Haem Flashcards
Leukaemias - ALL, CLL, AML , CML, Lymphoma, multiple myeloma, anaemia (3 types), myeloproliferative disorders, thrombotic disorders, ITP
What is acute lymphoblastic leukaemia vs chronic lymphoblastic leukaemia
- pathophys
-risk factors
Acute lymphoblastic
-Malignancy of B or T lymphocytes causing proliferation of immature blast cells which cause marrow failure and tissue infiltration
- Genetic plus environmental triggers -Association with Down’s syndrome.-Poor prognosis if Philadelphia chromosome
Most common leukaemia and children, very rare in adults –up to 90% cure rates in children, for adults only 40%
Chronic lymphoblastic
- Accumulation of mature B cells that have escaped apoptosis and undergone cell-cycle arrest. Commonest leukaemia.
What is the signs/ symptoms and investigations and management of
Acute lymphoblastic vs acute myeloid leukaemia
SIGNS AND SYMPTOMS
-Marrow failure = anaemia (low Hb), bleeding (low
platelets), infection (low functioning WCC)
-Infiltration = hepatosplenomegaly,
lymphadenopathy, CNS symptoms (meningism,
nerve palsies)
INVESTIGATIONS
-blood film = blast cells
-bone marrow = blast cells
-FBC – high WBC, low Hb and platelets
-CXR and CT – looking for lymphadenopathy
NOTE that for AML - WCC often raised but can be low, fewer blast cells than ALL
MANAGEMENT
-blood/platelet transfusion
-allopurinol (prevents tumour lysis syndrome)
-febrile neutropenia (from disease and treatment) =
immediate IV abx
-chemotherapy
-allogeneic bone marrow transplant
- management and signs and symptoms same for acute myeloid leukaemia
Definition of acute vs chronic myeloid leukaemia
Myeloid leukaemia = Uncontrolled clonal proliferation of myeloid cells (basophils, eosinophils, neutrophils).
ACUTE MYELOID LEUKAEMIA
Commonest acute leukaemia in adults quick onset and symptomatic
- caused byGene mutation in stem cells. Can be a complication of chemotherapy or radiation/ smoking, benzen exposure
CHRONIC MYELOID LEUKAEMIA
- Slower disease progression asymptomatic or constitutional
- Philadelphia chromosome
(tyrosine kinase activity) is present in 80% cases
better prognosis (unlike ALL)
Presentation, progression, exam for chronic myeloid leukaemia
HISTORY
Presentation
- Chronic and insidious
- Weight loss, tiredness, fevers, sweats
- Gout features due to purine breakdown
- Abdominal discomfort and fullness
- Infection
- Thrombotic episodes (stroke, MI, VTE)
Progression
- Fever and infections
- Bone and joint pain
- Haemorrhage or thrombosis
EXAMINATION
- Splenomegaly (massive and common)
- Hepatomegaly
- Anaemia
- Bruising
IX and management + SE for chronic myeloid leukaemia
INVESTIGATIONS
- FBC
o WBC high - neutrophils, basophils,
eosinophils and myelocyte
o Normochromic anaemia
o Low neut alk phos score
- Bone marrow aspirate
o Hypercellular
Cytogenetic analysis
o Philadelphia chromosome
MANAGEMENT
Chemotherapy: Imatinib – tyrosine kinase inhibitor
causing apoptosis of cells expressing BCR/ABL.
SE: hepatotoxicity, myalgia, fluid retention
Stem Cell Transplant (only curative tx): Allogenic
bone marrow transplant.
- First line in young
patients + those intolerant to imatinib
Hx, exam, investigations for chronic lymphocytic leukaemia
HISTORY
- Often nothing – incidental finding on bloods
- Recurrent infections
- Anaemia
- Painless lymphadenopathy
- LUQ discomfort from splenomegaly
EXAMINATION
- Fever
- Anaemia (haemolytic)
- Enlarged, tender, rubber nodes.
- Splenomegaly + hepatomegaly
INVESTIGATIONS
- FBC
o high lymphocytes >5x109
/L
o Later, decreased Hb, neutrophils and platelets secondary to bone marrow
infiltration
- Blood film
o Normal appearing lymphocytes
o No immature blast cells - Bone marrow
o Heavily infiltrated with lymphocytes - Direct Coomb’s test
o May be positive if haemolytic anaemia
RAI staging of leukaemia (o-4) and treatment accordingly for CLL.
what are absolute indications for treatment
0 – Lymphocytosis alone : Watch and wait
I – lymphocytosis + lymphadenopathy
II - lymphocytosis + spleno or hepatomegaly
- 1-2 treat if progression
III – Lymphocytosis + anaemia (HB < 110mg/L)
IV – Lymphocytosis + platelets < 100
- treatment indicated
Absolute indications for treatment:
- Marrow failure
- Recurrent infection
- Systemic symptoms
- Massive splenomegaly or lymphadenopathy
- Haemolysis
CLL treatment + complications
Supportive
- Steroids, transfusions,
IV Ig, prophylactic
antimicrobial, allopurinol for
hyperuricaemia
Specific
- FCR (fludarabine, cyclophosphamide and
rituximab)
- Allogenic stem cell transplantation
COMPLICATIONS
- Autoimmune haemolysis
- Infections due to hypogammaglobinemia
(low IgG)
- Bacterial/viral (zoster) infections
- Marrow failure
What is the difference between hodgkins and non-hodgkins lymphoma - risk factors, pathophys
Hodgkin’s Lymphoma:
- Characteristic Reed-Sternberg cells (mirror image nuclei)
- Risk factors = family hx, EBV, SLE, obese, - 2 peaks of incidence – young adults and elderly
- Types = Nodular sclerosing (70%), Mixed cellularity, Lymphocyte rich, lymphocyte depleted (worse prognosis)
Non-Hodgkin’s Lymphoma:
- Diverse: All lymphomas without Reed-Sternberg
cells
Risk factors = HIV, immunodeficiency, EBV,
toxins, congenital
- B (most common) and T cell lines. Include extra nodal tissue generating
lymphoma (MALT – mucosa associated lymphoid tissue e.g. gastric MALT) - Low Grade – indolent, widely disseminated
and incurable e.g. follicular and lymphocytic lymphoma (≈CLL) - High Grade – more aggressive, short history
of rapidly enlarging lymphadenopathy and
systemic symptoms e.g. Burkitt’s lymphoma
and lymphoblastic lymphoma (≈ALL)
Hx of symptoms, pmHx, investigations performed and treatment undertaken for lymphoma
HISTORY
Symptoms
- superficial, rubbery, painless nodes,
fluctuating size, typically cervical
- constitutional upset – fever, weight loss, night sweats, lethargy, fatigue
- mediastinal involvement – bronchial or SVC obstruction, pleural effusions
- extranodal disease of oropharynx (waldeyer’s ring causing sore throat and
obstructed breathing), bone, gut, CNS, lung (Only NH) - Infection and bleeding from pancytopenia
PMHx
- history of infection
- Predisposing conditions such as Kleinfelder’s, HIV, autoimmune disease,
immunosuppressive drugs, use of phenytoin.
Investigations Performed
- Lymph node biopsy
- CT or MRI
- Bone marrow aspirate
Treatment Undertaken
- Chemotherapy +/- Radiotherapy
- Peripheral stem cell transplants
Exam for lymphoma and differentials for cervical lymphadenopathy
EXAMINATION
- Cachexia
- Anaemia
- Lymph node enlargement
- Spleno/hepatomegaly
- Extra nodal involvement
DIFFERENTIAL DIAGNOSIS OF CERVICAL LYMPH
NODE ENLARGMENT
- Infection – acute pyogenic, mono, CMV, TB, sarcoidosis, HIV
- Autoimmune – rheumatoid arthritis
- Drugs - Phenytoin
- 1° lymph node malignancies – lymphoma,
chronic lymphocytic leukemia, acute
lymphoblastic leukaemia
- 2° malignancies – nasopharyngeal, thyroid,
lung, melanoma, breast, stomach
Ix and staging of lymphoma
INVESTIGATIONS
- Bloods – FBC, ESR differential count, film
Electrolytes, renal function, LFT’s,Ca
LDH (in high cell turnover)
- Imaging – CXR for mediastinal widening,
CT/PET chest/abdo/pelvis - Bone Marrow Biopsy
- Excision Biopsy
- Cytology of any effusion
- Cytology of CSF if CNS signs (NonH)
STAGING
Ann Arbor Staging System
I – confined to single lymph node
II - ≥ 2 nodal areas, same side of diaphragm
III - ≥ 2 nodal areas, both sides of diaphragm
IV – Spread beyond lymph nodes
Addition to each stage:
A – no systemic symptoms other than pruritis
B – B symptoms (weight loss, night sweats, fevers)
E – Extranodal disease
S – spleen involved
X – bulky nodal disease - nodal mass >1/3 of intrathoracic diameter
Management of hodgkins + Non- hodgkins lymphoma
Hodgkin’s Lymphoma 80% 5 yr survival
Stage Ia-11a – radiotherapy + short chemotherapy
Stage IIa-IVb – chemotherapy + radio in young pts
Relapsed disease – high dose chemo + peripheral
stem cell transplantation
Chemo Regime – (ABVD) Adriamycin, Bleomycin,
Vinblastine, Dacarbazine
Non-Hodgkin’s Type 40% 5 yr survival
Low Grade –
- Radiotherapy in localized disease,
- chemotherapy with chlorambucil in diffuse disease with a-interferon or rituximab for maintenance of remission
High Grade – (R’CHOP) Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin
(vincristine), Prednisolone. GCSF helps with
neutropenia
SE of radiotherapy and chemotherapy in lymphoma treatment
SIDE EFFECTS
Radiotherapy – increased risk of second
malignancies, IHD, hypothyroidism, lung fibrosis
Chemotherapy - myelosuppression, nausea,
alopecia, infection, AML, NH lymphoma, infertility
Definition of multiple myeloma, MGUS and signs and symptoms (ROAR)
3-4-year survival, death commonly due to infection
or renal failure.
DEFINITION
- Malignant proliferation of a single clone of plasma cells (derived from B cells), producing identical immunoglobins (IgG > IgA > IgM).
The other Ig levels are low immunoparesis) increasing susceptibility to infections.
- Some myeloma begin as MGUS (monoclonal gammopathies of uncertain significance).
o <30g/L monoclonal peak, <10% plasma cells in marrow, no lytic bone lesions
o This is present in 3% of the
population, 1% of which will become Multiple Myeloma. MGUS is indolent and does not require treatment.
Signs and symptoms can be related to ROAR:
R – Renal Impairment
o Light chain deposition which precipitates with Tamm-Horsfall protein in distal loop of Henle.
o Monoclonal Ig induce changes in glomeruli causing glomerulonephritis
O – Osteolytic Bone Lesions
o Myeloma cells signal increased osteoclast activation with hypercalcaemia
A – Anaemia, Neutropenia, Thrombocytopenia
o Result from bone marrow infiltration by plasma cells
R – Recurrent Bacterial Infections
o Due to immunoparesis and neutropenia from disease and chemotherapy
Hx, exam and diagnosis of mutliple myeloma
HISTORY
- Bone – pain, which is worse on movement,
pathological fractures
- Hypercalcaemia – abdo pain, vomiting, diarrhea, polyuria/dipsia, depression, weight loss, fatigue, weakness
- Renal – colic from stones, nephrotic
- Anaemia – SOB, pale, tired
- Bleeding – bruising, GI bleeding
- Hyperviscosity – headache, visual changes, reduced cognition
- Bacterial infections – UTI and pneumonia
- Skin – pruritis, purpura
EXAMINATION
- Weight loss, pallor, bruising
- Lymphadenopathy and splenomegaly rare
- Signs of chest infection
- Signs of spinal cord compression
- Bony tenderness and deformity (kyphosis)
DIAGNOSIS
1) Monoclonal protein band on serum or urine electrophoresis
2) Increased plasma cells found on bone marrow
biopsy
3) Evidence of end organ damage
o Hypercalcaemia
o Renal insufficiency
o Anaemia
o Bone lesions on skeletal survey
Ix for multiple myeloma
Bloods
- FBC – N/N anaemia, rouleaux formation on
film, high ESR
- U+E - High urea, creat, Ca
- Serum electrophoresis for serum free light chains and immunoglobins
Urine
- Urine electrophoresis for serum free light chains (bence-jones proteinuria) and immunoglobins
Imaging
- Skeletal survey – X-ray of chest, cervical, thoracic and lumbar spine, skull and pelvis
- CT or MRI may detect lesions not seen on XR
Bone Marrow
- Trephine or aspirate
- Look for B2 microglobulin
Management of multiple myeloma and complications
Supportive
-Analgesia for bone pain (Avoid NSAIDS)
- Bisphosphonate to reduce fracture risk, local radiotherapy for focal disease
- Transfusions and erythropoietin for anaemia
- Hydration and bicarbonate for renal disease
- Treat infections with broad spectrum until sensitivity obtained
- Regular IV immunoglobulin infusions
Chemotherapy
- Intensive if young with minimal comorbidities, older more comorbid patients unsuitable for intensive treatment.
Stem-cell transplant in younger patients.
COMPLICATIONS
-hypercalcaemia – IV bisphosphonates
-spinal cord compression - dexamethasone
-hyperviscosity - plasmapheresis
-AKI – rehydration +/- dialysis
What is the history, exam and cut offs for anaemia and levels to transfuse
HISTORY
- Fatigue, SOB, faintness, palpitations, HA, tinnitus,
anorexia, angina (if pre-existing CAD).
EXAMINATION
-Often nothing on examination
- Conjunctival or palmar pallor
- Severe: hyperdynamic flow- tachycardia, flow
murmur (ESM loudest over apex), carotid enlargement.
- Later; HF can occur (transfusion here can be fatal)
Hb levels
<135g/L men; <115 women
Reduced production or increased loss.
TRANSFUSIONS
-most people will not need a transfusion unless
<70g/L or very symptomatic
What are causes of high MCV. normal MCV and low MCV anaemia
TYPES
Low MCV – microcytic anaemia
- Iron deficiency (most common)
- Thalassemia
- Sideroblastic anaemia (rare)
Normal MCV – normocytic anaemia
- Acute blood loss
- Anaemia of chronic disease (or decr MCV)
- Bone marrow failure (WCC and plt decr also)
- Renal failure
- Hypothyroidism (or incr MCV)
- Haemolysis (or incr MCV)
- Pregnancy
High MCV – macrocytic anaemia
- B12/folate def
- alcohol excess – or liver disease
- reticulocytosis
- cytotoxins
- Myelodysplastic syndrome
- Marrow infiltration
- Antifolate drugs (MTX, phenytoin)
Suspect haemolytic anaemia if bilirubin is up.
IRON deficiency anaemia: what are the main causes, exam signs, investigations and management
IRON DEFICIENCY ANAEMIA
Causes:
- Blood loss: menorrhagia, GI bleeding
- Poor diet
- malabsorption: coeliac
- Hookworm
Signs;
- koilonychia
- atrophic glossitis, angular stomatitis
Ix:
- microcytic, hypochromic w anisocytosis & poikilocytosis
- low ferritin; low serum Fe; increased TIBC
NB: ferratin is APR and will incr w inflam
- serum transferring receptors increased (less
affected by inflammation).
- GI loss: scopes
Management;
- If Hx menorrhagia: given oral iron without further Ix
- Oral ferrous sulfate
SE: abdo discomfort, C, D, black stools
Hb should rise 10g/L/week, some reticulo
Continue until Hb normal (3months)
- IV iron
concordance issues?
why stopping them? GI?
may be continued blood loss, malabs, chronic
disease. Misdiagnosis? Thalassemia?
Patho (3) , causes, Ix, and management of anaemia of chronic disease
3 problems:
- Poor use of iron in erythropoiesis
- Cytokine-induced shortening of RBC survival
- Decr production of & response to EPO
Causes:
Vasculitis, RA, malignancy, renal failure
Ix:
- mild normocytic anaemia
- ferritin normal or increased.
- Do blood film, B12, folate, TSH, haemolysis tests
Management:
- Tx underlying disease more vigorously
- EPO effective at raising Hb level
SE: flu-like Sx, HTN, mild raised plt, VTE
Improves QoL in those with malignancy
- Parenteral iron can overcome functional iron def.
what does iron , TIBC and ferritin levels in chronic haemolysis , haemochromatosis,
iron def anaemia and chronic disease anaemia
chronic haemolysis ,
- high iron, low or normal TIBC, high ferritin
haemochromatosis,
high iron, low TIBC, high ferritin
iron def anaemia
- low iron, normal or high TIBC, low ferritin
chronic disease anaemia
- normal/ low iron, normal or low TIBC, low ferritin
What is polycythemia rubra vera + hx, exam
Polycythaemia rubra vera = cancer in which bone
marrow makes too many RBCs.
History
- Hyperviscosity – headaches, dizzy, tinnitus,
visual disturbance
- Itch after hot bath, erythromelalgia, abdo pain, epistaxis, gout
- Hx of arterial (MI, CVA, PVD) thrombus
or venous (DVT, hepatic) thromboembolism
- Sx of diseases causing 2° polycythaemia
(high alt, chronic lung disease, cyanotic
heart disease, renal disease e.g. RCC or hydronephrosis)
Exam
- Facial plethora, cyanosis
- Scratch marks, bruising, gout, PVD
- Cardioresp for potential underlying cause
- Massive Splenomegaly (in 1° not 2°)
Ix and management polycythemia rubra vera
Investigations
- FBC - inc Hb, HCT, PCV, WBC, platelets
- Inc Red cell mass, decr serum erythropoietin
- Inc Vit B12
- Marrow shows erythroid hyperplasia
- JAK2 in 90%
Management
- Venesection (bleeding)
- Hydroxyurea or interferon
- Low dose aspirin
Splenomegaly and low serum erythropoietin
distinguish from secondary polycythaemia
Hx exam investigations and management of essential thrombocythaemia (too many platelets)
History
- Abnormal bleeding – GI and bruising
- Arterial or venous thromboembolism
- Microvascular occlusion – headaches, atypical chest pain, light-headed,
erythromylalgia
Exam
- Bruising
- Modest splenomegaly
Investigations
Exclude other causes
- Bleeding
- Infection, chronic inflammation
- Malignancy
- Trauma/post-surgery
- Iron Deficiency
- inc platelets, often > 1000x109 /L
- Prolonged bleeding time
- Prolonged APTT if acquired VWF def
- JAK 2 in 50%
Management
- Don’t treat it asymptomatic. Bleeding
problems more common if pt >1million, thrombosis problems more common if
pt<1million
- aspirin
- hydroxyurea or interferon
- tranexamic acid for bleeding problems
Myelofibrosis - what is it - 1 and 2, hx, exam, investigations and managmeent
Description
Fibrosis of bone marrow and myeloid metaplasia in
liver, spleen and other organs.
1° - hyperplasia of megakaryocytes which release fibroblast stimulating factors
2° - late in the course of PCV or ET
History
- lethargy, weakness, weight loss, fever
- Infections, easy bleeding, thrombotic events
- Abdo discomfort, due to hepato and splenomegaly
- Bone and joint discomfort
Exam
- Anaemia, massive splenomegaly, fever,
bruising
Allogenic stem cell transplant.
Investigations
- Anaemia with tear drop cells on film
- Leucoerythroblastic cells (nucleated RBCs)
- Bone marrow trephine showing fibrosis
Management
- Marrow support – RBC and platelet transfusion
This may transform to acute leukemia.
What are 3 areas of virchows triad and examples contributing to each.
What are the 7 risk factors for VTE (arterial clotting rf are cvd risk factors)
VIRCHOWS TRIAD
- Stasis of blood flow
o Immobility, pressure on vessel, AF, increased viscosity
- Hypercoagulability
o Increased procoagulants, decreased inhibitors - Changes to blood vessel wall
o Trauma, surgical, prior thrombosis, atherosclerosis
VTE risk factors
- Active cancer
- Recent surgery or trauma
- Immobility
- Obesity
- Pregnancy
- Hormone replacement/COP
- Varicose veins
What are the 4 causes of inherited thrombophilia
what are the standard investigations and management - who to investigate
Abnormal inhibitor function
inhibitor deficiency - protein c, s def, antiphospholipid
increased factor levels -
dysfibrinogenemia (rare)
INVESTIGATIONS
-investigate those who have had unprovoked DVT/PE
-FBC, blood film, clotting (PT, APTT, thrombin time),
DNA analysis
MANAGEMENT
-treat clots as usual (heparin then warfarin/dabigatran)
-lifelong warfarin if more than one unprovoked VTE
example of abnormal inhibitor function (thrombophilia) patho phys and measurement
example of increased factor levels patho phys and measurement
Factor V Leiden (main cause)
Factor V is normally inactivated by protein C by cleaving the Factor V molecule
Factor V Leiden is a variant form meaning it is resistant to cleavage
Protein C is thus unable to act as an anticoagulant.
Measured by coagulationbased assays and direct DNA analysis
Increased factor levels
o Prothrombin 20210 mutation
Mutation in the promoter region of the prothrombin gene which leads to excessive production of prothrombin
o Elevated factor VIII
example of inhibitor deficiency patho phys and measurement
Inhibitor deficiency
o Antithrombin deficiency
Targets 9, 10, 11, thrombin
o Protein C deficiency
Targets V and VIII
o Protein S deficiency
Targets V and VIII
o Perform functional assays if abnormal perform antigenic (ELISA) based assays.
Immune thrombocytopenic purpura
definition, presentation, investigation
DEFINITION
Anti-platelet Ab - purpura is small vessel bleeding
PRESENTATION
- Acute - children, 2wks after viral infection w sudden self-limiting purpura.
- Chronic – (mainly women), fluctuating course; bleeding, purpura (pressure areas),
epistaxis, menorrhagia. No splenomegaly
INVESTIGATIONS
Bloods - incr megaK in marrow, anti-plts Ab
Management of immune thrombocytopenic purpura
Management
- none, if mild
- If symptomatic or plt <20 – prednisone 1mg/kg/d and reduce after remission
aim to keep plts >30 – takes a few days
- Splenectomy cures 80% if relapses
- Immunosuppression: Azathioprine,
cyclophosphamide
Other
- plt transfusion - only for life-threatening bleed or splenectomy (because plt quickly
destroyed by Ab).
- IV Ig may temporarily raise plt for surg/preg
- Eltrombopag new oral TP-R agonist
What are the 3 main pathologies of bleeding disorders and what is the history
Main causes
1) Vascular defects affecting vasoconstriction
2) PLatelet disorders affecting platelet aggregation
3) Coagulation disorders affecting coagulation cascade
History
- Bleeding – bruising, nose bleeds, postsurgical/traumatic bleeding, joint bleeds,
heavy periods.
- Need for blood transfusions
- Family history
Give differentials for causes of bleeding under the heading of vascular defects, platelet disorders (decreased production/increased destruction/ poor functioning)
Vascular Defects
Inherited: CT disease- ehler danlos)
Acquired
- Infection: o Meningococcal, o Measles
- Henoch-Schoenlein Purpura
Decreased production of platelets
- Viral infection
- Drugs (cytotoxic/ radiotherapy)
- Bone Marrow failure : aplastic anaemia/megaloblastic anaemia, marrow infiltration)
Increased destruction
- Autoimmune - ITP , SLE
- Non-immune: DIC, TTP, Sequestration
Poor functioning
- Myeloproliferative disease
NSAIDS
elevated urea
What are the 2 inherited coagulation disorders and the 5 reasons for acquired coagulation disorder
Haemophilia - x linked, treat with replacement
o Type A – Factor 8 deficiency
o Type B – Factor 9 deficiency
VW disease - Autosomal dominant - vw helps platelets to bind to eachother, the subendothelium and stops destruction of factor 8.
Acquired
- Anticoagulant drugs (warfarin)
- Vit K deficiency - leafy greens, malabsopriton
- Liver disease -decreased factor production
- DIC - widespread activation of coagulation
- Massive blood loss
What are the ix for bleeding disorder - what do they mean
- Platelet count
- Megakaryocytes in marrow
o Low – decreased production
o High – increased destruction - Antiplatelet antibodies if suspecting
autoimmune - APTT (Activated Partial Thromboplastin
Time)
o Intrinsic and Common Pathways
o All factors except 7
o Factors 8, 9, 11, 12 only assessed
through APTT
o If prolonged, add plasma
If corrects factor deficiency
If remains prolonged inhibitor present e.g Lupus Anticoagulant
o Unfractionated heparin prolongs - PR (Prothrombin Ratio)
o Extrinsic and Common Pathways
o Assesses factor 2, 5, 7, 10, fibrinogen
and prothrombin
o If PR high and APTT normal, must
be factor 7 that is affected
o Warfarin monitoring - TCT (Thrombin Clotting Time)
o Fibrinogen deficiency
o Thrombin inh e.g. heparin and
dabigatran - Factor assays
Management of bleeding disorder
Control any bleeding and eliminate the inhibitor.
- Reverse or stop any anticoagulants
- Give steroids and immunosuppressives in acute presentations
- Avoid giving plasma/blood if possible, but FFP is given if required
- Educate patients around increased bleeding risk
