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Dr Nnenna Osuji Dr Stella Appiah-Cubi Dr Stella Kotsiopoulou 21/05/2014.

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Presentation on theme: "Dr Nnenna Osuji Dr Stella Appiah-Cubi Dr Stella Kotsiopoulou 21/05/2014."— Presentation transcript:

1 Dr Nnenna Osuji Dr Stella Appiah-Cubi Dr Stella Kotsiopoulou 21/05/2014

2  Focus on primary/secondary care interface ◦ Discuss cases encountered and practical lessons ◦ Highlight diagnostic clues ◦ Encourage confidence around community care of chronic stable haematological malignancies  Information gathering ◦ Sickle vaccinations ◦ USC referrals

3  Name the current consultants.  How do you contact haematology for advice?  Blood film suggests CLL. How are you going to refer this patient?  Indications for 2WR Haematology Referrals

4  Monoclonal Protein  MGUS Vs Multiple Myeloma  Case Discussions  CLL  Case Discussion

5  It is a monoclonal immunoglobulin secreted by an abnormally expanded clone of plasma cells that can be detected by immunofixation of serum and/or urine.

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7  Varies greatly with age. ◦ 1 - 2% of people in their 6th decade ◦ 2-4% in their 7th decade ◦ 4-5% in their eighth decade ◦ 14% over the age of 90  Racial variation ◦ Twice as common in black people as white people

8 – Haematological malignancies –Multiple myeloma –Solitary plasmacytoma (skeletal or extra- medullary) –Waldenstrom's macroglobulinaemia –Low grade non-Hodgkin’s lymphoma – Monoclonal gammopathy of undetermined significance ( MGUS) – AL amyloidosis

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10  There were only 2 statistically significant risk factors for progression ◦ The concentration of monoclonal protein ◦ The type of monoclonal protein  IgA and IgM gammopathy more likely than IgG to progress  IgM rarely becoming myeloma

11  Low risk defined as  IgG M-protein <15g/l  IgA or IgM M-protein <10g/l  No symptoms/signs of myeloma, lymphoproliferative disorders or AL amyloidosis This group forms the vast majority of M proteins detected in routine practice

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13 New International Staging System: ISS Stage CriteriaMedian survival IBeta2 microglobulin<3.5 mg/L and Serum albumin >3.5 g/dl 62 mo. IINeither I or III45 IIIBeta2 microglobulin >5.5 mg/L29

14  Symptoms of bone disease, persistent unexplained backache  Impaired renal function  Anaemia  Hypercalcaemia  Recurrent or persistent bacterial infection  Hyperviscosity  Spinal cord/nerve root compression  Symptoms suggestive of amyloidosis (nephrotic syndrome, cardiac failure)  High ESR (incidental finding)

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18  67-year old Afro-Caribbean  Right hypochondrial pain  Fatigue  Recently treated for epistaxis

19  FBC: Hb 6.6, WBC 5.0, plts 85  Urea 10.2, creatinine 128  Adj. Ca 4.38, albumin 28  ESR 138 mm/h

20  Admit  Anaemia due to ◦ ? Malignancy ◦ ? Epistaxis  3 units of blood transfusion  i.v. fluids + Pamidronate

21  IgA λ paraprotein 75 g/l (t.prot 127 g/l)  β2 microglobulin 5.6mg/l  BM – plasma cells 40%  INR 1.32, APTTr 1.34, fibrinogen 1.67 g

22  Drowsy  Transfer to ITU  Bleeding from mouth  HD Methylprednisolone  Subsequent C-VAD, Z-Dex chemotherapy

23 EMERGENCY !!! Hyperviscosity Treatment – plasmapheresis, HD steroids, iv hydration

24  49-year old Afro-Caribbean  Back pain - 9 mo. (6 mo. off work)  Not able to mobilize from bed without help

25  FBC – clotted  Urea 6.5, creatinine 150  Adj. Ca 2.68, albumin 19  Total protein 174 g/L  Paraprotein 141 g/L IgGκ

26  Hb 3.4 (citrate blood)  INR 1.5, APTTr 1.25, fibrinogen 1.41

27  3 sessions of plasmapheresis at RMH  Blood transfusion after plasmapheresis  Z-Dex chemotherapy  Abnormal ECHO  CThal Dex chemotherapy

28  48-year old Afro-Caribbean  Sudden lower-back pain on sneezing 3 mo. prior to admission  5/7 history back pain and reduced power in left leg

29  FBC: Hb 10.5, WBC 6.8, plts 278  Urea, creatinine, adj. Ca - N  T. protein 99  paraprotein 45 g/L IgGκ

30 05/08/2005  Left leg – no movement  Right leg – reduced power

31 Spinal cord compression Treatment – radiotherapy, Dexamethasone

32  1 st August – Lumbar spine X ray  2 nd August – MRI  2 nd August – CT- guided biopsy  4 th August - diagnosis ?MM, ?NHL  4/5 th August – referral to haematology  5 th August - referral for radiotherapy  6 th August - radiotherapy

33  20 Gy – 5 doses  1 course Z-Dex, 3 courses C-VAD  !! Able to take few steps with crutches ◦ Right leg – back to normal ◦ Left leg – much improvement

34  71-year old Caucasian  3/12 back pain  Spine X-ray – lytic lesion lumbar

35  FBC: Hb 15.6, N 5.7, plts 237  Us&Es, Ca, t. protein – normal  Small IgG paraprotein  BM – 3% plasma cells  CT-guided biopsy – plasma cells

36 PLASMACYTOMA Treatment 40 Gy in 20 fractions

37 SURGERY  Contraindicated in the absence of structural instability  Management of persistent pain ◦ Vertebroplasty – bone strengthening and pain relief but does not restore height (polymethacrylate) ◦ Kyphoplasty – vertebral height can be restored (small inflatable balloon)

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41  Clonal B cell malignancy.  Progressive accumulation of long lived mature lymphocytes  Most common leukemia of Western world.  Male to female ratio is 2:1.  Median age at diagnosis is years.  Small proportion are familial  Aetiology unknown

42  Mostly disease of elderly with  Often asymptomatic.  Classic B symptoms  recurrent infections  Lymphadenopathy, hepatosplenomegaly.  Marrow failure  Autoimmune haemolytic anaemia/thrombocytopenia

43  Persistent lymphocytosis > 5 x 10 9 /l.  Morphology Mature looking lymphocytes -clumped chromatin  Immunophenotyping  BM not required for diagnosis.

44 Majority score 4/5 Score < 3 not CLL

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46 Complications  Infection ◦ Bacterial – hypogammaglobulinaemi a ◦ Viral – T-cell dysfunction (H. Zoster)  Autoimmune – ITP/Haemolytic anaemia  Other malignancies  Transformation ◦ Richter’s, Hodgkin’s

47  Many watch and wait – stage A  Chemotherapy ◦ Bone marrow failure=stage progression ◦ B symptoms – weight loss, sweats, fevers (unexplained) ◦ Symptoms from enlarged lymph nodes/spleen ◦ Transformation ◦ Not for high WBC alone  Median survival 10 years

48 When to refer back  B symptoms  Lymphadenopathy  Hepatosplenomegaly  Falling haemoglobin, platelets, rapidly rising lymphocyte count  Recurrent infections  Autoimmune complications How often? Every 3 months Then reduce frequency if stable

49  2004 Aged 80, Routine FBC- lymphs 8. Normal Hb/neutrophils/plats  Well, no B symptoms, lymphadenopathy or hepatosplenomegaly  Diagnosis CLL  ‘Watch and wait ’ policy  Discharged 2011 – GP to monitor

50  Anaemias  B12 Deficiency  Case Discussion  Iron Deficiency Vs Thalassaemia Trait  Elevated Ferritin

51  Definition  Categorized according to Red Cell indices MCV and MCH: ◦ Hypochromic and Microcytic (low indices) ◦ Normochromic and Normocytic (normal indices) ◦ Macrocytic (high MCV)  Can you name one cause for each category?

52  How do we investigate?  1 st line tests: B12, Folate, TSH, reticulocyte count, LDH, LFTs, blood film: ? Any dysplastic features. ? History of alcohol consumption  2 nd line tests: SPE, UPE, DAT/haemolysis screen  3 rd Line: Bone Marrow investigations

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54  Sources of dietary vitamin B12  Vitamin B12 absorption  Causes of B12 deficiency  Indications for measuring B12 level  Clinical presentation of B12 deficiency  Investigations to help define the cause of B12 deficiency.  A guide to management

55  Foods of animal origin- meat, fish, eggs, milk, cheese but not in plants.  Recommended daily requirement is 1- 2µg/day  Total body stores of µg  Mainly stored in the liver( up to 2yrs stores)

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58 Inadequate vitamin B12 in diet ▪ strict vegans Vitamin B12 malabsorption ▪ Pernicious anaemia-loss of GP→IF↓( increased of CA stomach ~ 2-3%) ▪ Long term use of PPI or H2-antagonist ▪ Chronic alcoholism ▪ Coeliac disease ▪ Small bowel( esp. terminal ileal) surgery ▪ Generalised malabsorption e.g. Tropical sprue, IBD ▪ Blind loop syndrome +/- small bowel bacterial overgrowth ▪ Fish tapeworm Drugs ▪ Biguanides e.g.. Metformin ▪ Oral contraceptive pill ▪ Slow K, Cholestyramine

59  Haematological ▪ Isolated red cell macrocytosis ▪ Macrocytic anaemia ( esp. if MCV> 110fl) ▪ Pancytopenia ( esp. if MCV> 110fl)  Neurological or psychiatric ▪ Peripheral neuropathy ▪ Cognitive change e.g. dementia ▪ Optic neuritis  Gastrointestinal ▪ Investigation of possible malabsorption process  Other (rare) ▪ Angular stomatitis, glossitis (sore beefy red tongue)

60  Slow onset – symptoms of mild anaemia  Pallor and mild Jaundice( ineffective erythropoesis)  Glossitis & angular stomatitis  Neurological changes( B12< 60ng/L)( SCDC) ▪ Glove and stocking parasthesia ▪ Early loss of vibration sense ▪ Progressive weakness and ataxia ▪ Dementia

61  FBC-Megaloblastic anaemia with hypersegmented neutrophils  Serum B12 level- low  Serum folate may be normal or high  Anti-intrinsic factor ( anti-IF)- highly specific but +ve in 50-60% of PA.  Parietal cell antibodies- +ve in 90%( PA) but less specific  TSH & anti-thyroid Ab  Test for coeliac disease  Test for generalised malabsorption  Endoscopy  Schilling test- obsolete

62  B12 deficiency without neurological involvement: ▪ 1mg Hydroxocobalamin 3 times a week for 2 weeks then every 3 months  B12 deficiency with neurological involvement: ▪ 1mg Hydroxocobalamin every other day until no further improvement then every 2 months ▪ Folic acid 5mg daily for 4 weeks

63  Serum B12> 150ng/l- Confirm test  Significant proportion of these patients will go on to become symptomatic.  Treat with oral vitamin B12 supplements + monitor level every 2-3months  If no response then consideration given to parenteral B12 replacement

64  53y old legal secretary  Previously fit & well  A&E with RIF  3/12 general lethargy & inability to concentrate

65  PMHx- Nil relevant  SHx-Single, good diet with mixture of animal & diary products  No alcohol or smoking

66 Pale, mildly jaundiced, sore and smooth tongue. No pedal oedema, no hepatosplenomegally BP- 95/50, Pulse -110/min RIF tenderness with guarding? appendicitis

67  FBC- Hb- 6.8g/dl, WBC- 2.5 Plt- 58, MCV- 130fl,  U&E- NAD, LFT-Normal except Bili=26( 21- …), LDH- 8498, TSH- Normal, Hepatitis, HIV –ve.

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71  Folate- 9  B  GP & IF ab- Positive  Coeliac Screen Negative

72  Vitamin B12 replacement- 1mg X 3 for 2 weeks.  Then 3 monthly  Folic acid 5mg daily for 4 weeks

73  South East Asian origin  Saw GP with tiredness, SOB O  PMHz- II DM on metformin  SHx- Banker, strict vegetarian, Alcohol o, smoking o  O/E- Unremarkable

74  Hb WBC- 3.2 Plt- 223, MCV- 120, LFT-NAD, LDH-640, U&E-NAD, TSH- Normal  Blood film- macrocytosis with occasional hypersegmented neutrophils seen  Serum B , Folate- 12, Ferritin 45  GP & IF ab, Coeliac screen- Negative

75  Oral supplementation  Cyanocobalamin µg PO daily  Recheck serum B12 in 2-3 months.  Continue if replete  If not, consider parenteral B12 injections.

76  48y old Female  Long H/O bloatedness  Good diet.  SHx- Research nurse, no smoking or alcohol

77  O/E- Unremarkable  Bloods- Hb-9.8 WBC- 7.8 Plt-350 MCV-86fl  Blood film unremarkable  U&E, LFT, TSH- NAD

78  Ferritin- 6µ/dl, serum B12 – 160, Folate- 8  Tissue transglutaminase (tTG)

79  Referred to gastroenterologist  Given oral B12 and FeSO4  Recheck 3 monthly

80  What tests would you consider in the following cases: (Fer Vs Fer+HbEP)  25 y.o. lady, Norwegian, fatigued, Hb:100 g/l, RBC: 3000, MCV: 74fl, MCH: 26pg  50 y.o. lady, Cypriot, routine check, Hb:100 g/l, RBC 5000, MCV 70fl, MCH 24 pg

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84  Low Ferritin = Iron Deficiency  Raised Ferritin = Too much iron??

85  Iron overload( Primary & Secondary)  High Ferritin Without Iron Overload

86  Primary ▪ Hereditary haemochromatosis ▪ Hereditary aceruloplasmin( Wilsons’ disease)  Secondary ▪ Transfusion overload ▪ Ineffective erythropoiesis( thalassemia, sideroblastic anaemia) ▪ Excess dietary iron

87  Liver disease  Alcohol excess  Chronic inflammatory conditions( RA, IBD, bacterial infection)  Malignancy

88  FBC  LFT  Ferritin  Transferrin saturation

89  59yr old female  Severe RA  On immunosuppressive drugs

90  Hb- 99 WBC Plt-420 ESR- 43 CRP- 35, Ferritin 400µg/l  U&E, LFT- NAD  Blood film Normochromic normocytic anaemia  Serum electrophoresis- polyclonal increase in immunoglobulin

91  Cause of raised ferritin- acute phase response  No specific therapy/investigations required for this  Treat the RA

92  38yr old German ancestry  Fit & well  City Banker  Alcohol- 10u/week  Well man clinic

93  Hb- 154 WBC- 8.7 Plt- 210  Ferritin- 200µg/l  B12/Folate- within normal range  U&E, LFT-NAD  O/E- Nicely tanned but unremarkable otherwise  Transferrin saturation ( serum Fe & TIBC)- 89%

94  Test for HFE mutation  Homozygous for C282Y/C282Y  Diagnosis: Hereditary Haemochromatosis

95  Elevated Fe with no obvious cause  Family history  TS > 55% for men & post menopausal women  TS > 50% in premenopausal woman  Test for HFE mutation  Refer patient to gastro-enterologist in CUH  Venesection will be done by haematologist

96  Sickle cell vaccination  Community follow up for long term haematological conditions  Any others?

97  Pneumococcal Vaccine every 5 years  Hib immunisation (once in lifetime)  Tetravalent Meningococcal vaccine ACWY (once in lifetime)  Hepatitis B vaccination  Seasonal Flu Vaccination (annually)  WHY????

98  Can you name 2 (previously presented in this talk)?  Can you suggest others?

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100 Thank you! Dr Stella Kotsiopoulou Haematology Specialty Doctor Croydon University Hospital


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