Phase 2 Jamie McConnell and Erin Whyte The Peer Teaching Society is not liable for false or...
-
Upload
allan-bishop -
Category
Documents
-
view
216 -
download
0
Transcript of Phase 2 Jamie McConnell and Erin Whyte The Peer Teaching Society is not liable for false or...
Phase 2
Jamie McConnell and Erin Whyte
The Peer Teaching Society is not liable for false or misleading information…
Haematology
• Anaemias• Leukaemias• Lymphomas• Myeloma
The Peer Teaching Society is not liable for false or misleading information…
aims
The Peer Teaching Society is not liable for false or misleading information…
Anaemia• What does anaemia mean?
• A reduction in the concentration of haemoglobin in the blood.
The Peer Teaching Society is not liable for false or misleading information…
Anaemia• What are the symptoms of anaemia?
• Pallor (subconjunctival)• Fatigue• Tachycardia• Breathless• Koilonychia
• 65 y/o man presents with a 2 month history of fatigue and weight loss. FBC shows he has a haemoglobin of 60g/l (normal 130-180g/l)
• What does this tell you?
The Peer Teaching Society is not liable for false or misleading information…
Case 1
• Anaemia ?cause
• Haematologically, what other information would you like to know?
The Peer Teaching Society is not liable for false or misleading information…
Case 1
• Haematinics: folate, B12 and ferritin.
• Our patient has a normal folate and B12, but ferritin is low at 7mcg/l.
• What is the diagnosis and most likely cause?
The Peer Teaching Society is not liable for false or misleading information…
Case 1
• Iron deficiency anaemia secondary to colon carcinoma
• What does iron deficiency anaemia look like on a blood film?
The Peer Teaching Society is not liable for false or misleading information…
Case 1
The Peer Teaching Society is not liable for false or misleading information…
Case 1
NormalAnaemic
• Iron deficiency anaemia is a hypochromic, microcytic anaemia.
• This is due to reduced iron content - haemoglobin gives RBCs their colour and volume
• Reduction in iron → reduction in haemoglobin
The Peer Teaching Society is not liable for false or misleading information…
Case 1
• 21 y/o lady presents with fatigue. • Hb 47g/l• What other information would you like?• Haematinics! B12 & folate normal, ferritin low• Diagnosis and most likely cause?• Iron deficiency anaemia 2ndary to
menorrhagia.
The Peer Teaching Society is not liable for false or misleading information…
Case 2
• 34 y/o lady presents with 3 months of fatigue. She has a history of coeliac disease. Hb is low…
• Haematinics! B12 and ferritin are normal, folate is low.
• Diagnosis and most likely cause?• Folate deficiency 2ndary to malabsorption due
to coeliac disease.
The Peer Teaching Society is not liable for false or misleading information…
Case 3
The Peer Teaching Society is not liable for false or misleading information…
Case 4• 34 y/o lady presents with 3 months of fatigue.
She has a history of hypothyroidism. Hb is low…• Haematinics! Folate and ferritin are normal,
B12 is low.• Diagnosis?• Pernicious anaemia
• Autoimmune destruction of parietal cells in the stomach
• Parietal cells produce intrinsic factor which is required for absorption of B12 in terminal ileum
• ↓ parietal cells → ↓ intrinsic factor → ↓B12
The Peer Teaching Society is not liable for false or misleading information…
Pernicious Anaemia
• What do RBCs in folate and B12 deficiency look like on a blood film?
• Macrocytic
• B12 and folate are involved in DNA synthesis
The Peer Teaching Society is not liable for false or misleading information…
B12 and folate
• Replace what’s missing:– Iron (e.g. oral ferrous sulphate)– B12 (im hydroxocobalamin)– Folate (oral folic acid)
The Peer Teaching Society is not liable for false or misleading information…
Management
• 47 y/o woman with 7 year history of RA is found to have a mild anaemia on FBC.
• What is the diagnosis?• Anaemia of chronic disease• What would you expect to see on blood film?• Normochromic, normocytic
The Peer Teaching Society is not liable for false or misleading information…
Case 5
• Patient on dialysis is anaemic.
• What is the pathophysiology?• Inadequate erythropoietin production by failing
kidneys• What would you see on a blood film?• Normochromic normocytic
The Peer Teaching Society is not liable for false or misleading information…
Case 6
• 12 month old Italian boy presents with failure to thrive. He has mild jaundice and lacks energy. On examination he has splenomegaly and subconjunctival palor.
• What is the probable diagnosis?• B Thalassaemia major
The Peer Teaching Society is not liable for false or misleading information…
Case 7
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• 2 main types of haemoglobin
• HbA (adult haemoglobin A2B2)• HbF (Foetal haemoglobin (A2G2)
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• Alpha-thalassaemia is dysfunction of alpha chain.• 4 genes control production of alpha chain. • being a carrier of 1 or 2 dysfunctional genes produces
a mild anaemia which would probably go unnoticed.• Carriers of 3 dysfunctional genes develop a severe
haemolytic anaemia• Having 4 dysfunctional genes is incompatible with life
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• In B thalassaemia there are no problems initially as foetal heamoglobin compensates
• As you grow up, your requirement for adult haemoglobin increases resulting in an increasingly severe anaemia
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• 2 genes produce b chains. • Being a carrier of one dysfunctional gene produces
"thalassaemia trait" which is a mild, haemolytic anaemia
• Being a carrier of 2 dysfunctional genes is B-thalassaemia major - severe haemolytic anaemia.
• Why do you get jaundice and frontal bossing?
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• Jaundice is a result of haemolysis (haem - biliverdin - bilirubin)
• Frontal bossing is due to extramedullary haemopoiesis
• How would you treat this?
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• Blood transfusion• Risks of transfusion?• Haemochromatosis• How would you prevent that?• Chelation
The Peer Teaching Society is not liable for false or misleading information…
Thalassaemia
• 7 y/o boy from Ghana presents with acutely painful left hip. He is slightly anaemic.
• Likely diagnosis?• Sickle cell• What would you see on his blood film?• Sickle cells• How is this condition inherited?• Autosomal recessive
The Peer Teaching Society is not liable for false or misleading information…
Case 8
The Peer Teaching Society is not liable for false or misleading information…
Any questions about anaemia?
• What are the symptoms of bone marrow failure?
• Anaemia (low RBC)• Immunocompromised (remember SPUR -
severe, persistent, unusual and recurrent infections)
• Bleeding, bruising, petechiae (low PLT)
The Peer Teaching Society is not liable for false or misleading information…
Bone Marrow Failure
• Neoplasm of the bone marrow• 2 broad types: acute and chronic• Further split into lymphoid or myeloid
The Peer Teaching Society is not liable for false or misleading information…
Leukaemia
The Peer Teaching Society is not liable for false or misleading information…
• Presentation of acute leukaemia?– Bone marrow failure– Systemically unwell (usual cancer stuff)– Rapidly progressive - death in weeks/months
if untreated
The Peer Teaching Society is not liable for false or misleading information…
Leukaemia
• Peak age 4y• Associated with Philadelphia chromosome in adults• FBC (↓RBC, ↓PLT)• Primitive white cells (lymphoblasts)• Bone marrow aspiration• Rx chemo, bone marrow transplant• Children have very good prognosis (>80% cure),
adults less good (30-40%)
The Peer Teaching Society is not liable for false or misleading information…
Acute lymphoblastic leukaemia
• Peak age 70• Associated with skin and gum infiltration• Investigations as for ALL (myeloblasts)• Auer rods in cytoplasm of blasts pathognomic
of AML• Rx with chemo, bone marrow transplant• Prognosis poor (12% 5y survival) - remember
this is disease of elderly
The Peer Teaching Society is not liable for false or misleading information…
Acute Myeloid Leukaemia
• Incidence increases with age, M>F 2:1• Family history of ALL, CLL• Malignant illness of B cells (lymphocytes)• 50% are diagnosed incidentally• Systemic symptoms, bone marrow failure,
lymphadenopathy and hepatosplenomegaly are late
The Peer Teaching Society is not liable for false or misleading information…
Chronic lymphocytic leukaemia
• What would you see on FBC?• Lots of lymphocytes• As these B cells will not mature in plasma cells, may
have hypogammaglobulinaemia• Usually chronic, incurable condition which patients
die with rather than from• Treat if symptomatic with chemo, bone marrow
transplant in the young• Prognosis: Stage A >10y, stage C<5y
The Peer Teaching Society is not liable for false or misleading information…
Chronic lymphocytic leukaemia
• Most common in middle age - ionising radiation• Associated with philadelphia chromosome• Clonal disorder arising from myeloid stem cell• Presents with systemic symptoms, abdominal
pain (splenomegaly), anaemia• Chronic phase → acute phase
The Peer Teaching Society is not liable for false or misleading information…
Chronic Myeloid Leukaemia
• FBC - leukocytosis, slightly low Hb, PLT normal or high
• Bone marrow aspirate hypercellular• Treatment with imatinib, bone marrow transplant in
young or resistant• Chronic phase responds to chemo, acute phase is
more resistant and usually rapidly fatal• Prognosis 60% 5y overall • 90% of those with chronic phase survive 5y
The Peer Teaching Society is not liable for false or misleading information…
CML
• Neoplasm of the "lymphoid system"• Symptoms: lymphadenopathy (neck), pain with
alcohol, general cancery things, can obstruct SVC• Peaks in young adults and elderly• Reed Sternberg cells• Associated with EBV• Staged by Ann Arbor system• Treat with chemo, PBSC transplant
The Peer Teaching Society is not liable for false or misleading information…
Lymphoma - Hodgkins
• 80% B-cell, 20% T-cell• Symptoms: similar to Hodgkin's but get bone
marrow infiltration earlier• Incidence increasing (AIDS)• Associated with RA, SLE, Sjogrens• Low grade: manage with chemo, unlikely to cure,
median survival 3-10 years• High grade: aggressive, e.g. Burkitt's lymphoma
(EBV), 30% curable
The Peer Teaching Society is not liable for false or misleading information…
Lymphoma - Non-Hodgkin's
The Peer Teaching Society is not liable for false or misleading information…
Myeloma
A malignancy of plasma cells in the bone marrow
The Peer Teaching Society is not liable for false or misleading information…
Myeloma - pathophysiology
• Clone of cancerous plasma cell forms filling the bone marrow producing a single (monoclonal) immunoglobulin (M band/paraprotein)• Normal immunoglobulin production suppressed - immunoparesis•Bence-Jones protein deposition in kidneys• Anaemia - due to tumour infiltration - anaemia of chronic disease
The Peer Teaching Society is not liable for false or misleading information…
Typically 60-70 years oldAfrican AmericansMen>Women (slightly!)
Myeloma - the patient
Confusion - due to hyperviscosity syndrome, hypercalcaemia or infection (e.g. UTI, septicaemia)
Renal Failure - producing malaise, anorexia, confusion. Due to hypercalcaemia, Ig deposition (Bence-Jones), septicaemia, drugs (e.g. NSAIDs)
Osteoporotic fractures and bone pain. Particularly to hips, ribs, skull and to back (height may decrease by 8-10cm).
Tired, breathless and fatigue due to anaemia and cachexia of chronic disease
The Peer Teaching Society is not liable for false or misleading information…
Myeloma - clinical presentation
• Bacterial infection - hypogammaglobulinaemia• Bone pain and fractures - resulting from lysis and resorption of cortical bone• Hypercalcaemia (nausea, thirst, polyuria, constipation, confusion)• Anaemia • Renal impairment - normal immunoglobulin = 1:1 ratio of heavy:light chains, in myeloma excess light chain (Bence-Jones protein) - deposition in kidney and impair function
The Peer Teaching Society is not liable for false or misleading information…
Myeloma - diagnosis• Monoclonal immunoglobulin band seen on protein electrophoresis• Bone marrow aspirate - excess plasma cells• Lytic lesions on X-rays of long bones, skull, spine
Also may have abnormal -• FBC - low Hb• Renal function - high creatinine• Albumin - low if chronically unwell• Cultures for bacteria - urine, blood
The Peer Teaching Society is not liable for false or misleading information…
The Peer Teaching Society is not liable for false or misleading information…
The Peer Teaching Society is not liable for false or misleading information…
Myeloma - management
• Depends on age of patient, co-morbidities etc.• Chemotherapy - common induction thalidomide-dexamethasone (initial disease control)• High dose treatment + autologous peripheral blood stem cell support increases response rate and remission in younger patients• Bone marrow transplant in some (young patients) - cure around 25%• Supportive care including bisphosphonates• Life expectancy 4-5 years (slowly increasing)
The Peer Teaching Society is not liable for false or misleading information…
Any Questions?