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Haematology in Primary Care Dr Josh Wright Consultant Haematologist Sheffield Teaching Hospitals.

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Presentation on theme: "Haematology in Primary Care Dr Josh Wright Consultant Haematologist Sheffield Teaching Hospitals."— Presentation transcript:

1 Haematology in Primary Care Dr Josh Wright Consultant Haematologist Sheffield Teaching Hospitals

2 Referral Hit Parade Raised Hb Leucocytosis- neutrophilia/lymphocytosis Thrombocytosis/thrombocytopeniaParaproteinsmacrocytosis Low B12 Anaemia in the elderly Microcytosis/ iron deficiency/alpha thal Microcytosis/ iron deficiency/alpha thal

3 Common haematological issues in primary care Lecture ► Interpreting the blood count ► Common referral issues  White cell problems  Platelet problems  Paraproteins Workshop ► Red cell issues  Erythrocytosis  Haematinics inc low B12 ► Haemoglobinpathy inc alpha thal ► Anaemia in the elderly ► Any cases brought for discussion

4 Full blood count Normal range Adult male Range Hb Hct RBC count MCV80-98 MCH WBC x10 9 /l Neutrophils Lymphocytes Platelets Adult female RangeHb Hct WBC Neutrophils Lymphocytes Platelets

5 What is the definition of normal?

6 White Blood Count Lymphocytosis History Male age 58 years Hypertension, Type II DM Smokes 10 cigs Drugs Simvastatin, Amlodopine Exam BMI 38 Bp 140/85 FBC September 2011 October 2011 November 2011 Hb WBCLymphocytesNeutrophils Platelets

7 Lymphocytosis ► Does the patient have haematological cancer? ► Should I refer to haematology? ► Should I continue to monitor the lymphocyte count? ► Are there any other tests I should do?

8 Lymphocytosis ► Primary  Lymphoid malignancies ► Chronic Lymphocytic Leukaemia ► Lymphoma ► Monclonal B Lymphocytosis ► Reactive  Viral (EBV, CMV, HSV, VZV)  Stress Lymphocytosis ► Drug induced ► Septic shock ► Myocardial infarct ► Trauma ► Other co-morbidities ► Chronic  Cigarette smoking  Autoimmune disorder  Chronic inflammation  Sarcoid  Raised BMI/metabolic syndrome

9 Malignant Lymphocytosis ? Lymphocyte count more than 10x10 9 /L Lymphocyte count less than 10x10 9 /L Refer to haematologist anaemia or thrombocytopenia recurrent infection? adenopathy, spleen, liver? Abnormal blood film? Yes No Repeat FBC, review 1yrly FBC

10 The Neutrophil Count

11 White Blood Count Neutrophil Leucocytosis History Male age 68 years Osteoarthritis, CABG 2001 Non smoker (stopped 2001) Type 2 DM Drugs Simvastatin, Aspirin, Gliclazide ExamUnremarkable FBC July 2009 December 2010 May 2011 May 2011 Hb WBCLymphocytesNeutrophils Platelets

12 Neutrophil leucocytosis ► Acute neutrophilia  Reactive ► Infection/Inflammation ► neoplasia ► Bleeding ► Pain ► Smoking ► Drugs (glucocorticoids) ► Chronic neutrophilia  Reactive  Drugs  BMI/metabolic syn  Haematologic  Eg CML 1/100000

13 (Neutrophil) Leucocytosis Important points ► Urgent referral ► >50 ► Blood film features of CML or CMML (film comment) ► Consider if ► Chronic neutrophilia>20 ► Chronic monocytosis >1 ► Chronic eosinophila>2 History & Exam infection, inflammation, autoimmune, neoplasia rash, arthritis, weight loss CRP, U&E, LFT, TFT Ca Auto Ab Micro culture FBC, ESR CXR Urine Reactive screen negative Haematology referral

14 Neutropenia ► Caucasian female aged 36yrs, no significant medical history, no regular medication ► FH rheumatoid arthritis ► Hb 126 g/l ► WCC 3.1 ► Neutrophils 0.4 ► Platelets 180

15 Neutropenia (Neutrophils<1.7) What do I need to know? ► Is the patient unwell? (Viral assoc transient) ► Any previous counts? ► Any other cytopenia? ► Is the patient on chemotherapy? ► Other drugs ► How severe is it?  Mild ( ), functionally normal  Moderate ( )  Severe (<0.5)-RISK OF INFECTION

16 Neutropenia What are the common causes? ► Infection ► Drugs (chemotherapy!) ► Autoimmune ► Hereditary  Racial (African origin )

17 Neutropenia <1.0 Patient on chemotherapy Discuss with haematologist and refer Unwell ? Moderate or Severe <0.5 No B12, Folate, LFT, GGT, autoAb Haematology advice/referral Neutropenia pathway Yes Consider repeat particularly if recent infection

18 Thrombocytosis ► Male 65yrs, mild hypertension on ramipril. ► Hb 160, Hct 0.50, WCC 11.0 plts 450 ► Refer or not?

19 Thrombocytosis ► Maybe reactive to inflammation, infection other malignancy, iron deficiency or bleeding ► In MPD very high counts >1500 assoc with vascular events and bleeding ► Urgent referral  >1000  if assoc with CVA, TIA, VTE ► Consider if  >600 consistently  >450 with vascular event  High wcc or Hb

20 Thrombocytopenia ► >100 functionally normal ► <20 bleeding risk rises but most symptom free ► Causes ► Immune ► Drugs eg quinine ► Bone marrow failure syndromes eg MDS ► ALCOHOL ► Liver disease ► Pregnancy

21 Thrombocytopenia ► Refer ► <50 ► if other cytopenia or planned surgery/dental work ► Investigations ► Blood film- platelet clumping ► Repeat sample ► Renal & liver function ► Clotting screen ► Autoantibodies

22 ?Myeloma History 68yr female, Back pain 4 months ESR 70 X Ray normal ► ► Hb 110 ► ► WCC & plts normal ► ► U&E normal ► ► Total Protein 66 (60-74) ► ► IgG * ► ► IgA 0.7 ( ) ► ► IgM 0.5 ( ) ► ► MIg 7.0 ► ► IgG Kappa monoclone Should I refer to haematology? Reminder…….Monoclonal or polyclonal?

23 Paraproteins ► Refer if  IgG>15g, IgA>10g  IgD or E  Lower levels if assoc with CRAB ► IgM usually assoc with lymphoma  Any other features?  Paraprotein>10g  ?Hyperviscosity ► C- Hypercalacemia ► R- unexplained renal failure ► A-anaemia/cytopenia ► B-bone pain/fracture

24 Paraproteinaemia ► ► Most will be MGUS ► ► Present 3% over 70 and 5% over 80 ► ► 1% risk of progression to MM per annum ► ► A few secondary to auto-immune disease & rarely other malignancies ► ► If criteria for referral not met then monitor on a 6-12 monthly basis.

25 Common Red cell Problems ► Macrocytosis ► Erythrocytosis ► Haematinic assessment ► Anaemia in the elderly ► Haemoglobinopathies including alpha thal carriage

26 Macrocytosis ► Approx 8% population ► Commonest causes alcohol & hypothyroidism  B12/ folate deficiency  Drugs (including those used for HIV)  Reticulocytosis  Paraprotein  Myelodysplasia (elderly population & associated with cytopenias)

27 Macrocytosis Raised MCV >100 Are there cytopenias? Check B12, Folate,, LFT, GGT, TFT, Igs, Refer to Haematology Yes No Isolated macrocytosis Monitor if screening normal

28 Erythrocytosis??

29 Erythrocytosis ► Apparent  Dehydration  Diuretics  Alcohol  Raised BMI ► True Physiological  Hypoxia eg COPD, sleep apnoea  High altitude  Smoking ► True none physiological  Primary polycythaemia  Certain rare tumours  Anabolic steroid usage URGENT REFERRAL Hct >60 males or 0.56 females

30 Erythrocytosis Male Hct >0.52 for at least 2 months Female Hct >0.48 for at least 2 months Refer to Haematology Incidence of polycythaemia rubra vera ~5/ Incidence of apparent polycythaemia~ HIGH!

31 Haematinics ► There is no entirely reliable blood test for iron status ► There is no entirely reliable blood test for folate ► There is no entirely reliable blood test for B12

32 ► Ferritin is the test of choice ► Inaccurate in the presence of inflammation ► A trial of iron remains a valid approach ► ALCOHOL/FATTY LIVER & FERRITIN ► Serum folate is a good reflection of what you have just eaten ► I’m not sure what B12 is a good reflection of! ► Interpret borderline values with caution ► Schilling test no longer available

33 Reduced serum B12 ► B12< 100 True deficiency highly likely parenteral replacement ► B replace especially if raised MCV, cytopenia, neuropathy ► B12>140 deficiency unlikely ► B12 is an unreliable test ► Always reduced in pregnancy ► Uncomplicated B12/folate deficiency does not require OP referral.

34 Anaemia in the elderly ► Frequency of anaemia increases with age ► Incidence 10-40% ► Many have diagnosable/treatable cause ► 25% no identified cause ► Investigations ► FBC & film ► Reticulocytosis ► Renal/ liver function ► Immunoglobulins ► Haematinics ► Coeliac screen ► Dietary history ► GI investigations ► Bone marrow

35 Anaemia in the elderly CAUSE OF ANEMIAPERCENTAGE OF CASES Anemia of chronic disease30 to 45 Iron deficiency15 to 30 Posthemorrhagic5 to 10 Vitamin B 12 and folate deficiency 5 to 10 Chronic leukemia or lymphoma 5 Myelodysplastic syndrome5 No identifiable cause15 to 25

36 The lost 25%? ► MULTIFACTORIAL ► Decline in erythropoetin ► Decline in androgens ► Increased inflammatory cytokines even in absence of a recognised disease ► Age associated decline in stem cell function ► Early MDS without other cytopenias or BM changes

37 Indications for haematology referral Pancytopenia Monoclonal gammopathy Suspicion of myelodysplastic syndrome Blood smear showing immature white cells or nucleated red cells Indeterminate status of iron stores Unexplained progressive or unresponsive anemia

38 What are haemoglobinopathies? ► Commonest single gene disorders worldwide ► Autosomal recessive ► Disease states homozygous or combined heterozygotes ► Defects of quality or quantity of haemoglobin ► QualityVariant Hbs eg sickle ► QuantityUnder production eg thals

39 National Screening Plan ► NHS Plan commits to “a new national linked antenatal & neonatal screening programme for haemoglobinopathy & sickle cell disease by 2004” ► Universal neonatal ► Antenatal ?selective ?universal

40 The haemoglobinopathy screen ► Haemoglobin ► MCV, MCH ► Hb A2 ► HPLC ► ZPP/ferritin ► Sickle solubility test, electrophoresis, molecular analysis

41 Case study ► 32 year old female of Pakistani origin attends c/o fatigue ► Hb 11.9, MCV 71, MCH 23 ► Ferritin 109 ► Hb A2 normal, no evidence of  thal

42

43  thalassaemia

44  thalassaemia

45

46  thalassaemia heterozygotes  0  + ► Cypriot2% ► Indiarare ► Africanrare ► Hong Kong5% ► China 3-9% ► Phillipines10% ► UK Africans25% ► India5-58% ► PNG20-80% ► Thailand3-17% ► Maori5-10%

47 Summary points ► Other than for individuals of E. Med or SE Asian origin  thal trait is insignificant ► Microcytosis is commonly due to thal trait ► A national antenatal/neonatal screening programme is now in place


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