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BPT > Haematology > Flashcards

Haematology Flashcards

1
Q

Pathophysiology of Anaemia of Chronic Disease

A

Reduced absorption of iron from GI Tract
Reduced EPO production
Sequestration of iron in macrophages
Increased Hepcidin

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2
Q

Causes of MAHA

A

DIC
HUS
TTP
Valve Related Haemolysis

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3
Q

What condition is the EMA Test for?

A

Hereditary Spherocytosis

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4
Q

What is the pathology in Hereditary Spherocytosis?

A

Mutations involving erythrocyte membrane proteins:
- Ankyrin
- Spectrin
- Band 3
Results in sequestration of spherocytes

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5
Q

What is the reason for/benefits of leukodepletion of donated blood?

A

Reduce dfebrile transfusion reactions
Reduced risk of CMV transmission
Reduced TRALI
Reduced Transfusion associated GVHD

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6
Q

What does Irradiation of donated red cells achieve?

A

Prevents proliferation of lymphocytes that haven’t been successfully filtered, which prevents transfusion associated GVHD

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7
Q

Which patients should receive irradiated RBCs?

A

Profoundly immunosuppressed patients
Stem cell transplants
Acute leukaemia

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8
Q

Which red cell surface antigen is most immunogenic?

A

ABO are the most immunogenic surface antigen group, followed by Rhesus antigens

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9
Q

Which blood group is the Universal Donor?

A

O

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10
Q

What is the cause of an acute haemolytic transfusion reaction?

A

ABO incompatibility.
Causes severe hypotension
Early suspension of transfusion → reduced mortality

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11
Q

What is the cause of delayed haemolytic transfusion reaction? What is the key risk factor?

A

Incompatibility of surface antigens other than ABO (i.e. less immunogenic antigens). Key risk factor is number of previous blood transfusions, as these sensitise immune system to these other antigens.

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12
Q

Transfusion Associated GFVH

A

Due to donor lymphocytes engrafting into recipient tissue. Extremely rare.
Causes fever, dermatitis and colitis, deranged LFTs and severe pancytopaenia. Management supportive

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13
Q

Febrile Non haemolytic reaction

A

Due to cytokines in transfusion product stimulating inflammatory response.
Starts either during or immediately after transfusion, presenting with fever, tachypnoea and tacycardia. Differentiated from acute haemolysis by appropriate Hb increment, lack of evidence of haemolysis

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14
Q

Transfusion Related Acute Lung Injury

A

Leading cause of transfusion related death. Mechanism not fully understood. Pregnancy is key risk factor (so plasma only comes from male donors). Donors who’ve caused it can’t donate again. Supportive management

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15
Q

What is the most common blood transfusion complication?

A

Transfusion related circulatory overload

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16
Q

What organism may be transmitted by blood products via its ability to survive refrigeration?

A

Yersinia

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17
Q

Indications for Platelet transfusion

A

Plt count <10
Plt count <50 and serious bleeding
Plt count <50 and needs surgery

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18
Q

Indications for Fresh Frozen Plasma

A

Severe bleeding
DIC
Hepatic Failure

Replenishes clotting factors

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19
Q

Anistocytes

A

Iron Deficiency

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20
Q

Target Cells

A

Haemoglobinopathy
Liver disease
Splenectomy

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21
Q

Schistocytes

A

Intravascular haemolysis

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22
Q

Spherocytes

A

Hereditary spherocytosis
Warm autoimmune haemolytic anaemia
Paroxysmal nocturnal haemolysis

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23
Q

Bite cells

A

G6pD

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24
Q

Tear Drop Cells

A

Myelofibrosis
Bone marrow infiltration

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25
Q

Spur Cells/Acanthocytes

A

Liver Disease

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26
Q

Burr Cells/Echinocytes

A

Renal Disease

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27
Q

Howell-Jolly Bodies

A

Hyposplenism

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28
Q

Iron Metabolism in acute inflammation

A

Hepcidin released as acute phase reactant → ferroportin breakdown → reduced iron absorption → contributes to anaemia of chronic disease

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29
Q

What conditions are type 1 cryoglobulinaemias associated with?

A

Waldenstrom’s macroglubulinaemia
Multiple Myeloma
Lymphoma

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30
Q

What are the Inherited Bone Marrow Failure Syndromes?

A

Fanconi Anaemia
Dyskeratosis Congenita
Shwachman-Diamond Syndrome
GATA2 Associated Syndrome

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31
Q

Myelodysplastic Syndrome

A

Clonal stem cell disorder leading to impaired production
One or more cytopaenias with morphological evidence of dysplasia in peripheral blood
Bone marrow blasts <20% for MDS
Genes: 5q minus syndrome, GATA2

32
Q

Paroxysmal Nocturnal Haemoglobinuria

A

Acquired stem cell disorder, PIGA gene mutation → lack of GPI anchor → Cd55 and CD59 can’t adhere to red cells (These agents block C3 convertase and C3b) → complement mediated intravascular haemolysis
Haemolysis, pancytopaenia, thrombosis
Confirm with flow cytometry
Treat with Eclulizumab which inhibits C5

33
Q

Diagnostic Criteria HLH

A

Either a molecular diagnosis of HLH, or 5 of the following:
- Fever
- Splenomegaly
- Cytopaenia affecting at least 2 lineages
- Hypertriglyceridaemia and/or hypofibrinogenaemia
- Fasting triglycerides ≥3, Fibrinogen ≤1.5
- Haemophagocytosis in bone marrow/spleen/nodes without evidence of malignancy
- Low or no NK cell activity
- Ferritin ≥500
- sCD25 ≥2400

34
Q

What are the key mutations in Essential Thrombocytosis?

A

JAK2 (50%)
CALR (40%) - mostly type 2 insertion mutation
Both increase megakaryocyte activity → increased platelet synthesis

35
Q

What is the management of Essential Thrombocytosis?

A

Monitor if Platelet count <600 and no additional thrombosis risks
Hydroxyurea first line to reduce thrombosis risk if required

36
Q

What is the key mutation in Polycythaemia Rubra Vera?

A

JAK2 V617F mutation → neoplastic haematopoietic stem cell dysfunction → increased erythrocyte production (EPO independent)

37
Q

Phases of Polycythaemia Rubra Vera

A

Latent: initial asymptomatic rise in RBC count
Proliferative: hyperviscosity, increased thrombosis risk
Spent: Fibrosis consumes marrow → pancytopaenia

38
Q

What haematological condition can cause transient hand/foot pain and erythema?

A

Erythromelalgia due to Polycythaemia Rubra Vera

39
Q

In what situation should correction of Iron Deficiency be avoided?

A

Polycythaemia Rubra Vera → worsens polycythaemia

40
Q

Medications that can cause thrombocytopaenia

A

Valproate
Platinum chemotherapy
Isoretinoin
Interferon

41
Q
A
42
Q

Key diagnostic criteria/clinical features of HIT

A

Fall in platelet count >50% and nadir ≥20
Onset 5-10 days after commencement (or ≤1 day if used previously)
Proven arterial or venous thrombosis

43
Q

Investigations for HIT

A

PF4 antibodies
Serotonin release assay → marker of platelet activation
Immunoassay + functional testing
Investigate if considering HIT as a possibility essentially

44
Q

What is the risk of using Warfarin in thrombocytopaenia?

A

Skin necrosis, particularly with counts <150

45
Q

Causes of ITP

A

Primary: Idiopathic (Most common)
Secondary: SLE< APLS, Lymphoma, Leukaemia (esp. CLL), HIV, HCV, Beta lactams, Heparin, Sulfonamides, Bactrim, quinine

46
Q

What antibodies develop in ITP?

A

IgG against GpIIb/IIIa → promotion of opsonisation → splenic sequestration

47
Q

Management of ITP

A

Stop medications that impair platelet function (NSAIDs, etc.)
Pulse MP
IVIg (induced rapid but transient recovery in counts to allow steroids to work)

48
Q

What is Romiplostim, and what is its indication?

A

Thrombopoietin receptor agonist → for treatment resistant ITP. Similar to Eltrombopag.

49
Q

A Plasmacytoma is a localised form of what?
And how is it differentiated?

A

Localised form of Myeloma

Differentiated by absence of:
- Anaemia
- Hypercalcaemia
- Other bony deposits
- Renal failure

Normal bone marrow biopsy, or <10% clonal plasma cells.

50
Q

Where do Plasmacytomas occur?

A

Marrow containing bones such as vertebrae, soft tissue including upper respiratory tract. Can present with bone pain or pathological fracture.

51
Q

Diagnostic Criteria for MGUS

A

M protein <30g/L
Clonal Plasma cells in Bone Marrow <10%
No myeloma defining events

52
Q

Diagnostic Criteria for Smouldering MGUS

A

M protein ≥30g/L in serum or ≥500mg/24hr in urine
Clonal plasma cells in Bone Marrow 10-60%
No myeloma defining events

53
Q

Diagnostic Criteria for Multiple Myeloma

A

Clonal bone marrow plasma cells ≥10 or ≥ 1 biopsy proven plasmacytoma
AND 1 or more myeloma defining events:

CRAB features:
- Hypercalcaemia >11mg/dL
- Renal impairment CrCl <40
- Anaemia Hb <100
- Bone disease ≥1 lytic lesion on imaging

Biomarkers of malignancy
- Clonal plasma cells in Bone marrow ≥60%
- Serum free light chain ratio ≥100
- >1 MRI focal lesion ≥5mm

54
Q

What cut off for Beta-2 Microglobulin indicates stage III disease for Multiple Myeloma?

A

> 5.5 mg/L

55
Q

Bortezomib

A

Proteasome inhibitor
Used for Multiple Myeloma treatment induction
Key side effects: peripheral neuropathy, thrombocytopaenia

56
Q

With which Immunoglobulin class is the risk of Hyperviscosity highest?

A

IgM

57
Q

What is the key cause of hyperviscosity?

A

Waldenstrom’s Macroglobulinaemia (because it leads to excessive IgM)

58
Q

What thresholds raise concern for hyperviscosity?

A

IgM >50g/L
IgA >70
IgG >100

59
Q

Waldenstrom Macroglobulinaemia

A

Low grade non hodgkin lymphoma involving B lymphocytes and plasma cells

Blood: IgM monoclonal gammopathy
Marrow: Lymphoplasmacytic lymphoma
MYD88 gene point mutations, CXCR4 has worse prognosis

60
Q

Causes of Macroglobulinaemia

A

IgM MGUS (>10% progress to Waldenstrom)
Smouldering Waldenstrom
Waldenstrom
CLL
Primary AL Amyloidosis

61
Q

What is the mechanism of neuropathy in Waldenstrom Macroglobulinaemia?

A

Paraprotein binding to myelin sheath → demyelination (IgM antiganglioside antibody). Sensorimotor, glove and stocking distribution, slowly progressive lower > upper limbs

62
Q

What antigen is a potential target for CAR-T Cell therapy in Multiple Myeloma?

A

BCMA

63
Q

What is the classic histology of AL Amyloidosis?

A

Positive Congo Red tissue staining

“apple green birefringence under polarised light”

64
Q

Pathology of TTR Amyloidosis

A

Abnormal tissue deposition of excess, misfolded TTR protein either due to TTR gene mutation or with age → heart failure.

Classic Echo finding: Reduced Global Longitudinal Strain (GLS)

65
Q

Treatment for TTR Amyloidosis

A

Tafamidis: prevents TTR synthesis → reduced tissue deposition. Improves overall survival

66
Q

Pathology/Pathophysiology of AL Amyloidosis

A

Most common form of amyloid. Related to plasma cell dycrasias (mostly multiple myeloma). Plasma cells form abnormal proteins, often in sheets, which don’t readily break down and are instead deposited in tissues including heart, nerves, kidney, thyroid.

Infiltrative, restrictive cardiomyopathy
Heart block due to conduction defects
Autonomic neuropathy
Carpal Tunnel syndrome
Nephrotic syndrome

67
Q

Management of AL Amyloidosis

A

Treat the underlying haematological malignancy

68
Q

MDS-5q Syndrome

A

Key subgroup of MDS/AML
Deletion on long arm of Chromosome 5
Responds very well to low dose Lenalidomide

69
Q

What are the favourable genetic mutations/markers in AML?

A
  • t(8;21)
  • inv16
  • Biallelic CEBPA mutation
  • NPM1 mutation without FLT3
70
Q

What mutation has a particularly poor prognosis in AML?

A

TP53

71
Q

AML Blood film

A

Auer rods within blasts

72
Q

Azacitidine

A

MoA: DNA hypomethylation → cytotoxic effect on cells
Indications: AML, high risk MDS
Can cause significant myelosuppression, and small risk of TLS particularly in first cycle, risk of toxicity higher in renal impairment

73
Q

What mutations in AML have targeted therapies available in Australia?

A

FLT3 mutations
Midostaurin and Gilteritinib are both FLT3 inhibitors, and improve overall survival added to chemotherapy

74
Q

What condition should be suspected in a patient initially thought to have AML but has marked coagulopathy?

A

APML

75
Q

Platelet count threshold for DAPT

A

> 50

76
Q

Platelet count threshold for single agent antiplatelet therapy

A

> 30

77
Q

Platelet count thresholds for anticoagulation

A

Therapeutic: >50
Prophylactic: >30

Can also give reduced dose DOAC at platelet counts 30-50

BPT (15 decks)

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