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Pathology BB quizzes > Haematology > Flashcards

Haematology Flashcards

1
Q

what is the management of haemophilia B?

A

factor IX replacement

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2
Q

what grade is follicular NHL?

(aggressive/variable/indolent)

A

indolent

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3
Q

treatment duration of 1st VTE with unknown cause?

A

3-6 months warfarin, possibly lifelong

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4
Q

how long to you give maintenance treatment for ALL in different patient groups?

A

girls and adults - 2 years

boys - 3 years

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5
Q

what is the poor outcome in CLL?

A

reichter transformation into DLBCL

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6
Q

managing elevated INR

>8, no bleed/minor bleed

A

Stop warfarin.

Vitamin K (oral/IV) no bleeding/if risk factors for bleeding or minor bleeding.

Check INR daily

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7
Q

what life-threatening complication is associated with APML

A

DIC

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8
Q

what cells stain +ve for CD15/30?

A

Hodgkin, Reed-Sterberg cells

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9
Q

what is the main difference between von willibrand disease and haemopihlia VIII on clotting studies?

A

both aPTT is normal

bleeding time is increased in VWD

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10
Q

what is the treatment for waldenstrom macroglobulinaemia?

A

plasmapheresis

chlorambucil, cyclophosphamide (& other chemo)

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11
Q

which inherited BM failure presents with renal malformations, microopthalmia and skeletal abnormalities?

A

fanconi anaemia

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12
Q

what is the diagnostic definition of accelerated phase CML?

A

>10% blasts in BM/blood

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13
Q

what is paraprotein?

A

A monoclonal immunoglobulin which appears as a dense narrow band (M band) on electrophoresis, seen in multiple myeloma

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14
Q

what is leukoerythroblastic anaemia?

5 features

A

immature cells from myeloid lineage (myelocytes/normoblasts) on blood film

features include:

  • poikilocytes
  • nucleated RBCs (normoblasts)
  • reticulocytosis
  • myelocytes/promyelocytes
  • thrombocytopenia
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15
Q

what is the lymphocyte count diagnostic of CLL?

A

>4.0 x10^6/mL

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16
Q

what infections give a monocytosis?

A

brucellosis, typhoid fever, tuberculosis, EBV/CMV, infective endocarditis, malaria, Chagas/ASS, visceral leishmaniasis, rickettsial infection

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17
Q

EBV-induced Burkitt lymphoma is responsive to what chemotherapy?

A

cyclophosphamide

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18
Q

The patients blood film shows classic Pelger-Huet neutrophils and the bone marrow biopsy reveals 15% blasts.

Diagnosis?

A

Refractory aneamia with an excess of blasts.

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19
Q

what is the treatment for chronic ITP?

A

IVIG, steroids, splenectomy

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20
Q

over-expression of cyclin D1

A

mantle cell lymphoma

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21
Q

Neutrophilia with visible toxic granulation and vacuoles on the blood film. suggests…

A

acute fungal infection

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22
Q

A 58 year old lady complains of lethargy and “easy bruising”. She presents with purpura. Her FBC reveals Hb 10.5g/dl; WBCs 2.3x109/l and platelets 8x109/l. Blood film reveals <1% Blasts, and marrow aspirate shows 20% dysplasia in erythroid lineage, 60% dysplasia in platelet lineage, 5% dysplasia in granulocyte lineage, and less than 5% blasts.

A

refractory cytopenia with multilineage dysplasia

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23
Q

which conditions give raised ESR and low CRP?

A

SLE, pregnancy, multiple myeloma, anaemia, lymphoma

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24
Q

which are the very aggressive NHLs?

A

Burkitt and ALL

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25
Q

what are the defective red cells seen in MDS?

A

ringed sideroblasts

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26
Q

what is the current treatment for hodgkin lymphoma?

A

ABVD

adriamycin/doxorubicin

bleomycin

vinblastine

dacarbazine/procarbazine

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27
Q

what is the diagnositic criteria for smouldering myeloma and what is the treatment?

A

EITHER serum monoclonal protein >30 g/L OR plasma blasts >10%

no CRAB symptoms or findings

Do not treat - no affect on mortality

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28
Q

what is the treatment for follicular lymphoma?

A

Rituximab CVP

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29
Q

a patient with long standing rheumatoid develops nephropathy and amyloid is found in the renal biopsy. what classification of amyloidosis is like likely to be?

A

secondary amyloidosis

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30
Q

which inherited BM failure has hepatic failure, short stature, endocrine abnormalities and pancreatic dysfunction?

A

Schwachmann-Diamond syndrome

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31
Q

what blood test reflects the acitvity of fibrinogen?

A

thrombin time

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32
Q

which immunoglobulin is commonly raised in MM?

A

IgG

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33
Q

what are the features that count as B symptoms in hodgkin lymphoma?

A

night sweats

fever

weight loss >10% in 6/12

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34
Q

which cytokine is important growth factor in the development of multiple myeloma?

A

IL-6

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35
Q

psot-transfusion: diarrhoea and maculopapular rash/skin necrosis…

A

GvHD

most commonly in immunosuppressed patients

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36
Q

t(11;14)

A

mantle cell lymphoma

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37
Q

what is the stardard prophylactic ajdunt to MTX therapy?

what is the adjunt for high-dose or intrathecal MTX?

A

folate

leucovorin

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38
Q

A 65 yr old man presents to his GP with general malaise, weight loss and visual disturbances that he likens to looking through a watery car windscreen. On examination he has peripheral lymph node enlargement. Protein electrophoresis shows an IgM paraprotein.

A

Waldenstrom macroglobulinaemia

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39
Q

benzene is a risk factor for what?

A

Acute leukaemia

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40
Q

between LMWH and UFH, which is more likely to induce HIT?

A

UFH

41
Q

what is the use of anti-lymphocyte/thymocyte globulin?

A

use in the setting of acute organ rejection

animal antibodies vs T cells, depletes the immune response

42
Q

Anti-proliferative used in polycythaemia rubra vera and essential thrombocythaemia

A

hydroxyurea

43
Q

what circulating serum proteins can be used to determine the severity of MM? (i..e NOT the monoclonal antibody, which is used to diagnose)

A

high beta-2 microglobulin & low albumin

part of the ISS staging system

44
Q

which inherited BM failure is a pure red cell aplasia?

A

Diamond-Blackfan syndrome

45
Q

what is Cooley’s anaemia?

A

beta-thalassaemia major

46
Q

hodgkin lymphoma accounts for what %age of all lymphoma?

A

20%

47
Q

what is the difference in specificity and sentitivity of pernicious anaemia antibodies?

A

sensitive - anti-parietal cell (>90%)

specificity - anti-intrinsic factor, as 10% of healthy people have anti-parietal cell

48
Q

which malignancy has the highest likelihood of promoting thombosis?

A

pancreatic CA - expresses high levels of tissue factor

49
Q

in patients with cancer and acute venous thromboembolism, which is the most effective drug at reducing the risk of recurrent VTE?

A

LMWH

50
Q

what is the management for haemophilia A?

A

desmopressin (increases vWF)

no IM injections or NSAIDs

factor VIII replacement

51
Q

what is the treatment for myelofibrosis?

A

hydroxycarbamide, thalidomide, steroids and SCT

52
Q

other than imatinib, what are the meds useful in CML?

A

hydroxyurea and IFN

53
Q

following massive transfusion, what are the considerations for platelets?

A

replacing with packed red cells and FFP will not replenish plts

expect plts = 50 x10^9/L after 20 U blood (= 2 blood volumes)

if <50-75 (depends on local guidelines) must transfuse plts as well

54
Q

Used in solid organ transplantation, multiple sclerosis, NHL. Depletes B lymphocytes more than T lymphocytes.

A

Rituximab (anti-CD20)

55
Q

a causative agent in hodgkin lymphoma?

A

EBV

56
Q

for which diseases may splenectomy be useful treatment?

A

The PIIES

thalassaemia, pyruvake kinase def, immune haemolytic anaemia, immune thrombocytopenic purpura, eliptocytosis, spherocytosis

57
Q

which are the indolent NHLs?

A

follicular and marginal

58
Q

treatment of recurrent VTE?

A

lifelong warfarin

59
Q

management of rised INR

5-8, no bleeding

A

withhold few doses.

reduce maintenance

restart when under 5

60
Q

what medication is given as thrombotic prophylaxis in DIC?

A

LMWH

61
Q

what is the clotting picture of vW disease?

A

elevated aPTT

elevated bleeding time

decreased VIII and vWF

normal PT, plts

62
Q

what is the treatment for mantle cell lymphoma?

A

rituximab CHOP

63
Q

what is anagrelide?

A

use in treatment of essential thrombocytosis when:

  • > 60 years
  • plts >1,000 x10^9/L
  • history of thrombosis

inhibits maturation of plts from megakaryocytes

64
Q

t(14;18)

A

follicular lymphoma

65
Q

chondrocalcinosis is commonly found in which diseases?

A

hypercalcaemia (hyperPTH), gout, arthritides, haemochromatosis, possibly post-trauma

66
Q

what is the diagnostic definition of CML?

A

<5% blasts in the bone marrow/blood

maybe slowing increasing over time

67
Q

A 10 year old boy presenting with tan-to-grey hyperpigmented patches on his upper trunk, neck and face, nail ridges and leukoplakia.

A

dyskeratosis congenita

68
Q

what are the abnormal plts seen in MDS?

A

micromegakaryocytes with hypolobated nuclei

69
Q

what biologic can be used in the treatment of all non-Hodgkin T cell lymphoma?

A

alemtuzumab (Campath, anti-CD52)

70
Q

what biologic agent used in NHL, solid tumour transplant and multiple sclerosis depletes lymphocytes and gives Graves/AITP as side effects?

A

Campath (alemtuzumab, anti-CD52)

71
Q

what is the diagnostic criteria for multiple myeloma?

A
  • M-protein in serum ≥30g/L and/or bone marrow clonal plasma cells ≥10%
  • Requires 1 or more of the following:
  1. Calcium elevation (>2.75 mmol/L)
  2. Renal insufficiency (creatinine >176 mmol/L)
  3. Anaemia (Hb <10 g/dL or 2 g/dL below the lower limit of normal)
  4. Bone disease (lytic or osteopenic)
72
Q

how quickly do you expect transient abnormal myelopoesis in a newborn to resolve?

A

a couple of weeks

73
Q

what are the blood findings in haemophilia A?

A

APTT elevated

PT normal

Factor VIII assay decreased

74
Q

what is the the first line mangement for MM?

how do you catagorise these patients?

A

suitable for auto-SCT: lenalidomide + dex

unsuitable for auto-SCT: melphalan + pred + thalidomide

75
Q

managing elevated INR

major bleeding

A

stop warfarin

give prothombin complex concentrate (if available), or FFP

IV Vitamin K

76
Q

what is the numerical definition of lineage displasia?

what is the definition of refractory cytopenia with multilineage dysplasia?

A

dysplasia in >10% of cell lineage

at least 2 myeloid lineages are dysplastic with bi-/pan-cytopenia in peripheral blood

77
Q

what is the treatment for CLL?

A

FCR

fludarabine - purine analog, inhibits DNA synthesis

cyclophosphamine - DNA crosslinks, leading to apoptosis

rituximab - anti-CD20, kills B cells

78
Q

which are the aggressive NHLs?

A

DLBCL and mantle cell

79
Q

what race most frequently carries the prothrombin G20210A mutation?

A

caucasians (5%)

80
Q

explain a normal G6PD assay in a patient with G6PD deficiency

A

if the assay is taken immediately during/after an acute challenge the bone marrow will have initiated erythropoesis to compensate therefore newly produced G6PD will give a false negative in the assay.

The assay should be done at a later date to confirm the diagnosis.

81
Q

TRAP +ve WBCs in serum suggest…

A

hairy cell leukaemia

82
Q

what is the treatment for DLBCL?

A

Rituximab-CHOP

83
Q

treatment duration of 1st VTE with known cause?

A

3 months warfarin

84
Q

which of the following is a immunohistochemical marker for multiple myeloma?

  • CD19
  • CD20
  • CD138
  • Surface Ig
A

CD 138

85
Q

what is Evan’s syndrome?

A

CLL associated with autoimmunity - AIHA and ITP

86
Q

CNS and testicular involvement are common in which disease?

A

ALL

87
Q

managing elevated INR

5-8, minor bleeding

A

Stop warfarin. Vit K slow IV. Restart when INR <5

88
Q

what is the staging system for CLL?

A

Binet staging

high WCC, <3 nodes - stage A

high WCC, >3 nodes - stage B

anaemia or thrombocytopenia - stage C

89
Q

which are the important CLL prognostic factors?

A

Zap 70

Immunoglobulin gene configuration (mutated vs. unmutated)

cytogenetics (trisomy 12, del 13q, del 17p)

90
Q

what is the dose of LMWH for treatment of DVT/PE

A

175 U/kg until INR in therapeutic range

91
Q

what T lymphocyte marker is abberently expressed on malignant B cells in chronic lymphocytic leukaemia?

A

CD5

92
Q

deletion of long arm chromosome 5

what is it and what is the treatment?

A

a MDS usually found in elderly women

lenolidomide - really good response

93
Q

which inherited BM failure presents with skin discolouration, oral leukoplakia and nail dystrophy?

A

dyskaratosis congenita

94
Q

what are the abnormal white cells seen on MDS?

A

hypogranulation/hyposegmented (pseudo-Pelger-Huet anomaly)

95
Q

which innate immune cell drives the response to salmonella typhi?

A

monocytes

96
Q

lymphadenopathy and HSM

elevated WBCs

hypercalaemia

predisposing viral infection

A

adult T-cell leukaemia

HLTV-1 from Japan or Carribean

97
Q

what age group get ALL and AML?

A

children get ALL

adults get AML (and under 2s)

98
Q

what is the second line management for MM in both groups of patients?

A

suitable for auto-SCT: bortezomib + dex

unsuitable for auto-SCT: melphalan + pred + bortezomib

Pathology BB quizzes (5 decks)

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