Presentation on theme: "Dr Nnenna Osuji Dr Stella Appiah-Cubi Dr Stella Kotsiopoulou 21/05/2014."— Presentation transcript:
Dr Nnenna Osuji Dr Stella Appiah-Cubi Dr Stella Kotsiopoulou 21/05/2014
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
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
Monoclonal Protein MGUS Vs Multiple Myeloma Case Discussions CLL Case Discussion
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.
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
– 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
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
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
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
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
71-year old Caucasian 3/12 back pain Spine X-ray – lytic lesion lumbar
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
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)
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 65-70 years. Small proportion are familial Aetiology unknown
Mostly disease of elderly with Often asymptomatic. Classic B symptoms recurrent infections Lymphadenopathy, hepatosplenomegaly. Marrow failure Autoimmune haemolytic anaemia/thrombocytopenia
Persistent lymphocytosis > 5 x 10 9 /l. Morphology Mature looking lymphocytes -clumped chromatin Immunophenotyping BM not required for diagnosis.
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
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
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
Anaemias B12 Deficiency Case Discussion Iron Deficiency Vs Thalassaemia Trait Elevated Ferritin
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?
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
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
Foods of animal origin- meat, fish, eggs, milk, cheese but not in plants. Recommended daily requirement is 1- 2µg/day Total body stores of 2000-5000µg Mainly stored in the liver( up to 2yrs stores)
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
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)
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
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
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
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
53y old legal secretary Previously fit & well A&E with RIF 3/12 general lethargy & inability to concentrate
PMHx- Nil relevant SHx-Single, good diet with mixture of animal & diary products No alcohol or smoking
Pale, mildly jaundiced, sore and smooth tongue. No pedal oedema, no hepatosplenomegally BP- 95/50, Pulse -110/min RIF tenderness with guarding? appendicitis
59yr old female Severe RA On immunosuppressive drugs
Hb- 99 WBC- 13.5 Plt-420 ESR- 43 CRP- 35, Ferritin 400µg/l U&E, LFT- NAD Blood film Normochromic normocytic anaemia Serum electrophoresis- polyclonal increase in immunoglobulin
Cause of raised ferritin- acute phase response No specific therapy/investigations required for this Treat the RA
38yr old German ancestry Fit & well City Banker Alcohol- 10u/week Well man clinic
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%
Test for HFE mutation Homozygous for C282Y/C282Y Diagnosis: Hereditary Haemochromatosis
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
Sickle cell vaccination Community follow up for long term haematological conditions Any others?
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????
Can you name 2 (previously presented in this talk)? Can you suggest others?