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Flashcards in Haematology Deck (61):
1

Acanthocytes (Spur/spike cells)

RBCs have many spicules. Abetalipoproteinaemia, liver disease, hyposplenism.

2

Basophilic RBC stippling

Small dots of RNA. Accelerated erythropoiesis or defectve Hb synth. Lead poisoning,

3

What is anaemia?

Men: Hb

4

S/S of anaemia

Fatigue, SOB, palpitations; Pallor, ?tachycardia, flow murmurs, CHF.

5

Microcytic anaemias

Fe definiciency, Anaemia of chronic disease, Sideroblastic, Thalassaemia.

6

Normocytic anaemias (7)

Blood loss, Haemolysis, Anaemia of chronic disease, Bone marrow failure, Renal failure, Hypothyroidism, Pregnancy.

7

Macrocytic anaemias

B12 / folate def, Alcohol, Reticulocytosis (eg. in haemolysis), Hypothyroidism, Antifolate (eg. phenytoin), MDS.

8

TTP pentad

MAHA, Fever, Renal impairment, Neuro signs, Low Plt.

9

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

10

Management of Iron deficiency anaemia

Thorough GI workup unless clear cause. Treat the cause. Oral iron - SFX: nausea, abdo discomfort, bowel change, black stools)

11

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).

12

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

13

Dx and Tx of sideroblastic anaemia

Ring sideroblasts seen in marrow. Tx: - Remove cause. - Pyridoxine (Vit B6 ^RBC production)

14

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.

15

Features of B12 deficiency

Mouth: glossitis, angular cheilosis; Psych: irritability, depression, dementia, psychosis; Neuro: paraesthesia, peripheral neuropathy.

16

Pernicious anaemia

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).

17

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.

18

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.

19

Paroxysmal cold haemaglobinuria

Viral infection (paroxysmal cold). Donath-Landsteiner Abs bind RBCs in cold; complement lyses on rewarming. Self-limiting.

20

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.

21

Tx for Haemophilia A

Avoid NSAIDs and IM injections. Desmopressin: more vWF, so less VIII degraded. Factor VIII concentrates.

22

Tx for Haemophilia B (Christmas disease)

Factor IX concentrates.

23

Tx for Von Willebrand's disease

Desmopressin, vWF concentrates, Factor VIII concentrates.

24

Tx for Vitamin K deficiency

IV Vitamin K, or FFP in acute haemorrage.

25

Tx for too much Heparin

Protamine sulphate

26

Tx for too much Warfarin

IV Vitamin K or factor concentrates

27

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).

28

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).

29

Tx for Chronic Myeloid Leukaemia

Imatinib 5y surivival >95%.

30

Tx for Chronic Lymphocytic Leukaemia

When symptomatic. Chlorambucil (alkylating agent). ? SCT if young and fit. ? fludarabine, alemtuzumab, steroids.

31

Tx for Lymphomas generally

1. Combination chemo: ABVD - Adriamycin, Bleomycin, Vinblastine, Dacarbazine. 2. Radiotherapy for bulk. 3. SCT.

32

Tx for Burkitt's lymphoma (all three types)

R-CHOP

33

What is CHOP?

Cyclophosphamide, Hydroxydaunorubicin, Oncovin = vincristine, Prednisolone.

34

Tx for Diffuse Large B Cell Lymphoma

R-CHOP, Auto-SCT for relapse.

35

Tx for Mantle Cell Lymphoma (B-cell)

R-CHOP, Auto-SCT for relapse.

36

Tx for Follicular Lymphoma (B-cell)

Watch and wait - indolent. When Sx, R-CVP.

37

Tx for MALT

Remove Ag! (H. pylori triple therapy); ?radiotherapy / ?rituximab.

38

Tx for Multiple Myeloma

1. CRAB: bisphosphonates, fluids. 2. Chemo: melphalan, bortezomib, lenalidomide, thalidomide, auto-SCT. 3. Steroids: dex or pred.

39

Tx for MGUS

None yet. Watch for transformation.

40

Tx for Smouldering Myeloma

None yet. Watch for transformation.

41

Tx for Waldenstrom's Macroglobulinaemia

Plasmapheresis for hyperviscosity; Chorambucil, cyclophosphamide, other chemo.

42

Tx for Amyloid light chain amyoidosis (Primary systemic amyloidosis)

Chemo, auto-SCT.

43

Tx for Myelodysplastic syndromes

1. Supportive: blood, EPO, G-CSF, Abx; 2. Biological modifiers: immunosuppressants, lenalidomide, azacytidine; 3. Chemo: like AML; 4. Allo-SCT.

44

Tx for Aplastic anaemia

1. Supportive: blood, iron chelation, Abx; 2. Marrow recovery: GFs and oxymethalone (an androgen); 3. Immunosuppressants in idiopathic 4. SCT

45

Tx for Polycythaemia vera

Venesection; Hydroxycarbamide, asprin.

46

Tx for Myelofibrosis

Blood products; ?splenectomy; ?: hydroxycarbamide, thalidomide, steroids, SCT.

47

Tx for Essential thrombocytosis

Aspirin; Anegrelide (stop megakaryocyte breakdown); Hydroxycarbamide.

48

Tx for Warm autoimmune haemolytic anaemia

Steroids, splenectomy, immunosuppression.

49

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.

50

Acquired Risk Factors for thrombosis

- Age- Obesity - Previous clot - Immobilisation, surgery (esp ortho), long distance travel - Malignancy, esp pancreas - Antiphospholipid syndrome - Polycythaemia, thrombocythaemia

51

DVT prophylaxis

TED stockings, daily subcut LMWH.

52

Tx of DVT/PE

LMWH + Warfarin: Stop LMWH when INR >2.5; Continue Warfarin 3-6 months (except cancer Pts continue LMWH only)

53

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.

54

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.

55

Reasons for target INR of >2.5

- 1st DVT/PE - AF - cardiomyopathy - symptomatic inherited thrombophilia - mural thrombus - cardioversion

56

Reasons for target INR >3.5

- Recurrent DVT/PE - mechanical valve - antiphospholipid

57

INR 5-8, no bleeding - management

Withhold warfarin. Restart when INR

58

INR 5-8, minor bleeding - Management.

Stop warfarin. IV Vit K slowly. Restart when INR

59

INR >8 (without major bleeding) - Management

Stop warfarin. Oral Vit K if no bleeding; IV if bleeding or has RF. Check INR daily.

60

Raised INR with major bleeding - Management

Stop warfarin. Prothrombin complex concentrate (or FFP). Vit K IV.

61

Acute transfucion reactions

Febrile non-haemolytic transfusion reaction:
mild fever
Mild allergic reaction:
Urticaria
ABO incompatibility ("haemolytic transfusion reaction"):
Haemaglobinuria, SOB, nauseous, sweaty, dizzy.
Severe allergic reaction:
Swelling, wheeze.
Bacterial infection:
High fever.
TACO:
Severe SOB, raised venous pressure, pink froth.
TRALI:
Sever SOB, normal CVP.