Haematology

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mewinstanley@googlemail.com
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260344
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Haematology
Updated:
2014-02-05 08:39:49
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Haematology
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haemo
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  1. Look over this nice picture of blood cells
  2. Define anaemia + the different types
    Anaemia = Hb <125g/dL

    Microcytic -> low MCV -> defective haemoglobinisation

    Macrocytic -> MCV >100 -> Defective DNA synthesis
  3. What are the causes of Microcytic anaemia?
    TAILS

    • Thalassaemia
    • Anaemia of Chronic disease
    • IDA
    • Lead Poisoning
    • Sideroblastic anaemia
  4. For IDA give;
    -3 CF
    -2 causes
    -Ix
    -Mx
    CF

    Spooned nails, angular stomatitis, atrophic glossitis [pale & painless], Oesophageal web [Plummer-Vinson syndrome]

    Causes;

    • Blood loss -> GI until proven otherwise
    • Malabsorption -> Gastric acid req for release, abs in small intestine [Coeliac]
    • ^^Physiological demand -> pregnancy, infancy, puberty

    Ix -> low Serum ferritin, TF saturation <16%

    Mx -> Treat cause, Ferrous fumerate
  5. What are the investigation results of Anaemia of chronic disease?
    • Film -> normocytic, normochromic, RBC rouleuax
    • ^ESRN/^Serum ferritin
    • Total iron binding capacity = low
  6. Breifly outline B-Thalassaemia
    • AR inherited disorder of globin genes [missing one]
    • CF -> dyserythropoeisis, hair on end skull XR, anaemia + spleenomegaly
    • Mx -> Blood transfusion + chelation therapy
  7. Give 4 causes of Marocytic anaemia?
    Defective DNA synthesis;

    • B12 deficit
    • Folate deficit
    • Cytotoxic drugs
    • Myelodysplasia

    Other;

    • Marked reticulocytosis
    • Elevated plasma lipid -> alcohol, pregnancy, hypothyroidism
  8. Ix of Macrocytic anaemia?
    • Hb = low
    • MCV >120
    • Blood film
    • SF = elevated
    • Plasma LDH = elevated
  9. Outline B12 deficiency and give 3 causes?
    B12 [Cobalamin]

    bound to intrinsic factor -> abs in terminal ileum

    Causes:

    • Diet deficit -> strict vegans, rare
    • Gastric factors -> impaired acid secretion
    • Pernicious anaemia -> autoimmune destruction of parietal cells [no intrinsic factor]
    • small bowel -> ceoliac, IBD, tapeworms
  10. Outline folate deficiency and causes?
    Required for DNA synthesis

    Causes:

    • diet -> poor green leafy vegetables
    • Malabsorption -> Coeliac diasease
    • ^^demand state -> pregnancy, cell proliferation
    • Drugs -> phenytoin, OCP, MTX [only worsen existing deficit]
  11. Outline the congenital anc acquired causes of haemolytic anaemia. Give 2 for each.
  12. What are the FBC + U+E + urinary features of haemolysis?
    • Low Hb
    • ^bilirubin
    • ^LDH
    • ^reticulocytes
    • ^Urinary bilinogen
  13. Outline the causes Ix + Mx of warm type AHA?
    IgG, middle aged females

    Causes -> Idiopathic, NHL, CLL, Methyldopa, pen

    Ix -> features of haemolysis, Direct Coombs may NOT be +ve

    Mx -> treat underlying disease
  14. Outline Cold AHA
    IgM binds to RBC in cold condtitions

    Causes: Idiopathic, old people w B cell lymphoma

    Ix -> blood film, cold agglutinins

    Mx -> Keep warm
  15. Outline the Direct coombs test [Direct Antiglobulin test] and its uses
    DAT = Ab directly bound to RBC

    ELISA -> Monoclonal Ab for Fc-R -> agglutination = positive

    For;

    • AHA
    • HDN
    • Transfusion reaction
  16. Outline indirect coombs test?
    Ab in plasma -> crossmathing of blood

    For;

    • Pre-transfusion screening
    • Pregnancy screening for risk of HDN
  17. Draw the clotting cascade;
  18. What are;
    -APTT
    -PT
    -INR
    APTT = Activated partial thromboplastin time = intrinsic + common

    PT = extrinsic pathway = warfarin etc

    INR = PT but standardised
  19. Define Leukaemia
    Malignant disorder of haematopoetic stem cell compartment, clinical course varies on type

    • ALL Children get leukaemia
    • AML mor common in adults
    • CLL
    • CML = philidelphia [9:22]
  20. In general what are the risk factors for Leukaemia?
    • Ionising radiation -> radiotherapy, foetal XR
    • Cytotoxic drugs -> Alkylating agents, Benzene
    • Retrovirus -> rare T cell cause
    • Genetic -> Philidelphia [9:22] Trisomy 21
    • Immune -> hypogammaglobulinaemia
  21. Define acute leukaemia. Give 4 CF and 2 Ix
    Acute Leukaemia -> proliferation of primitive stem cells [blasts]. BM infiltration + leak into circulation

    CF

    • BM failure -> anaemia, bruising/bleeding, infection
    • Also;
    • rapid onset, lethargy, bone pain, gum hyperplasia

    Ix

    • Bloods -> normocytic anaemia, severe thrombocytopaenia, neutropenia
    • Film -> blasts -> ^^=worse prognosis
    • BM trephine -> Hypercellular, ^^blasts [>20%], AML -> Auer rods
  22. What is the Mx of acute leukaemia? Include supportive Mx
    Sepcific therapy -> chemo trial ASAP

    • Aim -> eradicate abn clone + induce remission
    • ^^morbidity -> bleeing, infections, hair loss, sterility
    • AML -> ?BM transplant
    • ALL -> beware CNS invasion -> vincristine [neuropathy]

    Suppportive

    • Anaemia -> BTx
    • Bleeding -> PLTs + FFP
    • Infection -> treat rapid, ?Granulocyte TFx
    • Metabolic Problems -> monitor fluids + electrolytes
    • Tumour lysis syndrome -> allopurinol stop hyperuricaemia
  23. Define CML, outline its natural history + give 3 CF
    CML = chronic myeloid leukamia = Philadelphia [9:22]

    Peak ~55yo

    NH

    • Chronic phase -> Tx responsive [imatinib] last ~5years
    • Accelerated phase -> Mx = more dificult
    • Blast crisis -> transforms into AML -> refractory

    CF

    • Symp -> vague, TATT, weight loss, SoB, abdo discomfort, lethargy, anorexia, sweating, bruising
    • Sign -> Spleenomegaly, hepatomegaly
  24. Outline the Mx of CML
    Chronic phase -> Imatinib

    MoA = inhibit Tyrosine Kinase BCR ABL - reduces uncontrolled proliferation

    Accelerated + blast crisis = dificult = ?hydroxycarbamide?
  25. Define CLL
    Most common Leukaemia, M:F 2:1

    • Incidentaloma
    • anaemia, infections, painless lymphaedonpathy, systemic symptoms

    Mx -> treat only if BM failure

    Caveat;

    • 11q22 deletion -> younger + more aggressive
    • Richter transformation -> high grade B cell lymphoma [loss of p53]
  26. Define Lymphoma
    Lymphoma = lymphoid neoplasm -> mainly of B-cells

    2 types;

    HL = Hodgkins Lymphoma -> reed-sternberg cell

    • NHL -> Non-hodgkins -> High v low grade
    • High = rapid division, over weeks, life threatening
    • Low = Slow, present for months, indolent
  27. Define HL + give 3 CF
    Hodgkins lymphoma

    Characteristic Reed-Sterberg cell -> large bi-nucleate B-cell, sorrounded by reactive cells

    CF

    • Painless tubbery lymphadenopathy, generalised itch, ?alcohol induced LN pain
    • B symptoms -> weight loss, drenching night sweats
  28. Outline the staging of HL
    Ann-Arbor staging

    • 1 - single LN site/ extranodal site
    • 2 - 2/>2 LN - same side of diaphragm
    • 3 - both sides of diaphragm +/- spleen +/- extralymphatic tissues
    • 4 - diffuse involvement of lymphatic tissues

    can be A or B [B if night sweats, weight loss]
  29. Outline the Ix and Tx of HL?
    Ix

    • Excision Biopsy = gold, but scarring + slow
    • Core biopsy = little scar, easier
    • FBC - ?anaemia -> BM invasion
    • U+E -> eGFR
    • LFT -> hepatic infiltration
    • CXR -> Mediastinal Mass
    • CT CAP -> TNM staging

    Mx

    • 1 or 2A = single node Rt
    • Chemo = good 90% remission
  30. for NHL give CF + Mx
    NHL = monoclonal expansion of B or T cells, Grading = most important

    CF

    • Widely disseminated at Pc
    • Lymphadenopathy
    • Weight loss, fever, night sweats, itching
    • BM failure in Low grade
    • Compression syndromes -> obstruction, Ascites, SVC obstruction MCSC

    Mx

    • Low grade -> symptomatic -> BM failure, compression syndromes
    • Rituximab [Anti CD20 may be useful]

    High grade -> Tx at Pc -> R-CHOP
  31. Give 3 causes of secondary BM failure [Aplastic anaemia]
    Primary = rare

    Secondary;

    • Drugs -> cytotoxics, azathioprine, ABx
    • Chemicals -> benzene, insecticides
    • Viral hepatitis
    • Pregnancy
  32. Define Myeloma and give 4CF
    • malignant proliferation of plasma cells in marrow
    • producing proteins -> Paraprotein [Ig of single heavy + light chains] Bence-Jones [Light chain only -> urine]

    CF

    • Lytic lesions of bone
    • Bone pain
    • Patho #
    • Hypercalcaemia -> confusion, constipation, abdopain [stones, groans + psychiatric overtones]
    • BM failure
    • renal failure
  33. Mx of Myeloma
    Immediate

    • ^^fluids -> renal impairment + ^^Ca
    • Analgesia
    • Bisphosphonates
    • Allopurinol -> urate neuropathy
    • Plasmaphoresis

    • Oncological Mx
    • Chemo -> Thalidomide
    • Rt -> bone pain
  34. Insert BTx stuff

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