Flashcards in Haematology Deck (61):
Acanthocytes (Spur/spike cells)
RBCs have many spicules. Abetalipoproteinaemia, liver disease, hyposplenism.
Basophilic RBC stippling
Small dots of RNA. Accelerated erythropoiesis or defectve Hb synth. Lead poisoning,
What is anaemia?
S/S of anaemia
Fatigue, SOB, palpitations; Pallor, ?tachycardia, flow murmurs, CHF.
Fe definiciency, Anaemia of chronic disease, Sideroblastic, Thalassaemia.
Normocytic anaemias (7)
Blood loss, Haemolysis, Anaemia of chronic disease, Bone marrow failure, Renal failure, Hypothyroidism, Pregnancy.
B12 / folate def, Alcohol, Reticulocytosis (eg. in haemolysis), Hypothyroidism, Antifolate (eg. phenytoin), MDS.
MAHA, Fever, Renal impairment, Neuro signs, Low Plt.
Causes of Iron deficiency anaemia
- Blood loss: GI: - Meckel's diverticulum in older children, - Peptic ulcers (NSAIDs) - Polyps / colorectal Ca - Menorrhagia - Hookworm - Increased utilisation: pregnancy, growth. - Decreased intake: prematurity, poor diet. - Decreased absorption: Coeliac, post gastric surgery. - Intravascular hamolysis
Management of Iron deficiency anaemia
Thorough GI workup unless clear cause. Treat the cause. Oral iron - SFX: nausea, abdo discomfort, bowel change, black stools)
Mechanism and causes of Anaemia of chronic disease
Cytokines inhibit RBC production. Due to: - Chronic infection (TB, osteomyelitis) - Vasculitis - Rheumatoid arthritis - Malignancy - Renal failure: EPO not made (rather than suppressed).
Mechanism and causes of Sideroblastic anaemia
BM makes ringed sideroblasts rather than healthy RBCs, because it can't make haem. Causes: - Genetic - Myelodysplastic - Alcohol excess - Lead excess - Chemo - Radiation - TB drugs
Dx and Tx of sideroblastic anaemia
Ring sideroblasts seen in marrow. Tx: - Remove cause. - Pyridoxine (Vit B6 ^RBC production)
Vitamin B12: source and causes of deficiency
= cobalamin From meat and dairy. Deficiency: - Dietary (vegan) - Malabsorption due to stomach: lack of Intrinsic Factor - Malabsorption in terminal ileum: recsection, Crohn's, bacteria, tropical sprue, tapeworms.
Features of B12 deficiency
Mouth: glossitis, angular cheilosis; Psych: irritability, depression, dementia, psychosis; Neuro: paraesthesia, peripheral neuropathy.
Autoimm atrophic gastritis: lack of HCl and Intrinsic Factor. Commonest cause of macrocytic anaemia here. Parietal cell Abs, IF Abs (old: Schilling test). Replenish B12 (hydroxocobalamin IM).
Warm autoimmune haemolytic anaemia
IgG (warm hug) binds RBCs at 37deg (+ Coombs DAT). Spherocytes on blood film (IgG bitten off). Mainly idiopathic (can be lymphoma, CLL, lupus, methyldopa). Mx: steroids, splenectomy, immunosuppression.
Cold agglutinin disease
IgM binds RBCs in the cold (often with Reynauds). Mainly idiopathic (can be lyphoma or infections like EBV, mycoplasma). Mx: Treat underlying condition, avoid the cold, chlorambucil.
Paroxysmal cold haemaglobinuria
Viral infection (paroxysmal cold). Donath-Landsteiner Abs bind RBCs in cold; complement lyses on rewarming. Self-limiting.
Paroxysmal nocturnal haemoglobinuria
Non-immune: lose GPI from RBC surface, so complement lysis. Morning haemoglobinuria, thrombosis, Budd-Chiari. Dx: altered GPI on immunophenotype; Ham's test.
Tx for Haemophilia A
Avoid NSAIDs and IM injections. Desmopressin: more vWF, so less VIII degraded. Factor VIII concentrates.
Tx for Haemophilia B (Christmas disease)
Factor IX concentrates.
Tx for Von Willebrand's disease
Desmopressin, vWF concentrates, Factor VIII concentrates.
Tx for Vitamin K deficiency
IV Vitamin K, or FFP in acute haemorrage.
Tx for too much Heparin
Tx for too much Warfarin
IV Vitamin K or factor concentrates
Tx for Acute Lymphoblastic Leukaemia
Chemo: 1. Remission induction - chemo with steroids. 2. Consolidation - high dose multi drug chemo, including CNS (intrathecal). 3. Maintenance - 3 years in boys, otherwise 2 years. Consider allo-SCT. Supportive: blood products, Abx, allopurinol, fluid (prevent TLS).
Tx for Acute Myeloid Leukaemia
Chemo same as ALL, but no CNS Tx - remission, consolidation, maintenance. ATRA in M3 (APML). Supportive: blood products, Abx, allopurinol, fluid (prevent TLS).
Tx for Chronic Myeloid Leukaemia
Imatinib 5y surivival >95%.
Tx for Chronic Lymphocytic Leukaemia
When symptomatic. Chlorambucil (alkylating agent). ? SCT if young and fit. ? fludarabine, alemtuzumab, steroids.
Tx for Lymphomas generally
1. Combination chemo: ABVD - Adriamycin, Bleomycin, Vinblastine, Dacarbazine. 2. Radiotherapy for bulk. 3. SCT.
Tx for Burkitt's lymphoma (all three types)
What is CHOP?
Cyclophosphamide, Hydroxydaunorubicin, Oncovin = vincristine, Prednisolone.
Tx for Diffuse Large B Cell Lymphoma
R-CHOP, Auto-SCT for relapse.
Tx for Mantle Cell Lymphoma (B-cell)
R-CHOP, Auto-SCT for relapse.
Tx for Follicular Lymphoma (B-cell)
Watch and wait - indolent. When Sx, R-CVP.
Tx for MALT
Remove Ag! (H. pylori triple therapy); ?radiotherapy / ?rituximab.
Tx for Multiple Myeloma
1. CRAB: bisphosphonates, fluids. 2. Chemo: melphalan, bortezomib, lenalidomide, thalidomide, auto-SCT. 3. Steroids: dex or pred.
Tx for MGUS
None yet. Watch for transformation.
Tx for Smouldering Myeloma
None yet. Watch for transformation.
Tx for Waldenstrom's Macroglobulinaemia
Plasmapheresis for hyperviscosity; Chorambucil, cyclophosphamide, other chemo.
Tx for Amyloid light chain amyoidosis (Primary systemic amyloidosis)
Tx for Myelodysplastic syndromes
1. Supportive: blood, EPO, G-CSF, Abx; 2. Biological modifiers: immunosuppressants, lenalidomide, azacytidine; 3. Chemo: like AML; 4. Allo-SCT.
Tx for Aplastic anaemia
1. Supportive: blood, iron chelation, Abx; 2. Marrow recovery: GFs and oxymethalone (an androgen); 3. Immunosuppressants in idiopathic 4. SCT
Tx for Polycythaemia vera
Venesection; Hydroxycarbamide, asprin.
Tx for Myelofibrosis
Blood products; ?splenectomy; ?: hydroxycarbamide, thalidomide, steroids, SCT.
Tx for Essential thrombocytosis
Aspirin; Anegrelide (stop megakaryocyte breakdown); Hydroxycarbamide.
Tx for Warm autoimmune haemolytic anaemia
Steroids, splenectomy, immunosuppression.
Inherited Risk Factors for thrombosis
- Antithrombin deficiency- Protein C def - Protein S def - Factor V Leiden - Prothrombin G20210A - Lupus anticoagulant = Anti-phospholipid - Factor excess: VIII, II, Fibrinogen.
Acquired Risk Factors for thrombosis
- Age- Obesity - Previous clot - Immobilisation, surgery (esp ortho), long distance travel - Malignancy, esp pancreas - Antiphospholipid syndrome - Polycythaemia, thrombocythaemia
TED stockings, daily subcut LMWH.
Tx of DVT/PE
LMWH + Warfarin: Stop LMWH when INR >2.5; Continue Warfarin 3-6 months (except cancer Pts continue LMWH only)
Heparin: Action, route, antidote, SFX
Potentiates Antithrombin III (thus inactivates II, IX, X, XI). LMWH: subcut daily, doesn't need monitoring; Unfractionated: IV (loading then infusion), monitor APTT. Antidote: protamine sulphate. SFX: bleeding, heparin-induced thrombocytopaenia, osteoporosis.
Warfarin: Action, risk, anidote
Lowers active Vit K: less synthesis of II, VII, IX, X, and Proteins C, S and Z. Teratogenic. Reversal with IV Vit K / Factor concentrates.
Reasons for target INR of >2.5
- 1st DVT/PE - AF - cardiomyopathy - symptomatic inherited thrombophilia - mural thrombus - cardioversion
Reasons for target INR >3.5
- Recurrent DVT/PE - mechanical valve - antiphospholipid
INR 5-8, no bleeding - management
Withhold warfarin. Restart when INR
INR 5-8, minor bleeding - Management.
Stop warfarin. IV Vit K slowly. Restart when INR
INR >8 (without major bleeding) - Management
Stop warfarin. Oral Vit K if no bleeding; IV if bleeding or has RF. Check INR daily.
Raised INR with major bleeding - Management
Stop warfarin. Prothrombin complex concentrate (or FFP). Vit K IV.