Haem

Question 1

What is acute lymphoblastic leukaemia vs chronic lymphoblastic leukaemia
- pathophys
-risk factors

Answer 1

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.

Question 2

What is the signs/ symptoms and investigations and management of
Acute lymphoblastic vs acute myeloid leukaemia

Answer 2

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

Question 3

Definition of acute vs chronic myeloid leukaemia

Answer 3

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)

Question 4

Presentation, progression, exam for chronic myeloid leukaemia

Answer 4

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

Question 5

IX and management + SE for chronic myeloid leukaemia

Answer 5

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

Question 6

Hx, exam, investigations for chronic lymphocytic leukaemia

Answer 6

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

Question 7

RAI staging of leukaemia (o-4) and treatment accordingly for CLL.
what are absolute indications for treatment

Answer 7

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

Question 8

CLL treatment + complications

Answer 8

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

Question 9

What is the difference between hodgkins and non-hodgkins lymphoma - risk factors, pathophys

Answer 9

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)

Question 10

Hx of symptoms, pmHx, investigations performed and treatment undertaken for lymphoma

Answer 10

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

Question 11

Exam for lymphoma and differentials for cervical lymphadenopathy

Answer 11

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

Question 12

Ix and staging of lymphoma

Answer 12

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

Question 13

Management of hodgkins + Non- hodgkins lymphoma

Answer 13

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

Question 14

SE of radiotherapy and chemotherapy in lymphoma treatment

Answer 14

SIDE EFFECTS
Radiotherapy – increased risk of second
malignancies, IHD, hypothyroidism, lung fibrosis

Chemotherapy - myelosuppression, nausea,
alopecia, infection, AML, NH lymphoma, infertility

Question 15

Definition of multiple myeloma, MGUS and signs and symptoms (ROAR)

3-4-year survival, death commonly due to infection
or renal failure.

Answer 15

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

Question 16

Hx, exam and diagnosis of mutliple myeloma

Answer 16

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

Question 17

Ix for multiple myeloma

Answer 17

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

Question 18

Management of multiple myeloma and complications

Answer 18

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

Question 19

What is the history, exam and cut offs for anaemia and levels to transfuse

Answer 19

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

Question 20

What are causes of high MCV. normal MCV and low MCV anaemia

Answer 20

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.

Question 21

IRON deficiency anaemia: what are the main causes, exam signs, investigations and management

Answer 21

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?

Question 22

Patho (3) , causes, Ix, and management of anaemia of chronic disease

Answer 22

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.

Question 23

what does iron , TIBC and ferritin levels in chronic haemolysis , haemochromatosis,
iron def anaemia and chronic disease anaemia

Answer 23

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

Question 24

What is polycythemia rubra vera + hx, exam

Answer 24

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°)

Question 25

Ix and management polycythemia rubra vera

Answer 25

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

Question 26

Hx exam investigations and management of essential thrombocythaemia (too many platelets)

Answer 26

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

Question 27

Myelofibrosis - what is it - 1 and 2, hx, exam, investigations and managmeent

Answer 27

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.

Question 27

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)

Answer 27

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

Question 27

What are the 4 causes of inherited thrombophilia
what are the standard investigations and management - who to investigate

Answer 27

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

Question 27

example of abnormal inhibitor function (thrombophilia) patho phys and measurement

example of increased factor levels patho phys and measurement

Answer 27

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

Question 28

example of inhibitor deficiency patho phys and measurement

Answer 28

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.

Question 29

Immune thrombocytopenic purpura
definition, presentation, investigation

Answer 29

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

Question 30

Management of immune thrombocytopenic purpura

Answer 30

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

Question 31

What are the 3 main pathologies of bleeding disorders and what is the history

Answer 31

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

Question 32

Give differentials for causes of bleeding under the heading of vascular defects, platelet disorders (decreased production/increased destruction/ poor functioning)

Answer 32

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

Question 33

What are the 2 inherited coagulation disorders and the 5 reasons for acquired coagulation disorder

Answer 33

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

Question 34

What are the ix for bleeding disorder - what do they mean

Answer 34

• 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

Question 35

Management of bleeding disorder

Answer 35

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