Haematology Flashcards
How can myelodysplastic syndrome be distinguished from AML?
Percentage of blast cells in bone marrow
<20% myelodysplastic syndrome
>20% AML
What is an auer rod and which condition do you see them in?
Granular material that forms elongated needles in cytoplasm of myeloid leukaemic blasts
Myeloperoxidase aggregate
What are smudge/smear cells and what condition do you see them in?
Remnants of cells that lack identifiable cytoplasmic membrane or nuclear structure
Associated with fragile lymphocytes in conditions like CLL
Which leukaemia is response to tretinoin therapy?
Acute promyelocytic leukaemia
What is myelofibrosis?
Proliferation of abnormal clone of haematopoietic stem cells in bone marrow and other sites leads to fibrosis
What are signs and symptoms of myelofibrosis?
Splenomegaly Bone pain Bruising and easy bleeding Cachexia Hepatomagaly Fatigue Gout and high uric acid levels Increased susceptibility to infection Pallor and SOB - anaemia
Why does malignant hypertension lead to haemolytic anaemia?
Mechanical - red blood cells rupture as they become damaged when forced at high pressures through microvasculature
What makes autoimmune haemolytic anaemias warm or cold?
Description of what temperatures the antibodies against the RBCs best work
What blood test abnormalities would you expect to find in DIC?
Increased PT and APTT
Anaemia
Thrombocytopenia
Increased fibrin degradation products
What are some causes of DIC?
Leukaemias
Serious infections - meningococcus, E. coli
Obstetric emergencies
What are signs and symptoms of an acute haemolytic transfusion reaction?
> 2 degrees rise in temperature
Abdominal pain
Hypotension
What features are typical of Von Willibrands disease?
Lifelong tendency to easy bruising Frequent epistaxis Menorrhagia Purpura Post op bleeding
Which clotting result would be deranged in Von willebrands disease?
Prolonged activated partial thromboplastin time
What can be precipitants of haemolysis in G6PD deficiency?
Consumption of fava beans
Antimalarials
Nitrofurantoin
What is marrow involvement in Hodgkin’s disease best treated with?
Chemotherapy
What can cause a raised ESR?
RA
SLE
Polymyalgia rheumatica
Myeloma
What happens to ESR with age?
Increases
ESR normally 1/2 age
What happens to ESR in polycythemia?
Low
A 6 month old boy was noticed to be pale but otherwise well and gaining weight. The liver was 3cm below costal margin and 2cm spleen palpable. Haemoglobin 80, reticulocyte count 4%, WCC normal. What is the likely diagnosis? Why?
Beta thalasseamia major
Anaemia, extramedullary haematopoiesis, hepatosplenomegaly and raised reticulocytes
What does chloramphenicol toxicity do to bone marrow?
Complete suppression
What clinical features does G6PD deficiency cause?
Episodes of haemolysis - jaundice, abdominal pain, dark urine
What is the commonest presenting symptom of myeloma?
Bone pain
What causes polyuria in myeloma?
Hypercalcaemia due to bone destruction
What are the main antigens that give rise to graft rejection?
ABO blood group
Human leukocyte antigen (HLA)
Minor histocompatibility antigens
What are some causes of hyperacute organ rejection?
Blood group ABO incompatibility
Pre existing HLA antibodies
What are the 4 most important HLA types in organ rejection?
HLA A
HLA B
HLA C
HLA DR
If a patient is HLA mismatched with their donor for organ transplant, what will happen?
T lymphocytes will recognise antigens bound to HLA molecules and become activated
Clonal expansion occurs with a response directed at that antigen
What are the different types of organ rejection?
Hyperacute: immediately through pre formed antigens
Acute: during first 6 months, usually T cell mediated, tissue infiltrates and vascular lesions, mononuclear cell infiltrates
Chronic: after 6 months, vascular changes predominate, myointimal proliferation leading to organ ischaemia, loss of acinar cells in pancreas, rapidly progressive CAD in cardiac transplants
Which transplants are at the highest risk of hyperacute organ rejection?
Renal
Give some examples of immune mediated blood transfusion reactions
Pyrexia Alloimmunisation Thrombocytopenia Transfusion associated lung injury Graft versus host disease Urticaria Acute or delayed haemolysis ABO incompatibility Rhesus incompatibility
Give some examples of non immune mediated blood transfusion reactions
Hypocalcaemia
CCF
Infections
Hyperkalaemia
What is graft versus host disease?
Lymphocyte proliferation causing organ failure
What is transfusion associated lung injury?
Neutrophil mediated allergic pulmonary oedema
What are some oral signs of AIDS?
Oral candidiasis
Hairy leukoplakia
Oral gingival regression
Kaposis sarcoma
What is wrong with the haemoglobin of patients with sickle cell anaemia? What is different with sickle cell trait?
Substitution of valine for glutamic acid at position 6 of the beta chain
Abnormal haemoglobin depolymerises at PO2 of 5-6 kPa which is found in normal venous blood so they are continuously sickling
Heterozygotes contain normal and abnormal Hb and only sickle at extremely low PO2 values of 2.5-4kPa
What is important in the peri operative management of sickle cell disease patients?
Keep well oxygenated Keep warm Keep them hydrated Provide adequate analgesia with PCA Avoid acidosis (venous stasis)
What can precipitate sickling in sickle cell disease?
Hypoxaemia Hypothermia Dehydration Infection Exertion Stress
What are clinical features of multiple myeloma?
Bone disease: pain, osteoporosis, pathological fractures, osteolytic lesions Lethargy Infection Hypercalcaemia Renal failure Amyloidosis Carpal tunnel syndrome Hyperviscosity
What investigations should be done for multiple myeloma?
Monoclonal proteins: IgG or IgA in serum and urine (bence jones proteins)
Increased plasma cells in bone marrow
Bone lesions on skeletal survey
What are the criteria for diagnosis of multiple myeloma?
Major: plasmacytoma, 30% plasma cells in bone marrow sample, elevated levels of M protein in blood or urine
Minor: 10-30% plasma cells, minor elevations in M protein, osteolytic lesions, low levels of antibodies in blood
Diagnosis requires 1 major and 1 minor or 3 minor in someone with signs and symptoms
Why does hypercalcaemia occur in multiple myeloma?
Increased osteoclastic bone resorption caused by local cytokines released by myeloma cells
Impaired renal function
Increased renal tubular calcium reabsorption and elevated PTHrP levels
What features might be seen on a blood film in a hyposplenic patient?
Target cells Howell Jolly bodies Pappenheimer bodies Siderotic granules Acathocytes
What features might be seen on a blood film of a patient with iron deficiency anaemia?
Target cells
Pencil poikilocytes
What feature is seen on a blood film of a patient with myelofibrosis?
Tear drop poikilocytes
What feature is seen on blood film of a patient with intravascular haemolysis?
Schistocytes
What is seen on a blood film of a patient with megaloblastic anaemia?
Hypersegmented neutrophils
What is the most common inherited bleeding disorder?
Von Willebrands disease
What are common features of Von willebrands disease?
Epistaxis
Menorrhagia
Bleeding after tooth extraction
What are the different types of Von willebrands disease?
Type 1: partial reduction in vWF
Type 2: abnormal form
Type 3: total lack of (autosomal recessive)
How is Von willebrands investigated?
Prolonged bleeding time
APTT may be prolonged
Factor VIII levels moderately reduced
Defective platelet aggregation with ristocetin
How is Von willebrands managed?
Tranexamic acid for mild bleeding
Desmopressin: raises levels of vWF by inducing release from Weibel-Palade bodies in endothelial cells
Factor VIII concentrate
What are the 4 different crises of sickle cell anaemia?
Thrombotic (painful)
Sequestration
Aplastic
Haemolytic
What is a thrombotic crisis in sickle cell anaemia?
Painful, vaso occlusive
Precipitated by infection, dehydration, deoxygenation
Infarction organs including bones
What is sequestration crisis in sickle cell anaemia?
Sickling within organs such as spleen or lungs causes pooling of blood with worsening of anaemia
Acute chest syndrome: dyspnoea, chest pain, pulmonary infiltrates, low PO2
What is the most common cause of death after childhood in sickle cell anaemia?
Acute chest syndrome
What is aplastic crisis in sickle cell anaemia?
Infection with parvovirus
Sudden fall in haemoglobin
What is a haemolytic crisis in sickle cell anaemia?
Rare
Fall in haemoglobin due to increased haemolysis
What are the histological classes of Hodgkin’s lymphoma? Which has the best and worst prognosis?
Nodular sclerosing - good prognosis
Mixed cellularity - good prognosis
Lymphocyte predominant - best prognosis
Lymphocyte depleted - worst prognosis
What are B symptoms of Hodgkin’s lymphoma?
Weight loss >10% in last 6 months
Fever >38
Night sweats
What factors are associated with poor prognosis of Hodgkin’s lymphoma?
B symptoms Age >45 Stage IV disease Haemoglobin <10.5 Lymphocyte count <600 Male Albumin <40 White cell count >15000
What is Burkitts lymphoma?
High grade B cell neoplasm
Endemic (Africa): maxilla or mandible
Sporadic: abdominal (ileo-caecal), commonly with HIV
What gene translocation is associated with Burkitts lymphoma?
C myc t(8:14)
Which virus is strongly implicated in the development of the African form of Burkitts lymphoma?
Epstein Barr virus
What are microscopy findings in Burkitts lymphoma?
Starry sky appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
How is Burkitts lymphoma managed? What problem can this cause?
Chemotherapy
Rapid response which may cause tumour lysis syndrome
What is given before chemo in Burkitts lymphoma to reduce the risk of tumour lysis syndrome occur if?
Rasburicase
What are complications of tumour lysis syndrome?
Hyperkalaemia Hyperphosphataemia Hypocalcaemia Hyperuricaemia Acute renal failure
What is the Philadelphia chromosome?
t(9:22)(q34;q11)
Associated with CML
Results in tyrosine kinase activity in excess of normal
A 68 year old male presents to GP with enlarged nodes in neck, axillae and groin. Blood tests show Hb 105, WBC 19. A blood film shows 60% white cells are small mature lymphocytes. What is the most likely diagnosis?
Chronic lymphocytic leukaemia
What are features of CLL?
Often none Constitutional: anorexia, weight loss Bleeding Infections Lymphadenopathy
What are complications of CLL?
Hypogammaglobulinaemia leading to recurrent infections
Warm autoimmune haemolytic anaemia
Transformation to high grade lymphoma (Richters transformation)
What investigations are done for CLL?
Blood film: smudge cells/smear cells
Immunophenotyping
What is the Ann Arbor staging for non hodgkins lymphoma?
Stage 1: involvement of single lymph node region
Stage 2: involvement of 2 or more regions on same side of diaphragm
Stage 3: involvement of lymph node regions on both sides of the diaphragm
Stage 4: multifocal involvement of 1 or more extralymphatic sites +/- associated lymph nodes
Which vaccines are live attenuated and therefore shouldn’t be given to patients who are immunosuppressed?
BCG MMR Oral polio Yellow fever Oral typhoid
By how much does 1 unit of blood increase Hb concentration?
10g/L
What are the most important symptoms/complications of myeloma?
CRAB Calcium raised Renal failure Anaemia Bone pain
What is the difference between warm and cold agglutinins?
Autoantibodies react with red cells at temps above 37 in warm and below 37 in cold
Both can be lymphoproliferative disorders
Warm primarily in spleen, IgG
Cold: infections (mycoplasma/EBV)
What is the diagnosis in a 7 year old boy presenting with haemarthrosis of his left knee. He has had repeated episodes of this since his early years. His APTT is prolonged but bleeding time is normal?
Haemophilia A
How are minor and major bleeds associated with haemophilia treated?
Minor: desmopressin which raises factor VIII levels
Major: factor VIII concentrate to raise levels above 50%
What is the management for an alcoholic patient presenting with ruptured oesophageal varices who has received 6 units of blood but has a prolonged prothrombin time?
Fresh frozen plasma to replace clotting factors
A day old baby who’s mother takes anticonvulsants is admitted with unexplained bruising. Clotting profile shows PT and APTT are prolonged. What is the likely diagnosis and what is the management?
Haemorrhagic disease of the newborn
Give Vit K to correct absence of Vit K dependent clotting factors
What is coombs test?
Direct and indirect
Direct used for autoimmune haemolytic anaemia
Indirect used for prenatal testing of pregnant woman and prior to transfusion
What are poor prognostic features of acute myeloid features?
> 60 years
20% blasts after first course of chemo
Cytogenetics: deletions of chromosome 5 or 7
What is Waldenstroms macroglobulinaemia?
Lymphoplasmacytic lymphoma
Affects B cells which produce excess immunoglobulin - monoclonal IgM paraproteinaemia
Bone marrow infiltration
Splenomegaly
Lymphadenopathy
Hyperviscosity of blood so risk of strokes/clots
Why does alcohol cause a large MCV?
Alcohol has effect on alkaline phosphatase on RBC membrane producing relative swelling of the cell
What condition is suggested by a prolonged APTT and thrombocytopenia with a background of recurrent DVT?
Antiphospholipid syndrome
Name some clotting disorders which make you pro thrombotic (thrombophilias)
Factor V Leiden Prothrombin mutation Antithrombin III deficiency Protein C deficiency Protein S deficiency Factor XIII mutation Familial dysfibrinogenaemia Antiphospholipid syndrome
What is hereditary spherocytosis?
Autosomal dominant abnormality of erythrocyte membrane proteins which interfere with cells ability to travel through capillaries
More prone to rupture, cells taken to spleen
Haemolytic anaemia
Howell Jolly bodies on blood smear
What is Virchows triad which describes factors that contribute to thrombosis?
Venous stasis
Endothelial damage
Blood hypercoagulability: hyperviscosity, deficiency of antithrombin III, nephrotic syndrome, disseminated malignancy, pregnancy, smoking
What should be done to manage a case of DIC?
Clotting studies and platelet count
Advice from haematologist
Up to 4 units of FFP and 10 units of cryoprecipitate whilst awaiting results
What is waldenstrons macroglobulinaemia?
Lymphoplasmocytic lymphoma
High levels of IgM
Preceded by IgM monoclonal gammopathy of undetermined significance
Weakness, fatigue, weight loss, chronic oozing of blood from the nose and gums
What is porphyria cutanea tarda?
Presents with skin manifestations later in life
Low levels of enzyme responsible for 5th step in heme production
Onycholysis and blistering of sun exposed skin
Which cytokine is involved in the reaction to a Mantoux test?
Interferon gamma secreted by t helper 1 cells which stimulate macrophage activity
In which condition are smudge cells seen on blood film?
Chronic lymphocytic leukaemia
What is the most common childhood malignancy?
Acute lymphoblastic leukaemia
Which cell lineage is most commonly affected in ALL?
B cell lineage
What are some causes of AML/ALL?
Ionising radiation
Chemicals
Viruses
Chromosomal abnormalities
What are clinical features of ALL?
Critically ill due to bone marrow failure: Anaemia, Infection, Haemorrhage
Signs of leucostasis
Bone/joint pain
Widespread lymphadenopathy
Mild to moderate hepatosplenomegaly + orchidomegaly
CNS involvement – meningism, facial nerve palsy
What investigations should be used for ALL and what might the results show?
FBC – Hb, neutrophils and platelets low Total WCC high with blast cells on film Blood film – blast cells Bone marrow aspirate - >20% blast cells CXT/CT – mediastinal/abdominal lymphadenopathy LP – CNS involvement
What is the management of ALL?
Resuscitation if septic / shock / haemorrhage
Supportive treatment
Chemotherapy: Aim to eliminate leukaemic cells and achieve complete haematological remission
4 continuous phases: Remission induction, Consolidation therapy, CNS prophylaxis, Maintenance therapy
Consideration of allogenic stem cell transplantation
What are poor prognostic factors in ALL?
Patient age
High leucocyte count
Cytogenetics
Long time to complete remission (>4 weeks)
What is the most common leukaemia in adults?
AML
What are associated conditions with AML?
Myelodysplasia, prior chemotherapy, radiation, chromosomal abnormalities (Down syndrome)
What are clinical features of AML?
Present critically ill due to bone marrow failure
Anaemia: Weakness, lethargy, breathlessness, palpitations
Infection: Chest, mouth, perianal, skin
Haemorrhage: Purpura, epistaxis, bleeding gums
Gum hypertropy / skin infiltration
Hepatosplenomegaly
Leucostasis: Hypoxia, confusion, CVA
What investigations should be done for AML?
Bloods: FBC – leukocytosis, anaemia, thrombocytopaenia
Blood film – presence of blast cells
Bone marrow aspirate - >20% blast cells
Differentiate from ALL with immunophenotyping or cytoplasmic myeloperoxidase (+ve in AML)
What is the management of AML?
Resuscitation – treat for neutropenic sepsis, haemorrhage
Fluids/allopurinol for tumour lysis syndrome
Chemotherapy: Aim to achieve complete haematological remission (<5% blast cells) + normalisation of peripheral blood count
2 phases: Remission induction – 2 cycles of combination chemo, Consolidation therapy – 2 cycles – reduce risk of relapse
What are poor prognostic factors for AML?
Advancing age >60 years
Specific cytogenetic abnormalities – ‘poor risk’ groups
Failure to achieve complete remission with 1st cycle
What is chronic lymphocytic leukaemia?
Progressive accumulation of mature appearing B lymphocytes in theperipheral blood, bone marrow, lymph nodes, spleen and liver
What are clinical features of chronic lymphocytic leukaemia?
Asymptomatic – incidental lymphocytosis on routine FBC
Advance disease associated with: Weight loss, fever, malaise, Lymphadenopathy, hepatosplenomegaly
Bone marrow failure due to infiltration: anaemia, neutropenia,
thrombocytopenia
Recurrent infections due to reduced antibody production: Hypogammaglobulinaemia
What investigations should be done for CLL? What might they show?
FBC – lymphocytosis > 5.0, Autoimmune haemolysis, Thrombocytopenia, anaemia, neutropenia
Blood film: Lymphocytosis, Presence of ‘smear’ / ‘smudge’ cells – damage to cells during preparation
Immunophenotyping
BM - >30% mature lymphocytes
Immunoglobulins – hypogammaglobulinaemia
What is the management of CLL?
Choice between observation, palliation or aim for complete remission
Management based on age, performance status, co-morbidities,
symptoms present and prognostic factors
Palliative approach treating symptomatology with minimal toxicity OR prolong disease-free survival
Palliative - alkylating agents
What is the prognosis of CLL?
Incurable disease
Early stage, asymptomatic patients – die of unrelated causes Advance stage - refractory disease and bone marrow failure
What are poor prognostic factors for CLL?
Male
Initial lymphocytosis >50 x 109/L
Lymphocyte levels doubling in <12 months
Specific cytogenetic abnormalities/ mutations
Poor response to therapy
What is CML?
Characterised by large granulocytes and presence of the Philadelphia chromosome
What are clinical features of CML?
Accelerated phase characterised by blood count and organomegaly becoming refractory to therapy 30% asymptomatic at diagnosis Lethargy, weight loss, sweats Splenomegaly, bruising/bleeding Occasional signs of leucostasis
What investigations should be done for CML and what might they show?
FBC: Raised WCC (often 100-300 x 109/L) – predominantly neutrophils, Anaemia, Platelets often unaffected or raised
Raised urate
BM: Hypercellularity – granulocyte hyperplasia, Chronic phase <10% blasts / accelerated >10% / crisis >20%
Cytogenetic analysis: Presence of Philadelphia chromosome
What is the management of CML?
Commence allopruinol
Consideration of leucapheresis in leucostasis
Tyrosine kinase inhibitor (Imatinib) – 1st line – turns off signalling cascade
What are the myeloproliferative disorders?
Polycythemia vera – excessive proliferation of the erythrocyte,
myeloid and megakaryocyte lineages
Essential thrombocythaemia – persistent thrombocytosis not due to any other cause
Primary myelofibrosis – neoplasm of early haematopoietic stem cell leading to marked hyperplasia of abnormal megakaryocytes and
monocytes stimulating marrow fibrosis
What are important features of management of polycythemia vera?
Venesection
Manage thrombosis risk
What are features of myeloma?
Usual presentation with bone pain (back) or pathological fracture
Weakness, fatigue, thirst, polyuria, oedema due to renal impairment
Acute hypercalcaemia
HyperCalcaemia, Renal impairment, Anaemia, Bone problems (CRAB)
What investigations should be done for myeloma and what might they show?
FBC – anaemia / U+Es – renal impairment
Serum protein electrophoresis – detect paraproteins
X-ray site of bone pain – pathological fracture / lytic lesions
BM aspirate – plasma cell infiltration
What is the management of myeloma?
Not curable
Transient responses to chemotherapy
Pain control
Management of complications: Renal impairment, Hypercalcaemia, RTx for bone disease, Anaemia, Cord compression
What are causes of DIC?
Sepsis/trauma/organ necrosis/malignancy/liver failure/transfusionin compatibility
What factors can be used in a scoring system to help with diagnosis of DIC?
Plt count
D-dimer
Prolonged PT
Fibrinogen level
What is the management of DIC?
Treat precipitating cause
Supportive therapy
Replacement therapy of platelets, cryoprecipitate, FFP
What is thrombotic thrombocytopenic purpura?
Increased platelet aggregation and occlusion of arterioles and capillaries of the microcirculation due to antibodies that activate the immune system to inhibit the ADAMTS13 enzyme
What are clinical features of TTP?
Severe thrombocytopenia Neurological involvement Renal impairment Fever Haemolysis (micro-angiopathic haemolytic anaemia)
What is the management of TTP?
Haematological emergency May require ITU level of care Plasmapheresis FFP for fluid replacement Platelet transfusions can worsen condition therefore only give in major bleed Steroids
In which condition are Auer rods seen?
AML
What are Howell jolly bodies? When are they seen?
Blue black inclusions of red blood cells which represent residual nuclear fragments Seen in asplenic patients Haemolytic anaemia Leukaemia Thalassaemia
What are pappenheimer bodies, when are they seen?
Violet staining granules in periphery of RBCs containing siderblastic bodies
Seen in sideroblastic anaemia, lead poisoning, thalassaemia, asplenic patients
What is the pathophysiology behind beta thalassaemia major?
Not enough b chain produced
Alpha chains precipitate in erythroblasts and mature red cells
Ineffective erythropoiesis and haemolysis
Anaemia occurs at 3-6 months when switch from g to b chain production takes place
Marrow hyperplasia to compensate leads to thalassaemic facies: hair on end appearance of skull X-ray and thinning of cortex with increased risk of fractures
Which atypical pneumonia can be associated with cold agglutinins and therefore Raynauds and autoimmune haemolytic anaemia?
Mycoplasma
Which crises can occur with sickle cell anaemia?
Haemolytic crisis
Splenic sequestration crisis
Aplastic crisis
To which bugs are patients with sickle cell susceptible?
Encapsulated bacteria due to hyposplenism: pneumococci, meningococci
How long after a DVT are patients recommended not to fly for?
2 weeks
What is the triad of symptoms in yellow nail syndrome?
Lymphoedema
Pleural effusions
Yellow discolouration of nails
What are indications for red cell transfusion?
Acute blood loss: especially if >1.5 L in adult
Symptomatic anaemia with no easily treatable cause
Long term transfusion dependent anaemia
Radiotherapy patient Hb <100
Chemotherapy patient Hb <90
What are potential complications from a blood transfusion?
Acute haemolytic reaction Anaphylaxis Transfusion associated lung injury Non haemolytic reaction Allergic reaction Fluid overload
What is the name of the haemovigillance scheme that monitors adverse events in blood transfusion?
SHOT
Which drugs should be co prescribed alongside a blood transfusion?
Hydrocortisone
Chlorpheniramine
Furosemide
In which patients can giving vitamin K cause haemolysis?
G6PD deficiency
What is octaplex used for?
Reversal of warfarin therapy or vit K deficiency
What is octaplex?
Prothrombin complex concentrate
After giving octaplex, when should you re check INR?
30 mins
What causes transfusion related lung injury?
Donor Ab (esp HLA or neut) react with recipient WBCs leads to lung infiltrate
Occurs 1-6 hours post transfusion
Associated with FFP
What are indications for FFP?
Coagulation factor deficiency where specific concentrates not available
Multiple clotting factor deficiency: liver disease, DIC, massive transfusion
What is the greatest cause of transfusion death in the U.K.?
Transfusion associated circulatory overload
What are causes of massive splenomegaly?
Myelofibrosis CML Visceral leishmaniasis Malaria Gauchers syndrome
With which condition is the Philadelphia chromosome associated?
CML
How does CML present?
Middle age Anaemia Weight loss Abdo discomfort Splenomegaly Spectrum of myeloid cells in peripheral blood Decreased leukocyte alkaline phosphatase
What is the management of CML?
Imatinib: tyrosine kinase inhibitor
Hydroxyurea
Interferon alpha
Allogenic bone marrow transplant
What is the maximum time for transfusion of red cells?
3.5 hours
When would you prescribe a platelet transfusion?
Prophylaxis if platelets <10
If septic, abnormality of coagulation <20
Bleeding/procedure/surgery <50
Brain/eye/bleeding surgery <100
How does acute intermittent porphyria present?
Abdominal pain
Neuropathies
Constipation
How can a diagnosis of acute intermittent porphyria be made?
Elevated level of porphobilinogen on a spot urine test during acute attack
How is acute intermittent porphyria managed?
Mild: high doses of glucose
Severe: haematin
What is acute intermittent porphyria?
Autosomal dominant disease resulting from defect in porphobilinogen deaminase so porphyrin precursors accumulate leading to neurological damage and psychiatric manifestations
What is idiopathic thrombocytopenic purpura?
Immune mediated reduction in platelet count
Antibodies directed against glycoprotein IIb/IIIa or Ib/V/IX complex
What is the most common type of Hodgkin’s lymphoma?
Nodular sclerosing
Which factors are associated with a poor prognosis in Hodgkin’s lymphoma?
Lymphocyte depleted type B symptoms: weight loss, fever, night sweats Age >45 Stage IV disease Haemoglobin <10.5 Lymphocyte count <600 Male Albumin <40 White cell count >15
What are possible complications of CLL?
Hypogammaglobulinaemia leading to recurrent infections
Warm autoimmune haemolytic anaemia
Transformation to high grade lymphoma (richters transformation)
What is beta thalassemia major?
Absent synthesis of beta haemoglobin chains due to mutation in HBB gene on chromosome 11, autosomal recessive
Inability to construct new beta chains leads to underproduction of HbA which leads to microcytic anaemia
What is HbF and HbA2?
Different forms of haemoglobin which are present in beta thalassemia
HbF: alpha alpha gamma gamma
HbA2: alpha alpha delta delta
What are the causes of DIC?
Severe sepsis and septic shock Malignancy Obstetric disease: eclampsia, abruption, amniotic fluid embolism Rheumatological disease Severe liver disease Transfusion reactions
What does a schilling test assess? What is it?
Absorption of vitamin B12
Following overnight fast, body injected with non labelled B12. This is followed by oral labelled B12 with cobalt 58, and a second dose with cobalt 57 bound to intrinsic factor
Malabsorption if less than 10% labelled excreted in 24 hours
Pernicious anaemia if elevated ratio of Co-57 to Co58
What is a typical finding of myelofibrosis on blood film?
Tear drop shaped cells
What is used in mild Von willebrand disease if there is evidence of bleeding?
DDAVP (desmopressin)
How is idiopathic thrombocytopenic purpura managed?
If symptomatic or platelet count below 20 then prednisolone
Splenectomy cures most cases
How is a bleed on warfarin managed?
If significant active bleeding then octaplex (prothrombin complex concentrate)
In which patients is pernicious anaemia most common?
Fair haired Elderly Women Blue eyes Autoimmune conditions: thyroid disease and vitiligo
How is pernicious anaemia managed?
IM vitamin B12/hydroxycobalamin
What is the leading cause of death in ITP?
Intracranial haemorrhage
What is the treatment choice for a patient with ITP and massive haemorrhage?
Platelet transfusion
IV methylprednisolone
IV immune globulin
Splenectomy
In a patient presenting with an unprovoked DVT, other than commencing warfarin, what further actions are required?
Chest X-ray Bloods: FBC, calcium, LFTs Urinalysis If all normal: abdo pelvis CT and mammogram for woman in all patients over 40 Check antiphospholipid antibodies
What is haptoglobin?
Binds haemoglobin that has been released from erythrocytes
Can be used as measure of Haemolysis - levels will be decreased in intravascular
What are provocative factors for a sickle cell crisis?
Exercise Infection Fever Anxiety Hypoxia Changes in temperature
What is acute chest syndrome?
Vaso occlusive crisis of pulmonary vasculature seen in patients with sickle cell anaemia
What are complications of sickle cell?
Acute chest syndrome Pulmonary infarction Pneumonia Stroke Renal papillary necrosis Priapism Aseptic necrosis of bones and joints Digital infarction Leg ulcers
What can be given to induce release of Von willebrands factor from endothelial cells and therefore reduce bleed risk in theatre?
Desmopressin
What is the most common inherited thrombophilia?
Activated protein c resistance (factor V Leiden)
What are some inherited causes of hypercoagulable state?
Factor V Leiden Prothrombin gene mutation Anti thrombin deficiency Protein c and s deficiencies Elevated homocysteine Dysfibrinogenaemia Sickle cell disease
What are some causes of acquired hypercoagulable state?
Antiphospholipid syndrome Supplemental oestrogen use Heparin induced thrombocytopenia and thrombosis (HITT) Cancer Obesity Pregnancy
What is the function of activated protein c?
Along with cofactor protein s it inactivates factor Va and VIIIa
What is the mechanism of action of heparin?
Unfractioned - binds to antithrombin III to help inactivate thrombin
LMWH - factor Xa inhibitor
What is waldenstroms macroglobulinaemia?
Lymphoplasmacytic lymphoma producing excess IgM
Presents with bone marrow infiltration, splenomegaly and lymphadenopathy, raynauds, visual disturbance due to hyperviscosity
How is a diagnosis of hereditary spherocytosis made?
Osmotic fragility test
What is the management of hereditary spherocytosis?
Folate replacement
Splenectomy
Which factors are associated with increased bleeding risk when on anticoagulant drugs?
Poor INR control Hepatic or renal disease EtOH abuse Malignancy Anaemia Prior bleeding history Age Excessive fall risk Prior history of stroke Concomitant antiplatelets
Which indication for anticoagaultion has a target INR higher than 2.5?
Recurrence of VTE while on warfarin
Mechanical prosthetic heart valve
Why do warfarin and heparin need to overlap when treating acute venous thromboembolism with loading doses of warfarin?
Time delay in achieving an antithrombotic effect based on the elimination half-lives of the vitamin K dependent clotting factors
Protein C: Warfarin is initially prothrombotic due to decreasing protein c levels due to short half life
What are the pharmacokinetics of warfarin?
Readily absorbed from the gastro-intestinal tract
Plasma half-life is about 40 hours
It is metabolised in the liver, and is excreted in the urine mainly as metabolites
What are some non haemorrhagic adverse effects of warfarin?
Rash Alopecia Nausea Diarrhoea Effects in utero: Congential abnormalities and foetal death if given to pregnant woman 6-14 weeks gestation
Which foods and drinks does warfarin interact with?
EtOH: Binge drinking
Vitamin supplements: Vitamin K containing
Diet: Foods rich in vitamin K eg broccoli, brussels sprouts, cabbage, kale, lettuce, spinach, watercress, and other green leafy vegetables
What colours are the different doses of warfarin?
0.5mg white
1mg brown
3mg blue
5mg pink
What baseline tests need doing before commencing warfarin?
FBC
LFTs
INR
Why is it important to ensure the blue top bottle is filled correctly for calculating an INR?
Underfilled citrate bottle will cause high INR result as it will cause high citrate levels
Citrate binds calcium. Calcium is important for initiating clotting
What patient advice should be offered when starting warfarin?
Individual dose is adjusted to achieve desired level of anticoagulation
Dose made up of tablets of different strengths and colours
INR monitored regularly so must attend for checks
Monitor for bleeding and know what to do if it happens
Keep record safe and up to date
Avoid medicines containing aspirin
Don’t start or stop new drugs including OTC without checking with dr or pharmacist first - huge number of interactions
Avoid binge drinking
Tell anyone involved in treating them that they are on warfarin
What does an oral anticoagulant patient pack contain?
Yellow Book (treatment record)
Patient information
Patient alert card
When should INR be checked after starting warfarin?
Day 5, 8 and 12
What are options to reverse warfarin?
Vitamin K: IV or PO
Prothrombin Complex Concentrate eg octaplex/beriplex: Factors II, IV, IX, X, protein c and s
How long does oral and IV vitamin K take to work?
Oral: Takes 24 hours to work
IV: Takes 6-8 hours to work
What are different levels of thrombotic risk assessed for patients on warfarin before surgery? What does this mean for peri operative bridging therapy?
High risk: recent history of VTE (<3 months), or recurrent VTE on increased target INR (3.5) warfarin and severe prothrombotic disorder eg known Antiphospholipid Syndrome or Antithrombin
Deficiency. Need therapeutic LMWH
Moderate risk: history of recurrent VTE within last 3-12 months. Need prophylactic LMWH
Low risk: majority of AF patients and patients with past history of single episode of VTE >12 months ago. No bridging required
What monitoring is required when a patient is on unfractionated heparin?
Need for regular APTTr and FBC every 6 hours
In which patients is routine monitoring required for LMWH? What monitoring is done?
Pregnancy Infancy <1 year old Renal failure Morbid obesity Active bleeding with no apparent cause Anti Xa levels
What are the pharmacokinetics of heparin?
LMWH: Rapidly and completed absorbed via S/C injection. Peak activity 1-4 hours post dose. Renal elimination primarily (half life 4-5 hours) although small degree of hepatic metabolism
IV heparin: Elimination as above, Half life 30 mins – 2 hours
What are non bleeding adverse effects of heparin?
Allergic HITT Local skin reaction Hyperkalaemia Osteoporosis
What are some indications for therapeutic LMWH?
Treatment of VTE / peripheral vascular surgery
Treatment of unstable angina and non-Q-wave myocardial infarction
Treatment of acute ST-segment elevation myocardial infarction
Prevention of extracorporeal thrombus formation during haemodialysis
When should a sample be taken to check for anti Xa levels?
4 hours post dose
2 tubes required
What can be used to reverse heparin?
Protamine
What are contraindications to use of DOACs?
Heart valves Renal failure GFR <15 Strong CYP inhibitors eg systemic azoles HIV drugs PGP inhibitors: Amiodarone, clarithromycin, colchicine, omeprazole, nifedipine, sertraline, tamoxifen, verapamil
When should DOACs be stopped before surgery?
Minor surgery 24 hours
Major 48 hours
Renal failure 72 hours
What is the novel antidote for dabigatran? When can it be used?
Idarucizumab (Praxbind)
Emergency major surgery (not to be used in elective or non-emergency cases)
Life-threatening or uncontrolled bleeding
You are asked to see a 49 year old woman with a mechanical mitral heart valve on long term warfarin in the pre-op assessment clinic prior to laparascopic cholecystectomy.
How would you manage her peri-operative anticoagulation?
Stop warfarin 5 days pre op
Give bridging LMWH from 3 days pre op
Last dose 24 hours before surgery
What are features of HIIT syndrome?
DVT PE MI Occlusion of limb arteries (possibly resulting in amputation) Cerebrovascular accidents (stroke,TIA) Skin necrosis End-organ damage (adrenal, bowel, spleen, gallbladder or hepatic infarction, renal failure) Death
24 year old woman with x2 previous DVTs on long term warfarin admitted c/o abdominal discomfort. Routine pregnancy test positive. What action needs taking?
Stop warfarin immediately and commence LMWH / liaise with obstetrics
76 year man, known atrial fibrillation on warfarin. Attends routine cardiology clinic. INR noted to be high (6.5). No bleeding apparent. What treatment is required?
PO vitamin K
78 year old man with inoperable lung carcinoma is.During admission develops a swollen, painful leg. Ultrasound confirms a popliteal DVT. How do you manage his initial anticoagulation?
Start LMWH monotherapy
What goes wrong that leads to leukaemogenesis?
Loss of cell-cycle control Autonomous proliferation Increased self-renewal Differentiation block Resistance to apoptosis: increased or decreased Dissemination Loss of tumour suppressor genes
What is the management of neutropenic sepsis?
Treat as neutropenic without waiting for FBC result
Blood cultures
Broad spectrum antibiotics within 60 minutes of presentation
IV fluid resuscitation
Get help
What living conditions are important when undergoing chemo for AML?
Filtered air No plants or flowers No unnecessary visitors Washed food – no salad or grapes or blackpepper Antifungal prophylaxis Mouthcare
What are indications for bone marrow biopsy?
Diagnostic: Abnormal FBC, Paraproteinaemia, Bone lesions in pelvis accessible by this route, Pyrexia of unknown origin, Isolated splenomegaly with diagnosis unclear from peripheral blood
Staging: Hodgkin Lymphoma / Non Hodgkin Lymphoma
Treatment response: Leukaemia, Myeloma, Lymphoma
What investigations needs to be done for multiple myeloma?
Protein electrophoresis serum/urine
Bone marrow examination
Skeletal survey
What causes rouleaux formation in blood film?
Plasma protein concentration high, raised ESR
Caused by: infections, myeloma, waldenstrons macroglobulinaemia, connective tissue disorders, cancer
What causes renal failure in myeloma?
Light chain deposition in kidney Hypercalcaemia Hyperuricaemia Dehydration Non-steroidal anti-inflammatories Plasma cell infiltration of kidney
How does the Philadelphia chromosome lead to CML?
Translocation causes constitutive activation of a tyrosine kinase at basis of the disease
Constant activation of cell turnover
What is the management of CML?
Initial control of very high white cell count to prevent stroke
– aspirin
Drugs to control white cell count
Leucopheresis
Imatinib: inhibitor of the BCR-ABL tyrosine kinase inhibitor
What levels of haematocrit (PCV) are high?
PCV>0.60 – men
PCV>0.56 – women
Which mutation is found in association with primary polycythemia?
JAK2
Signals from EPO into cell
Red cell production in absence of growth factor when mutated
What is management of primary polycythemia?
Reduce risk of cardiovascular events as result of increased abnormal red cell production:
Venesections
Aspirin
Drugs to suppress bone marrow function
What are myeloproliferative disorders?
Clonal, pre-leukaemic
Uncontrolled proliferation of one or more bone marrow lineages:
Red cells – primary polycythaemia
Platelets – essential thrombocythaemia
White cells (myeloid) – chronic myeloid leukaemia
Fibroblasts - myelofibrosis
What are malignant causes of hyperviscosity?
Paraprotein (IgM > IgA > IgG)
High WCC (CML / AML > CLL)
High red cell mass (polycythaemia)
Raised platelet count (>1,000, myeloproliferative rather than reactive)
What are causes of splenomegaly?
Haematological: Chronic myeloid leukaemia, Myelofibrosis, Chronic lymphatic leukaemia, Acute lymphoblastic leukaemia, Lymphoma
Infective: EBV, Chronic malaria, Visceral Leishmaniasis
Liver: HCV / HBV with portal hypertension, Any cause cirrhosis with portal hypertension
What are indications for lymph node biopsy?
Generalised lymphadenopathy, FBC unhelpful
Palpable cervical LN with mediastinal LN on CXR
Isolated lymphadenopathy – no obvious pathology in anatomical region drained, (ENT: nasendoscopy NAD, FNA unhelpful)
Regional lymphadenopathy with obvious primary pathology inaccessible to biopsy
What are some possible findings from lymph node biopsy?
Reactive Necrotic Granulomatous – TB, Sarcoid? HIV Metastatic Carcinoma Metastatic Melanoma Lymphoma
What are risks of chemo and radiotherapy?
Breast cancer Thyroid cancer Secondary leukaemia / myelodysplasia Infertility Other endocrine failure - early menopause Bones Cardiac damage (chemo + radiotherapy)
What are causes of anaemia?
Physiological Impaired production: BM failure, Dyserythropoesis- eg thalassaemia, Aplasia, leukaemia, Anaemia of chronic disease Reduced haematinics (Fe, Folate, B12) Increased breakdown (Haemolysis) Increased loss (Haemorrhage) Multifactorial
What are the stages of development of iron deficiency anaemia?
Normal
Low iron stores: low ferritin
Pre-clinical iron deficeiency: low MCV
Clinical iron deficiency: low Hb
What are causes of iron deficiency anaemia in children?
Dietary: especially infants and toddlers, Fussy eaters, High milk intake, High requirements/prematurity
Malabsorption
Increased loss: GIT, menstruation
How should haemolytic anaemia be investigated?
Reticulocytes: increased
LDH: high levels
Haptoglobin: absent (intravascular haemolysis)
Direct Coombs Test (autoimmune haemolysis, Looking for immunoglobulins on red cells)
Blood Film for medical review
Thalassemia and haemoglobinopathy screen as appropriate
Further testing depending on results of initial tests
How can haemolytic anaemia present?
Anaemia
Jaundice
Splenomegaly
Hyperbilirubinaemia
What are some causes of haemolytic anaemia?
Intrinsic: Membrane defects: hereditary spherocytosis
Abnormal Hb: Sickle cell, Thalassaemia
Enzyme defect: G6PD
Extrinsic: Haemolytic Disease of newborn: Rh Dx and ABO incompatibility
Drug induced
HUS
How can you prevent megaloblastic crisis in hereditary spherocytosis?
Folate supplementation
What causes red cell aplasia in hereditary spherocytosis?
Parvovirus infection
Which populations are mainly affected by sickle cell disease?
African, Middle Eastern, Asian
What is sickle cell disease?
Amino acid substitution in Beta-globulin chain of haemoglobin Autosomal Recessive Normal (adult) Hb AA Carrier trait AS Sickle cell Diagnosis: Hb SS, SC, SD
How can sickle cell patients present?
Crisis: Pain- limbs, chest, abdomen, Aplastic, Sequestration
What are complications of sickle cell disease?
Anaemia Hb ~80 Splenic infarction – hyposplenism Infection Poor growth Leg ulcers, stroke
What is the management of sickle cell disease?
Prevention: antenatal diagnosis
Prophylaxis: folate, antibiotics, avoid cold and dehydration
Crisis: fluids, antibiotics, analgesia, exchange transfusion, Mobile auromated red cell exchange service
Longer term management: Hydroxycarbamide, Bone marrow transplantation
Experimental: gene therapy, anti P-selectin monoclonal antibody, Crizanlizumab
What is thalassaemia?
Due to reduced globin chain synthesis
Normal: HbA (alpha 2 and beta 2) +small amounts of HbF and A2)
Beta-thalassaemia: Genes on Chromosome 11, Thalassemia major– HbF (alpha 2 and gamma 2) and Hb A2 (alpha 2 and delta 2) – Thalassemia intermedia, Thalassemia minor
Alpha-thalassemia: 2 alpha globin genes on each chromosome 16, Loss of 1 or 2 alpha genes: asymptomatic: alpha Thal Trait, Loss of 3 genes: HbH Disease, Loss of 4 genes: HbBarts Hydrops Foetalis
What is the management of beta thalassaemia?
Genetic counselling Regular blood transfusions Fe overload- complications: Liver, heart, pancreas, endocrinopathy Fe chelation BM Transplantation
What is the prognosis for acute leukaemia in children?
Prognosis >80% cured (age 2-10, female, WCC<50, no CNS involvement)
What are causes of bruising in children?
Trauma: Accidental, non-accidental
Vascular: HSP, scurvy, ehlers danlos
Platelets: ITP, leukaemia, Von willebrand
Coagulation abnormality: haemorrhagic disease of newborn, haemophilia
What is Von willebrand factor?
Secreted by endothelium/platelets
Carrier for FVIII:c
Facilitates platelet adhesion
What tests and results would you expect in Von willebrand disease?
Prolonged bleeding time, PTT
Low FVII:C, vWF, ristocetin cofactor assay
vWF multimer analysis
DNA tests
What are auer rods?
Material seen in cytoplasm of AML cells
Can also be seen in megaloblastic anaemia, leukaemia, iron deficiency anaemia hyposplenism - coeliac, thalassaemia
What are Howell jolly bodies?
Spherical blue black inclusions of red blood cells which represent residual nuclear fragments Commonly seen in asplenic patients Also severe haemolytic anaemia Pernicious anaemia Leukaemia Thalassaemia
What are pappenheimer bodies?
Violet staining granules in periphery of RBCs Sideroblastic anaemia Lead poisoning Thalassaemia Asplenic patients
With which condition are smudge cells associated?
CLL
How is a diagnosis of CLL confirmed?
Lymphocytosis
Characteristic lymphocyte morphology (smudge cells)
Immunophenotyping
What are features of polycythemia rubra Vera?
Hyperviscosity Pruritis (typically after hot bath) Splenomegaly Haemorrhage (2ary to abnormal platelets) Plethoric appearance HTN
What investigations should be done for polycythemia rubra Vera?
FBC Blood film JAK2 mutation Serum ferritin U and Es LFTs If JAK2 negative: red cell mass, arterial O2 sats, abdo USS, serum EPO, bone marrow aspirate and trephine, cytogenetic analysis, erythroid burst forming unit culture
What is the mechanism of action of DDAVP in Von willebrands disease?
Stimulates release of vWF from endothelial cells
Can increase levels long enough for surgery or dental procedures to be performed
Who should be screened for thrombophilia?
Unprovoked DVT or PE with first degree relative also had DVT or PE
What would you expect on a blood film in a patient with G6PD?
Heinz bodies
What is the diagnostic test for hereditary spherocytosis?
Osmotic fragility test
What is the inheritance pattern of G6PD deficiency?
X linked recessive
What are presenting features of hereditary spherocytosis?
Failure to thrive Jaundice Gallstones Splenomegaly Aplastic crisis precipitated by parvovirus MCHC elevated
What is management of hereditary spherocytosis?
Folate replacement
Splenectomy
What blood results would you expect to see with hypersplenism?
Pancytopenia
Raised reticulocytes
What is paroxysmal nocturnal haemoglobinuria?
Acquired clonal defect of red cells resulting in production of erythrocytes with increased susceptibility to lysis by complement
Intermittent passage of bloody urine
Prothrombotic tendency caused by complement induced platelet aggregation
What is protein S?
Vit K dependent anticoagulant which acts as non enzymatic co factor to activated protein c in proteolytic degradation of factor Va and VIIIa
What is factor V Leiden?
Hypercoagulability disorder resulting from variant of factor V which cannot be inactivated by activated protein c
What are features of fanconi anaemia?
Autosomal recessive Aplastic anaemia Increased risk of AML Neurological problems Short stature Cafe au lait spots
What are antibodies directed against in ITP?
Glycoprotein IIb/IIIa complex
What are indications for splenectomy?
Traumatic rupture
ITP
Spherocytosis
What prophylaxis do splenectomy patients need?
Vaccines 4 weeks before: pneumococcus, meningococcus, HIB
Lifelong Pen V
What investigations need to be done for myeloma?
Serum electrophoresis, serum free light chains
Bone marrow examination
Skeletal survey
What does vitamin K do?
Gamma carboxylation of factors II, VII, IX and X
Where does vitamin K come from?
50% from food (leafy vegetables, eggs)
50% from bacteria in gut (why antibiotics affect warfarin control)
Why don’t we use FFP in warfarin reversal any more?
High volume required in order to raise factor levels by small amounts
Which is the only DOAC that can be reversed?
Dabigatran (can be dialysed and has a Mab antidote)
What type of antibodies are anti A/B?
IgM (complement fix)
What is a delayed transfusion reaction?
Later onset, due to minor antibodies in the transfused blood
Secondary immune response
Not ABO incompatibility
What are important questions with regards to spine treatment for vertebral collapse in myeloma?
Have they had radiotherapy?
Have they had vertebroplasty?
What are markers of red cell breakdown?
Bilirubin
Haptoglobin (absent)
LDH
What are markers of increased red cell production?
Reticulocytosis
How do you investigate a cause of Haemolysis?
Blood film
Direct Coombs test (look for antibodies)
What are spherocytes?
Red cells more round as their membrane is lost
E.g. Autoimmune haemolytic anaemia, macrophages eat membrane
What are different causes of haemolytic anaemia?
Congenital: G6PD deficiency, sickle cell, hereditary spherocytosis
Immune: autoimmune, alloimmune (ABO incompatibility)
Non immune: intravascular, DIC, HUS/TTP, mechanical valve, accelerated HTN
What are causes of retinal haemorrhages?
Diabetes
HTN
Haematology: CML/AML hyperviscosity, paraproteinaemia IgM, polycythemia, raised platelet count
What are causes of splenomegaly?
Haem: CML, CLL, ALL, lymphoma
Infective: EBV, chronic malaria, visceral leishmaniasis
Liver: HCV/HBV with portal HTN, cirrhosis
RA
SLE
What is haptoglobin?
Binds free Hb with high affinity and so inhibits oxidative activity
What is the definition of a massive transfusion?
Entire patients blood volume/10 units of blood in 24 hours
What are early and late transfusion complications?
Early: acute haemolysis, bacterial contamination, allergic reaction, anaphylaxis, TRALI, fluid overload, non haemolytic febrile transfusion reaction
Late: iron overload, graft vs host disease, post transfusion purpura, infection
What are complications of a massive transfusion?
Hypocalcaemia
Hypothermia
Hyperkalaemia
Dilutional effect on other blood components eg thrombocytopenia
What are non megaloblastic causes of macrocytic anaemia?
Alcohol Liver disease Hypothyroidism Pregnancy Reticulocytosis
What drug is given to treat B12 deficiency?
Intramuscular hydroxycobalamin (B12)
What are causes of folate deficiency?
Poor diet: vegans
Increased demand: pregnancy
Malabsorption: coeliac disease
Anti folate drugs: methotrexate
What might be examination findings in beta thalassaemia major?
Hepatosplenomegaly Frontal bossing Jaw enlargement Dental malocclusion Flow murmur Generalised pallor Conjunctival pallor
What is the common name for beta thalassaemia major?
Cooleys anaemia
What advice should be given to haemophiliacs?
Wear medic alert bracelet
Avoid NSAIDs and IM injections
Avoid contact sports and other activities
