Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition...

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Anaemia Nov 2010

Transcript of Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition...

Page 1: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Anaemia

Nov 2010

Page 2: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Overview

Iron deficiency anaemiaMacrocytic anaemiaHaemolytic anaemia

Recognition and management of bleeding disorders

Page 3: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Case history (1)

45yr old female3 month history of fatigue and shortness of breath on exertionO/E pallor ++FBC – Hb 6.2g/dl

Page 4: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Case history (1)

What further results are important in the full blood count?What further details are important in the clinical history and examination?What further investigations should be carried out?How should the patient be managed?

Page 5: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Full Blood Count

Hb 6.2 g/dl (12-16)WCC 7.0 x 109/l (4-11)Platelets 300 x 109/l (140-440)MCV 60fl (76-96)MCH 24 pg (27-32)

WCC differential –NBlood Film

Page 6: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Some causes of microcytic anaemia…..

AcquiredIron deficiencyAnaemia of chronic diseaseMyelodysplastic syndromesLead poisoning

Page 7: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

More causes of microcytic anaemia…..

InheritedThalassaemiaSickle cell trait

Page 8: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

What further details are important in the clinical history?

Dietary intake of ironSymptoms of malabsorption / weight lossOvert GI blood lossMenorrhagiaPregnancyOral iron therapyBleeding history/ family history of bleeding disorder

Page 9: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Iron Requirements

Males 0.5-1mg per dayMenstruating females 1-2mg per dayPregnant females 1.5-2.5mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron, 10% of which is absorbed.

Page 10: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Dietary iron

Red meat , liver, beansAbsorbed in the duodenum and jejunumAbsorption enhanced by ascorbic acid, citrus fruitsAbsorption reduced by phytates, alkalis, tea, tetracyclines

Page 11: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Causes of iron deficiency anaemia

Inadequate intake

Failure of absorption

Increased blood loss

Increased requirements

Dietary ironIron supplementsGastrointestinal symptomsHx of Coeliac diseaseOvert blood loss from bowel or change of bowel habitMenorrhagiaPregnancy

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Common causes of gastrointestinal bleeding

Oesophagus

Stomach

Small bowel

Hiatus herniaVaricesGastritis Ulcer CarcinomaUlcerMeckels diverticulumCarcinoma

Page 13: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Common causes of gastrointestinal bleeding

Colon

Rectum

Ulcerative colitisCarcinoma Diverticulitis

HaemorrhoidsUlcerationCarcinoma

Page 14: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Iron loss in pregnancy

Obligatory iron loss 150-200 mgFetal iron 200-370 mgIron in placenta and cord 30-170 mgIron in blood lost at delivery 90-310 mg

Total iron loss 470-1050mg

Page 15: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

What further investigations should be carried out?

Serum ferritin +/- serum ironB12 / folateFaecal Occult Blood+/- Coeliac screen+/- Gastroscopy and/or colonoscopy+/- Gynaecology referral

Page 16: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Causes of raised ferritin levels

Acute inflammationAcute liver diseaseLymphomasSolid tumoursHaemochromatosis

Page 17: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

How should the patient be managed?

Treat the underlying causeOral iron supplements

correct anaemiareplenish iron stores

IV ironmalabsorptionintolerance

Is there a role for blood transfusion?

Page 18: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Failure to respond to oral iron

Is the diagnosis correct?Is the patient taking the iron?Is there evidence of malabsorption?Is there evidence of persistent blood loss?

Page 19: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Case (2)

45yr old female3 month history of fatigue and palpitationsO/E pallor ++FBC – Hb 5.3 g/dl

Page 20: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Case 2

What further results are important in the full blood count?What further details are important in the clinical history and examination?What further investigations should be carried out?How should the patient be managed?

Page 21: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Full Blood Count

Hb 6.2 g/dl (12-16)WCC 7.0 x 109/l (4-11)Platelets 120 x 109/l (140-440)MCV 120fl (76-96)MCH 28pg (27-32)

WCC differential –NBlood Film

Page 22: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Some causes of macrocytosis…...

Megaloblastic anaemiaVitamin B12 deficiencyFolate deficiencyMyelodysplasia

Page 23: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

More causes of macrocytosis….

Liver diseaseAlcohol excessHypothyroidismCytotoxic drugs

Page 24: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

What further details are important in the clinical history?

DietSymptoms of malabsorption / weight lossFamily history of anaemia or autoimmune disordersThyroid diseaseAlcohol intake

Page 25: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

What further investigations should be performed?

Blood filmB12, Folate, FerritinLiver function testsThyroid function tests

Coeliac screenIntrinsic factor and parietal cell antibodies?Bone marrow – only if above normal

Page 26: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Vitamin B12

Sources – liver meat fish and dairy productsDaily intake 3-30 microgramAdult daily requirement 1-2 microgramBody stores 3-5 mg in the liver (2-4 yr supply)Important for pyrimidine synthesis in the production of DNA

Page 27: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Vitamin B12 absorption

B12 attaches to intrinsic factor (IF) in the stomachIF – a glycoprotein secreted by the parietal cellsB12/IF passes to the terminal ileum where absorption takes place

Page 28: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Causes of B12 deficiency

Strict vegetarianismMalabsorption

Pernicious anaemiaGastrectomyCoeliac diseaseDisease involving the terminal ileum

ResectionCrohn’s disease

Page 29: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Pernicious anaemia

Autoimmune diseaseGastric atrophyAnti parietal cell antibodies 90%Anti intrinsic factor antibodies 70%

Often associated with other autoimmune disorders

Page 30: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

B12 deficiency – clinical features

Related to anaemiaNeurological

Peripheral neuropathyLoss of vibration and position senseDemyelination of the cordIrreversible

Page 31: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Management

Lifelong replacement with B12 usually requiredIM Hydroxocobalamin 1000 microgram every 3 months

Page 32: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Folate

Dietary sources- eggs, green vegetables, liver, nutsAbsorbed in the jejumunDaily intake 600-700microgramDaily requirement 100 microgramStored in the liver (4-6 months supply)Important in DNA synthesis

Page 33: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Causes of folate deficiency

Dietary – infancy and old ageMalabsorption – coeliac diseaseIncreased utilisation – pregancy, lactation, haemolytic anaemiaAntifolate drugs – methotrexate, anticonvulsants

Page 34: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Management of folate deficiency

Treat underlying causeCorrect folate levels : oral folic acid 5-15mg dailyProphylactic folate to at risk groups eg pregnancy, congenital haemolytic anaemias

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Haemolytic Anaemias

Page 36: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemolytic anaemia

Normal red cell life span 100-120 days

A haemolytic anaemia occurs if that life span is shortened

CongenitalAcquired

Page 37: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Mrs C

31 year old femaleHistory of recent “viral illness”C/O increasing tirednessNoticed to look jaundiced by her family

Page 38: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Mrs C

What laboratory investigations should be carried out?

How should the patient be managed?

Page 39: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Mrs C –laboratory results

FBC – Hb 7.7 g/dl Blood film – polychromasiaRaised reticulocyte countRaised bilirubinRaised lactate dehydrogenase (LDH)

Page 40: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemolytic Anaemia

How would you investigate the patient?

What are the causes of acquired haemolytic anaemia?

Page 41: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Idiopathic Secondary

A u to im m u ne A llo im m u ne D rug ind uced

Im m une N on Im m une

Acquired Haem olytic anaem ia

Page 42: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Autoimmune haemolytic anaemia

Warm AntibodyIdiopathicSecondary

CLLConnective tissue disordersLymphomasDrugs eg Methyldopa

Cold AntibodyIdiopathicSecondary

MycoplasmaInfectious mononucleosisLymphoma

Page 43: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Alloimmune Haemolytic Anaemia

Haemolytic transfusion reactions eg ABO mismatch

Haemolytic disease of the newborn eg Rhesus incompatibility

Page 44: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Non immune haemolytic anaemia

Red cell fragmentation

Infections

Chemical/ Physical

DIC, Cardiac valvesHUS, TTPMarch haemoglobinuria

Malaria, Clostridium

Drugs, chemicalsVenomsBurns

Page 45: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Clinical features of haemolytic anaemias

Symptoms related to anaemiaJaundiceIncreased incidence of pigmented gallstonesSplenomegalyLeg ulcers- sickle cell, hereditary spherocytosis

Page 46: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Warm autoimmune haemolytic anaemia

Antibody usually IgG – maximum activity @370

Any age, either sexSplenomegaly commonBlood film – microspherocytes, polychromasiaDirect Coombs test (DCT) positive

Page 47: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Warm autoimmune haemolytic anaemia

ManagementTreat the underlying causeCorticosteroidsSplenectomy Other immunosuppressants eg azathioprine, cyclosporinBlood transfusion – if life threatening

Page 48: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Cold autoimmune haemolytic anaemia

Antibody usually IgM – maximum activity @40

Raynauds PhenomenonPositive DCT – complementCold agglutinins – agglutination on blood film

Page 49: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Cold autoimmune haemolytic anaemia

ManagementTreat the underlying causeKeep the patient warmConsider immunosuppression

Page 50: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemolytic anaemia and infections

Direct damage to cells eg malariaToxin production eg clostridiumOxidant stress in G6PD deficiencyDIC eg meningococcusAutoantibody formation eg infectious mononucleosis

Page 51: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Inherited haemolytic anaemias

Red cell membrane defects

Disorders of red cell metabolism

Abnormal haemoglobins

Page 52: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Peter M

10 yr oldLife long history of recurrent anaemia and jaundiceFather gives similar history and required cholecystectomy when he was 20yr

Page 53: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Peter M – Laboratory investigations

Hb 9.2g/dlBlood film – microspherocytes, polychromasiaElevated bilirubinDCT negativeOsmotic fragility testing- increased haemolysisHereditary Spherocytosis

Page 54: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Hereditary Spherocytosis

Autosomal dominantVariable severityDefect in a red cell membrane proteinCells destroyed prematurely in the spleenSplenomegaly is common

Page 55: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Hereditary Spherocytosis

Splenectomy for severe casesIncreases the red cell survivalDefer until >6yrs

Folic acid

Page 56: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Abnormalities of Red Cell Metabolism

Glucose-6-phosphate dehydrogenase deficiencyPyruvate Kinase deficiency

Page 57: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Inherited haemolytic Inherited haemolytic anaemiasanaemias

Red cell membrane defects

Disorders of red cell metabolism

Abnormal haemoglobins

Page 58: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Abnormal Haemoglobins

Disorders of globin chain synthesis – the thalassaemias

Structural defects of haemoglobin eg sickle cell disease

Page 59: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease - Pathogenesis

Chronic haemolytic anaemia caused by a point mutation in the globin gene Causes insolubility of Hb in the deoxygenated stateInsoluble chains crystallise in the red cells causing sicklingVascular occlusion

Page 60: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease – clinical features

Vaso-occlusive crisesCommon precipitants are infection, dehydrationBone pain StrokeVisceral infarction – spleen, kidneysDactilytis - children

Page 61: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease – clinical features

Sequestration crisesSickling with pooling of red cells in liver or spleen – severe anaemia, rapid enlarging liver or spleenAcute chest syndrome – chest pain, hypoxia, diffuse shadowing on CXR

Page 62: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease – laboratory features

Hb 7-9 g/dlSickle cells on blood filmAbnormal haemoglobin electrophoresis

Page 63: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease – management

GeneralAvoid known precipitantsFolic acidVaccinate, prophylactic penicillin (reduced splenic function)

Page 64: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease – management

Vaso-occlusive crisesHydrationAnalgesia – opioidsAntibiotics if infection

Page 65: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Sickle cell disease – management

TransfusionFor severe anaemiaRed cell exchange

Severe crisesLung sequestrationStokeHepatic sequestration

Page 66: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Other sickling disorders

Sickle cell traitBenign conditionUsually asymptomaticAdvice re carrier state

Page 67: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Thalassaemias

2 globin genes4 globin genes

Autosomal recessive disordersCharacterised by ineffective haemopoiesis

Page 68: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Thalassaemias

ThalassaemiaSeverity depends on the number of genes deletedTrait – 1 or 2genes deleted, mild anaemia, hypochromic microcytic filmHb H disease – 3 genes deleted , splenomegaly, Hb 6-10g/dl, hypochromic microcyticHyrdrops fetalis – 4 gene deletion, death in utero

Page 69: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Thalassaemias

ThalassaemiaTrait – mild hypochromic microcytic anaemia, advice re carrier state thal major

Severe anaemia @ 3-6 months whem switch is made from fetal HbHepatosplenomegalyExpansion of bones

Page 70: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Thalassaemias

Laboratory featuresSevere anaemiaHypochrominc microcytic cells, target cellsBM erythroid hyperplasiaDNA analysis

Page 71: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Thalassaemias

ManagementRegular transfusionIron chelationBone marrow transplantation

Reduced life expectancy

Page 72: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

BLEEDING DISORDERSRecognition and continuing

care

Page 73: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

TISSUE FACTOR

+TFVIITISSUE FACTOR

COMPLEXXI

IXX

VIII

V

PROTHROMBINTHROMBIN

VII VIIa

FIBRINOGEN

Va

VIIIa

XIa

IXaXa

TRIGGER

FIBRIN

Page 74: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Vesselinjury

PlateletRelease rxn

PlateletAggregation

Vasoconstriction CoagulationCascade

StableHaemostatic Plug

Page 75: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Recognition of Bleeding Disorders

Page 76: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Bleeding History

Personal historyEpistaxisBleeding post surgeryBleeding post dental extractionMenorrhagiaHistory of anaemiaEasy bruising

Family history NB

Page 77: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.
Page 78: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.
Page 79: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

The Bleeding history

Coag. DeficienciesProlonged bleeding after trauma and surgery (>24 hrs).Haemarthroses Muscle bleeding.

Platelet defects and VWD:Bruising.Petechiae or purpura.Epistaxis.Menorrhagia Prolonged post-trauma bleeding

Page 80: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Investigations

Page 81: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Investigation of bleeding disorders

FBC - platelet countProthrombin time (PT) - factors V, VII, XActivated partial thromboplastin time (APTT)- factors VIII, IX, XI, XIIFibrinogen

Page 82: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Investigation of bleeding disorders

Von Willebrand FactorSpecific clotting factor assaysPlatelet function testing

Page 83: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Treatment of bleeding disorders- general principles

Avoid IM injections and NSAIDsAvoid delay in treating the patient. Treat on suspicion of a bleedListen to the patient - he/she has lifelong experienceRecord any treatment given including batch numbers to ensure full traceability of factor concentratesSeek help early

Page 84: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Treatment of bleeding disorders- general principles

VaccinationAgainst Hepatitis A and BGive by SC routeChidhood vaccinationsCheck antibody levels annually

Page 85: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemophilia

Haemophilia A VIII deficiency sex-linked, 1/3rd carriers <50% VIIIC.1:20,000 births

Haemophilia B IX deficiencysex-linked, 1/3rd carriers <50% IX.1:100,000

These are clinically indistinguishable

Page 86: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemophilia

Mild haemophilia ( 5 - 20 %): – bleed only with trauma and surgery.

Severe haemophilia( < 1%) : – Haemarthroses 2-8 times/month.– Muscle bleeds.– Intracerebral bleeding– Prolonged bleeding with trauma and

surgery.

Page 87: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

HAEMOPHILIA - HAEMARTHROSIS

Page 88: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemophilia Care

Treatment of acute bleedsManagement of HIV and Hep C infectionManagement of arthropathyDental review / dental hygieneAnnual clinic reviewVaccination

Page 89: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemophilia treatment

A bleed or potential bleed requires immediate treatmentIf in doubt manage as a bleed Identify site of suspected bleed and assess for compression of vital structuresAvoid unnecessary investigations - eg Xrays, coagulation profiles – unless clinically indicated

Page 90: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemophilia treatment

FACTOR VIII and IX

Previously plasma derived

Now all recombinant in Ireland

Delivered directly to patients homes

Page 91: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Haemophilia - treatment

Moderate and severe bleeds need admission

Daily factor concentrateImmobilisationAnalgesiaPhysiotherapy

Page 92: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Von Willebrand Disease

19265yr old girl – died at 13yr during 4th menstrual period4 siblings died from gastrointestinal haemorrhageBoth parents had significant bleeding historyVWF – identified 1950s, purified 1972, sequenced 1985

Page 93: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Von Willebrand Disease

Up to 1% of the population125 / million have a clinically significant bleeding disorderAutosomal inheritance

Page 94: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Von Willebrand factor

Large glycoprotein produced by endothelial cells and megakaryocytes Mediates platelet to endothelial adhesion Mediates platelet to platelet interactionCarrier protein for Factor VIII

Page 95: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

Von Willebrand Disease

MILD/MODERATE BLEEDING TENDANCY

mucocutaneous bleedingeasy bruisingepistaxismenorrhagiarecurrent iron deficiencyfamily history

Page 96: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

VWD diagnosis

Coag screen often normal

VWF - quantiative assays

VWF Ricof - functional assay

Page 97: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

VWD - diagnosis

Levels increased by menstrual cycle, OCP, pregnancy, smoking, stress, inflammatory disorders

Repeat sampling recommendedequivocal resultsminor abnormalitiesstrong personal or family history

Page 98: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

VWD Treatment

Avoid NSAIDsAvoid IM injectionsVaccinate against Hepatitis A and BTreat anaemiaDental hygiene

Very few patients require treatment with clotting factor concentrate

Page 99: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

VWD TREATMENT -Specific measures

Clotting factor concentrates

DDAVP

Cyclokapron

Page 100: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

DDAVP

Promotes release of VWF and factor VIII from endothelial cells0.3ug/kg in 100mls N/Saline over 30 minsAverage response is a threefold rise in VWF and FVIIITreatment of choice in responsive patients for spontaneous bleeding , trauma or minor surgeryIntra nasal DDAVP

Page 101: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.

VWD TREATMENT -Specific measures

Cyclokapron Antifibrinolytic agentStabilises clotGiven orallyProvides adequate cover for minor procedures or dental work

Page 102: Anaemia Nov 2010. Overview Iron deficiency anaemia Macrocytic anaemia Haemolytic anaemia Recognition and management of bleeding disorders.