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Third year > Week 4 - Haematology > Flashcards

Week 4 - Haematology Flashcards

1
Q

Where would you find sites of hemopoiesis in adults?

A

Bone marrow of: ribs, vertebrae, sternum, skull, pelvis

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2
Q

What is Leukemogenesis?

A

A multi step process. When the neoplastic cells is a heamopoietic cell in early myeloid or lymphoid. There is dysregulation of cell growth and differentiation. Proliferation of leukaemia clone with differentiation blocked at an early stage. So cells can’t mature- bone marrow failure.

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3
Q

What is Essential Thrombocytosis?

A

Sustained megakaryocytic proliferation, leading to an increase in the number of circulating platelets.

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4
Q

What are the complications of a major haemorrhage?

A

Acidosis, Hypothermia, Hypocalcaemia, coagulopathy

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5
Q

What testifies a myeloma?

A

Increased plasma cells in bone marrow.
Clonal Ig or paraprotein
Lytic bone lesions

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6
Q

What is the gold standard treatment for fanconi anaemia?

A

Allogenic SC transplant

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7
Q

What cells are characteristic in Hodgkins lymphoma?

A

Reed stern burg cells

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8
Q

What presentation is characteristic in meningitis?

A

Pyrexia and headache

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9
Q

What factors are in teh extrinsic pathway?

A

7

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10
Q

What factors are in the intrinsic pathway?

A

8, 9, 11, 12

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11
Q

What is inherited thrombophilia?

A

Deficiencies of natural anticoagulant (protein S, protein C, antithrombin)

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12
Q

Give an example of a JAK2 inhibitor

A

Ruxolitinib

For myeloproliferative disorders

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13
Q

What is anaemia of chronic disease

A

Failure of iron utilisation. iron trapped in RES

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14
Q

What are some reasons for Hypochromic microcytic RBCs?

A

IDA - not enough harm
Thalassaemia - Not enough global
ACD
Sideroblastic anaemia - iron doesn’t get incorporated into haem

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15
Q

Define major haemorrhage

A

Loss of more than 1 blood volume within 24 hours

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16
Q

What are some hereditary conditions impairing bone marrow function?

A

Sickle cell anaemia, thalassaemia, fanconi anaemia, dyskeratosis congentia

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17
Q

What are some acquired conditions impairing bone marrow function?

A

aplastic anaemia, leukaemia, metastatic malignancy, infections, drugs and toxins.
Chemo, haematinic diseases

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18
Q

What is a myeloproliferative disorder?

A

Clonal disorder of hemopoesis.
Increase in the number of one or more blood progeny
JAK2 or Calreticulin. Can transform into AML

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19
Q

What is fanconi anaemia?

A

Genetic disease due to an impaired response to DNA damage. FANCA-G. AR

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20
Q

What are the types of allogenic sc transplants?

A

Full intensity ‘myeloblastive’ - a severe form of myelosuppression. (conditioned with high dose chemo)
Reduced intensity ‘mini-transplant’ - in older pts (conditioned with low dose chemo)

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21
Q

How may GvHD present

A

Skin rash, jaundice or diarrhoea
Acute - within first 100 days
Chronic - after first 100 days

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22
Q

What are some problems with SC transplants?

A

GvHD, limited donor availability, age limit = 65, hypothyroidism, infertility, cataracts, osteoporosis, relapse.

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23
Q

What is sideroblastic anaemia?

A

Iron doesnt get incorporated into haem.

Hypochromic microcytic

24
Q

What are some effects of B12 deficiency?

A

Bilateral peripheral neuropathy
Demyelination of the posterior and pyramidal tracts of the spinal cord.
Beefy red Tongue, pallor

25
Q

Define Major haemorrhage.

A

Loss of more than one blood volume in 24 hours

26
Q

Explain how you would perform the PT test?

A

Add the patients plasma to thromboplastin (TF and phospholipids), warm to 37 and add Ca. Time for long it takes to clot - 10-13 seconds.
looks at extrinsic and common pathways
(in APTT add contact factor (silica/kaolin) to partial thromboplastin (phospholipids) - 26-38s)

27
Q

When does haem synthesis take place in a RBC

A

Between the pro-erythroblast to reticulocyte stage

Hb- 3 coding axons and 2 intervening introns

28
Q

What are some examples causing warm type (IgG) haemolytic anaemia (AI)?

A

Idiopathic, drug induced - Hapten, lymphoproliferative disorders (NHL/CLL) (spherocytic and polychromatic)

29
Q

Give some features and examples of severe inherited platelet disorders.

A

Mucosal type bleeding pattern, AR
Bernard soulier syndrome - absent/defective GP iB/ V/ IX, macrothrombocytopenia
Glansmann thrombasthenia - Absent/defective GP IIb/IIIa, normal platelet count

30
Q

What is characteristic of MDS?

A

Increased myeloblast cells

Progressive bone marrow failure

31
Q

What is the IPSS risk score in MDS based off?

A

% blast cells in BM
Karyotype
Cytopenia

32
Q

What is Thalassaemia?

A

Inadequate production/ decreased synthesis of normal globulin. Relative lack of globulin genes

33
Q

What cells may be seen in a peripheral smear in sickle cell disease?

A

Howell bodies

34
Q

What are some consequences of sickle cell disease in other organs?

A

Stroke, acute chest syndrome, pulmonary hypertension, vascular retinopathy, kidney infarction, osteonecrosis

35
Q

what antigen characterises a leukaemia cell?

A

CD34

36
Q

what is the main cell produced in acute leukaemia?

A

Blast cells

37
Q

What are some clinical signs in Acute leukaemia?

A

Lethargy, infection, bleeding and bruising, bone pain, gum swelling, lymphadenopathy. Rapid onset

38
Q

What chemo agents may be used in AML

A

Anthracycline and cytarabine based

39
Q

What are the translocation in M3 and M2 AML?

A

M3 - t(15, 17) - Acute Promyeloblastic leukaemia

M2 - t(8, 21)

40
Q

What antigens are expressed in CLL?

A

CD5, CD19, CD23

41
Q

What are some immune complications of CLL?

A

AI haemolytic anaemia

AI thrombocytopenia

42
Q

What is 17p deletion?

A

In CLL
Upper part of Ch17 is missing which allows the cell to recognise and eliminate cells that don’t make the quality grade. Loss of P53

43
Q

How might someone with plasma cell myeloma present?

A

Recurrent infection, back or rib pain, fatigue, hypercalcaemia, renal impairment

44
Q

What is the diagnosis criteria for plasma cell myeloma?

A

Neoplastic plasma cells in BM >/= 10% of total cells plus at least one of:

  1. Evidence of end-organ damage attributable to plasma cell proliferation - CRAB (Hypercalcaemia, renal insufficiency, anaemia, Lytic bone lesions)
  2. Biomarkers of malignancy - Clonal plasma cell % >/= 60%, serum free light chain ratio, focal lesion on MRI)
45
Q

What is follicular lymphoma at the molecular level?

A

t(14,18), over expression of BCL2, slow growth but reduced apoptosis

46
Q

What immunophenotype is associated with chronic Lymphocytic leukaemia?

A

CD 5, 19, 23

47
Q

What may cause purpuric rash with low platelets in the absence of any other cause?

A

Immune thrombocytopenia purpura

48
Q

Why shouldn’t you give amoxicillin in infectious mononucleosis?

A

Causes a rash

49
Q

What might you see in GvHD?

A

Skin rash, jaundice, diarrhoea

50
Q

What cells might you see a lot of in MDS?

A

Myeloblasts

51
Q

What does sideroblastic anaemia mean?

A

Failure of Iron to be incorporated into Haem

Hypochromic microcytic

52
Q

What causes B12 deficiency?

A

Pernicious anaemia
PPi, metformin, contraceptive pills
Terminal ileal problems - crohns

53
Q

Give examples of severe inherited platelet disorders and what it means?

A

AR. mucosal type bleeding pattern
Bernard soulier syndrome
Glansmann thrombasthenia

54
Q

What is the most common Leukaemia?

A

Chronic lymphocytic leukaemia

Its incidence rises with age

55
Q

What Tx would you give in essential thermbocytosis?

A

Hydroxycarbamide + aspirin
Anagredlide + aspirin
Ruxolitinib
INF- alpha

Third year (15 decks)

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