Automimmune conditions
Family history
What is the presentation of Hodgkin’s lymphoma?
Lymphadenopathy
Pain in lymph nodes when drinking alcohol
Fever, Weight loss, Night sweats
Investigations for hodgkin’s lymphoma?
Lactate dehydrogenase is raised
Lymph node biopsy is key diagnostic test
What is found on lymph node biopsy in Hodgkin’s lymphoma?
Reed-sternberg
Which staging system is used in Hodgkin’s lymphoma?
Ann Arbor staging
What is the management Hodgkin’s lymphoma?
Chemotherapy and radiotherapy
What is the name of the monoclonal antibody which can be used to treat non-hodgkin’s lymphoma?
Rituximab
Which cells is myeloma a cancer of?
Plasma cells
What are plasma cells?
B lymphocytes which have become activated to produce a certain antibody
What can be found in the urine of myeloma patients?
Bence jones proteins
What causes myeloma bone disease?
Increased osteoclast activity and decreased osteoblast activity
Where does myeloma bone disease usually affect?
skull, spine, long bones and ribs
what metabolic effect does myeloma bone disease?
Causes hypercalcaemia
What are the 4 main features of myeloma?
CRAB Calcium is elevated Renal failure Anaemia Bone lesions/ pain
What are the main investigations for myeloma?
FBC (low WCC)
Calcium(++++)
ESR (+)
Plasma viscosity (+)
Followed by BLIP B-bence jones L-serum free Light chain assay I- serum Immunoglobulins P- serum Protein electrophoresis
What is necessary to confirm the diagnosis of myeloma
Bone marrow biopsy
What can be seen on an x-ray of myeloma?
Punched out lesions
Lytic lesions
Raindrop skull
What is the management of myeloma?
Chemo with: borteazomid, thalidomide and dexamethasone
Stem cell transplant
Bisphosphonates for bone disease
What are the three main types of myeloproliferative disorder?
primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia
What is ruxolitnib?
JAK2 inhibitor
What is myelofibrosis?
Proliferation of a cell line leads to fibrosis of bone marrow
What are the main signs of polycythaemia vera?
Conjunctival pletheroa
ruddy complexion
splenomegaly
What is the management of polycythaemia vera?
Venesection
Aspirin
Chemo
What is myelodyplastic syndrome?
Myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells. Causes anaemia, neutropenia and thrombocytopenia
What is there often on the blood film in myelodysplastic syndrome?
blasts
What is the management of myelodysplastic syndrome?
Supportive transfusions
Chemo
Stem cell transplant
What can thrombocytopenia present with?
Bruising, nosebleeds, bleeding gums, heavy periods, blood in urine or stools
What are the 4 main differentials for prolonged bleeding?
Thrombocytopenia
haemophillia A and B
Von Willebrand disease
DIC
What is the management of ITP
Prednisolone
IV immunoglobulins
Rituximab
Splenectomy
What is the inheritance pattern of von willebrand disease?
Autosomal dominant
What is the presentation of vonwillebrand disease?
Bleeding gums
epistaxis
menorrhagia
Heavy bleeding during operations
What is the management of von willebrand disease
does not require day to day treatment. Management is in response to major bleeding or trauma.
Desmopressin (stimulates release of VWF)
VWF can be infused
Factor VIII infused
Which factor is there is deficiency of in Haemophillia A?
VIII
Which factor is there is deficiency of in Haemophillia B?
IX
What is the inheritance pattern of haemophillia?
X linked recessive
What are signs and symptoms of haemophillia?
Spontaneous haemorrhage
Haemoarthritis
Haematuria
What is the management of haemophillia?
Infusion of VIII or IX
Which syndrome is the main risk factor which predisposes patients to getting clots?
Anti-phospholipid syndrome
What is a contraindication for compression stockings?
Peripheral arterial disease
What are the symptoms of DVT?
Calf swelling Dilated superficial veins Tenderness to the calf Oedema Colour changes to the leg
Which score is used to predict the likelihood of a patient having a DVT or PE?
Well’s score
What is the diagnostic test for DVT?
ultrasound doppler of the leg
What is the diagnostic tes for PE?
CT pulmonary angiogram (ventilation perfusion scan if contrindicated)
What is the initial management of DVT?
Treatment dose of LMWH
When are inferior vena cava filters used?
In cases of recurrent PE or those who are unsuitable for anticoagulation
What is Budd-Chiari syndrome?
When a blood clot develops in the hepatic vein, blocking the outflow of blood
What does Budd-Chiari syndrome present with?
Abdominal pain, hepatomegaly, ascites
