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Path 2022 > Haem > Flashcards

Haem Flashcards

1
Q

Signs/Symptoms of Bone Marrow Failure

A

o Anaemia – fatigue, pallor, SOB

o Neutropenia – infections
 Can be severe + life threatening – septic shock, renal failure, DIC
 Pneumonia, impetigo, necrotising fasciitis

o Thrombocytopenia – bleeding, bruising
+ DIC in acute promyelocytic leukaemia

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2
Q

Microscopic features of Acute Myeloid Leukaemia

A

o Circulating blasts (in peripheral blood film) - granules

o Auer rods!!

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3
Q

Supportive Tx in Acute Leukaemia

A
  • Red cells
  • Platelets
  • FFP / cryoprecipitate if DIC
  • Antibiotics if infections/sepsis
  • IV Long line
  • Allopurinol (uric acid is released from dying leukaemic cells when started on Tx)
  • Fluid + electrolyte balance
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4
Q

Local infiltration in AML vs ALL

A 
AML:
Splenomegaly
Hepatomegaly
Gum infiltration (monocytic disease)
Lymphadenopathy (occasional)
Skin
CNS (monocytic)
ALL:
Lymphadenopathy +/- thymic enlargement
splenomegaly
Hepatomegaly
Testes
CNS
Kidneys
Bone - causing pain in children (not adults)
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5
Q

Negative prognostic factors in AML

A

-5. Del 5
7q, 3q-. 11q23
Complex karyotype

+ Patient characteristics e.g. comorbidities, age
Response to Tx

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6
Q

Negative prognostic factors in ALL

A 
o	T(4;11)
o	Hypodiploidy
o	?t(9;22) - Philadelphia Chr - used to be bad prognostic indicator but now improved with tyrosine kinase inhibitors

+ Patient characteristics e.g. comorbidities, age
Response to Tx

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7
Q

Causes of thrombocytopaenia (+ most common)

A

Most common = immune mediated
o Idiopathic / Auto ITP – most common immune type!
o Drug-induced – Quinine, rifampicin, vancomycin
o Connective tissue disease – SLE, Rheumatoid
o Lymphoproliferative disease – CLL, lymphoma
o Sarcoidosis

Non immune mediated
o DIC
o Microangiopathic haemolytic anaemia (MAHA)

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8
Q

Haemophilia Pathophysiology

A

Congenital deficiency if Factor 8 (A) or 9 (B)

X linked recessive

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9
Q

Pathophysiology of bleeding in Vitamin K deficiency

A

Vit K acts as co-enzyme for gamma protein carboxylase

Required for synthesis of:

  • Factors 2, 7, 9, 10
  • Protein C, S, Z
Causes:
o	Most common cause = Warfarin
o	Malnutrition
o	Biliary obstruction
o	Malabsorption
o	(Broad spectrum) Abx - kills intestinal flora that generates Vit K
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10
Q

Ix results in DIC

A 
Prolonged aPTT
Prolonged PT
Prolonged TT
Decreased fibrinogen
High FDP
Low platelets
Schistocytes on blood film
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11
Q

Von Willebrand pathophysiology

A

Autosomal dominant

3 types:
o 1 = partial quantitative deficiency
o 2 = qualitative deficiency
o 3 = total quantitative deficiency

Outcome = platelets cannot adhere (via Glycoprotein Ib) to exposed subendothelial connective tissue to form primary platelet plug

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12
Q

Most thrombogenic (genetic) condition?

A

Anti-thrombin deficiency

Familial deficiency usually presents in 20s with thrombosis Sx

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13
Q 
MOA of anticoagulant drugs:
Heparin
Rivaroxaban/Apixaban/Edoxaban
Dabigatran
Warfarin
A

Potentiate antithrombin
Direct acting anti Xa
Direct acting anti IIa
Vitamin K antagonist - prevents recycling of vitamin K
* Procoagulant factors 2, 7, 9, 10 fall
* NOTE: anticoagulant protein C and S also fall but NET effect = anticoagulant

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14
Q

Thromboprophylaxis regimen

A

LMWH

o E.g., Tinzaparin 4500u / Enoxaparin 40mg OD

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15
Q

Treatment regiments after thrombosis due to:

  • Surgery
  • COCP, trauma, flight
  • No trigger
A

After surgical precipitant = VERY LOW risk of recurrence
-No need for long term Tx

After minor precipitants e.g., COCP, flight, trauma = moderate risk of recurrence

  • 3-month Tx usually adequate
  • Longer duration may be needed if other thrombotic/haemorrhagic RF

After idiopathic VTE (no circumstantial reason) = HIGH risk of recurrence

  • Long term anticoagulation needed
  • DOAC
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16
Q

Features of Leucoerythroblastic blood film

A

Teardrop RBCs - aniso, poikilocytosis
Nucleated RBCs
Immature myeloid cells

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17
Q

Causes of leucoerythroblastic anaemia

A

Malignancy

  • Haem: leukaemia, lymphoma, myeloma
  • Non haem: metastatic breast, bronchus, prostate

Myelofibrosis

Severe infection

  • Miliary TB
  • Severe fungal infection
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18
Q

Key features of Acquired Immune mediated Haemolytic anaemia

A

Spherocytes on blood film
DAT+
Agglutination

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19
Q

Key features of Acquired Non-immune mediated haemolytic anaemia

A

RBC fragments ‘schistocytes’
Thrombocytopaenia
DAT-

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20
Q

Reactive vs malignant neutrophilia

A

Reactive (infection): toxic granulation, no immature cells, vacuoles in neutrophils

Malignant:
Neutrophilia + basophilia + immature cells (myelocytes) + splenomegaly –> CML
Neutrophilia + even more immature cells (myeloblasts) –> AML

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21
Q

Causes of Secondary polycythaemia

A

Appropriately increased EPO:

  • High altitude
  • Hypoxic lung disease
  • Cyanotic heart disease
  • High affinity haemoglobin

Inappropriately increased EPO:

  • Renal disease – cysts, tumours, inflammation
  • Uterine myoma
  • Other tumours - liver, lung
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22
Q

Features of polycythaemia vera

A

Asymptomatic - raised Hb and Hct on routine FBC

Hyperviscosity Sx:

  • Headaches, light headedness, stroke
  • Visual disturbance
  • Fatigue
  • Dyspnoea

Increased histamine release

  • Aquagenic pruritis (after hot bath/shower)
  • Peptic ulceration

Variable splenomegaly?

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23
Q

Features of Essential thrombocythaemia

A

Asymptomatic - incidental thrombocytosis >600 on FBC in 50%

Thrombosis – arterial or venous
o CVA, gangrene, TIA
o DVT, PE

Bleeding – mucous membranes, cutaneous

Headaches, dizziness, visual disturbance

Modest splenomegaly

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24
Q

Clinical features of Primary Myelofibrosis

A

Asymptomatic - Incidental finding in 30%

Related to bone marrow failure / cytopenias
o Anaemia – pallor, SOB
o Thrombocytopenia – bruising, bleeding

Thrombocytosis

(Hepato)Splenomegaly – massive
o Budd-Chiari syndrome

Hypermetabolic state – weight loss, fatigue, dyspnoea, night sweats, hyperuricaemia (causing gout)

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25
Q

Clinical features of CML

A
  • Lethargy, hypermetabolism
  • Thrombotic event
  • Monocular blindness (CVA)
  • Bruising, bleeding
  • Massive splenomegaly +/- hepatomegaly
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26
Q

CML Treatment

A

1st line = Oral 1st gen TKI e.g. Imatinib

No complete response at 1 year OR acquire resitance –> 2nd line = 2nd gen TKI e.g. Dasatinib OR 3rd gen TKI e.g. Bosutinib

+/- Allogeneic stem cell transplant

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27
Q

Treatment of Ph Chr -ve Myeloproliferative disease

A

PCV = Venesection, aspirin, hydroxycarbamide

ET = Aspirin, Hydroxycarbamide - reduce platelet count +/- anagrelide (rare)

PM
Supportive:
- RBC & platelet transfusion

Symptomatic:
hydroxycarbamide (but may worsen anaemia), JAK 2 inhibitor e.g. Ruxolotinib

Curative: allogenic stem cell transplant

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28
Q

Primary genetic events in Multiple myeloma

A

Hyperdiploidy - 60% of cases
- E.g. trisomy 1, 9

IGH rearrangements involving Chr14q32
- E.g. t(4;14) IGH/FGFR3

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29
Q

Natural History of Multiple Myeloma

A 
MGUS
Smouldering myeloma
Symptomatic myeloma
Remitting-relapsing
Refractory
Plasma cell leukaemia
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30
Q

Diagnostic Features of Multiple Myeloma

A

10% or more plasma cells in bone marrow PLUS 1+ of the following:

CRAB
Hypercalcaemia - >2.75mmol/L
Renal disease – creatinine >177umol/L or eGFR <40ml/min
Anaemia – Hb <100g/L or drop by 20g/L
Bone disease – 1 or more lytic lesions on imaging

MDE
Bone marrow plasma cells 60+%
Involved: uninvolved FLC ratio >100
>1 focal lesion in MRI (>5mm)

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31
Q

Clinical Features of AL Amyloidosis

A 
Nephrotic syndrome - proteinuria, peripherla oedema
Unexplained heart failure
Sensory neuropathy
Abnormal LFTs
Macroglossia
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32
Q

Microscopic features of AL Amyloidosis

A

Congo Red stain
Apple green birefringence (under polarised light)
Fibrils are solid, non branching, randomly arranged and 7-12nm diameter.

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33
Q

Chromosome translocations in lymphoma

A

Follicular NHL - IgH;BCL2
Mantle Cell Lymphoma - IgH;cyclinD
Burkitt Lymphoma - IgH;c-MYC

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34
Q

Specific mechanisms of Lymphomagenesis

A
  1. (Constant antigenic stimulation) secondary to Chronic bacterial infection
    - B cell non-Hodgkin lymphoma marginal zone sub type (MZL)
    - H. Pylori –> Gastric MALToma of stomach
  2. (Constant antigenic stimulation) secondary to AI disorders
    - B cell non-Hodgkin lymphoma marginal zone sub types (MZL)
    • Hashimoto’s –> MZL of thyroid
    • Sjogren’s syndrome –> MZL of salivary glands
    - Coeliac disease –> small intestinal enteropathy-associated T cell non-Hodgkin lymphoma (EATL)
  3. Direct viral integration
    - HTLV1 retrovirus infects T cells via vertical transmission
    - Risk of adult T cell leukaemia lymphoma(ATLL) – a subtype of T cell non Hodgkin lymphoma = 2.5% at 70 years
  4. EBV driven proliferation (with immunosuppression)
    - NORMALLY EBV infects B lymphocytes –> healthy carrier state where proliferating B cells targeted and killed by EBV specific cytotoxic T cells
    - LOSS of T cell function can –> failure to eliminate EBV-driven proliferation of B cells:
    • HIV infection –> B NHL
    • Transplant immunosuppression –> Post-transplant lymphoproliferative disorder (PTLD)
    N
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35
Q

Key Histological features of B cell Non Hodgkin’s Lymphomas

A

B cell types

  • Burkitt’s = starry sky appearance
  • Small lymphocytic lymphoma = small lymphocytes, naive or post-germinal centre cells, CD5+CD23+
  • Follicular lymphoma = follicular pattern, CD10+bcl6+
  • Diffuse large B cell lymphoma = sheets of large lymphoid cells, germinal centre (good) or post-germinal centre cells
  • Mantle cell = pre-germinal centre cells, cyclin D1, aberrant CD5
  • Marginal zone = post-germinal centre memory cells
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36
Q

Key Histological features of T cell Non Hodgkin’s Lymphomas

A
  • Peripheral T cell lymphoma NOS = large T cells + EOSINOPHILS

Cutaneous T cell lymphomas = CD4+ T cells infiltrating epidermis

  • Anaplastic large cell lymphoma = large epithelioid lymphocytes, T cell or null phenotype
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37
Q

Key Histological Features of Hodgkin’s Lymphoma

A

Classical type

  • Reed Sternberg cells!
  • Hodgkin cells - large nuclei, prominent nucleoli -> ‘Owl’s eye’ appearance
  • Eosinophils

Non classic / Lymphocyte predominant

  • B cell rich nodules
  • Scattered L & H cells
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38
Q

Immunophenotype of B cells in CLL

A 
CLL = Aberrant expression of CD5
Will also have:
o	CD23+
o	FMC7-
o	CD79b +/-
o	SmIG +/-
o	CD19+

Normal peripheral B cell = CD3- CD5- CD19 +

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39
Q

Ann Arbor staging system for Lymphoma

A

FDG-PET-CT

  • I - one group of nodes
  • II - >1 group of nodes, same side of diaphragm
  • III – nodes above and below diaphragm
  • IV - extra-nodal spread

+ A - no constitutional Sx or B = any constitutional symptom

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40
Q

Poor prognostic factors in CLL

A

Tp53 mutation - del17p
IgH mutation status - unmuated = worse (8 year survival vs 25 years)

+ extent of disease (IPI score)

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41
Q

Diagnostic findings in CLL

A
  • Lymphocytosis 5-300 x109/L
  • Smear cells
  • Normocytic normochromic anaemia
  • Thrombocytopenia
  • Bone marrow lymphocytic replacement of normal elements

+ malignant cells on immunophenotyping e.g. aberrant expression of CD5 in peripheral B cells

42
Q

Acute Adverse Blood Transfusion Reactions

A

Acute haemolytic (ABO incompatible) - severe or fatal

Allergic - mild to moderate

Anaphylaxis - severe or fatal

Infection (bacterial) - severe or fatal

Febrile non-haemolytic - mild to moderate

Respiratory

  • Transfusion associated circulatory overload (TACO) - moderate, severe or fatal
  • Acute lung injury (TRALI) - severe or fatal
  • Transfusion associated dyspnoea (TAD) - diagnosis of exclusion
43
Q

Delayed Adverse Blood Transfusion Reactions

A

Delayed haemolytic transfusion reaction (Abs) - previous transfusion forms Ab to RBC antigen, next transfusion causes IgG mediated extravascular haemolysis, mild-moderate.

Infection - presents months-years later

TA-GvHD - rare, always fatal, patient tissue HLA attacked by donor lymphocytes (normally destroyed by patient immune system), immunosuppressed patients.

Post-transfusion purpura - settles in 1-4 weeks, give IV Ig, risk of life-threatening bleeding.

Iron overload - numerous transfusions (>50) accumulate iron, damage to organs e.g. liver, endocrine, heart,

44
Q

Sensitising events in Haemolytic disease of newborn

A

o Delivery – if baby RhD+
o Spontaneous miscarriage (if surgical evacuation needed)
o Termination of pregnancy
o Invasive procedures in pregnancy – amniocentesis, chorionic villus sampling
o Abdominal trauma
o External cephalic version
o Stillbirth or intra-uterine death

All require dose of anti-D

  • 250 iu if <20 weeks
  • 500 iu if >20 weeks
  • larger dose if large foetal bleed - use Kleihauer test!
45
Q

Components of cryoprecipitate

A 
o	Fibrinogen
o	FVIII and vWF
o	Fibronectin
o	fXIII
o	Platelet microparticles
o	IgA
o	Albumin
46
Q

Timeline of infections after BM transplant

A

Early = bacterial

Viruses

  • HSV re-activation early
  • BK = within first 30 days
  • CMV = after first month
  • EBV = late

Fungal = throughout?

  • Candida early
  • PCP later
  • Aspergillus has early + late peak
47
Q

HLA Types relevant to BM transplant

A 
o	HLA-A, -B, -C (class I) – present peptide to CD8+ cytotoxic T cells
o	HLA-DP, -DQ, -DR (class II) – present peptide to CD4+ T helper cells

HLA-A, -B, -DR most important!

48
Q

Possible indications for autologous + allogeneic BM transplant

A 
Autologous
o	Acute leukaemia
o	Myeloma
o	Lymphoma
o	Chronic lymphocytic leukaemia
o	Solid tumours
o	AI disease
o	Multiple sclerosis
Allogeneic
o	Acute leukaemia
o	Chronic leukaemia
o	Myeloma – most elderly patients cannot tolerate levels of myeloablation needed!
o	Lymphoma
o	BM failure
o	Congenital immune deficiencies
49
Q

Physiological changes in FBC during pregnancy

A

Mild anaemia
o Red cell mass rises – 120-130%
o Plasma volume rises 150%

Macrocytosis
o Normal physiology OR Folate or B12 deficiency

Neutrophilia

Thrombocytopenia
o with increased platelet size (more immature + more active platelets released due to rapid turnover)

50
Q

Cut offs for anaemia in pregnancy

A
  • <110 in 1st trimester
  • <105 in later trimesters
  • <100 Post-partum
51
Q

Causes of thrombocytopenia in pregnancy

A
  • Physiological / gestational / incidental - Mechanism poorly understood (partially dilution + increased consumption), >50 sufficient to deliver, >70 sufficient for epidural, foetal + baby UNAFFECTED
  • Pre-eclampsia (PET) - increased activation + consumption?, usually resolves post delivery
  • ITP - hardest to distinguish from physiological, baby can be affected - check cord blood!, may continue to fall up to 5 days post delivery, Tx = IVIg or steroids (if bleeding / needed for delivery)
  • Microangiopathic syndromes (MAHA) - shearing + fragmentation of RBC due to platelet deposition in vessels, includes HELLP, TTP, HUS (latter 2 not changed by delivery)
  • Other - Bone marrow failure, leukaemia, hypersplenism, DIC etc.
52
Q

Management principles of VTE in pregnancy

A

LMWH OD or BD (as for non pregnant)
Monitoring after 1st trimester - check anti Xa 4 hours post dose
STOP Tx for labour/planned delivery
- epidural can be given after 24 hours (12 if only prophylactic dose)

NO WARFARIN - causes chondrodysplasia punctata (but is safe to use when breastfeeding)

NO DOAC - during pregnancy or breastfeeding

53
Q

Stages of granulocyte maturation

A 
Myeloblast
Promyelocyte
Myelocyte
Metamyelocyte
Band cell
Immature --> mature neutrophil
54
Q

Features of a blast cell

A

Large
High nuclear: cytoplasmic ratio
Diffuse / primitive chromatin pattern
Visible nucleoli

55
Q

Microscopic/blood film features of acute promyelocytic leukaemia

A

classical subtype = hyper granular, multiple Auer rods

variant = bilobed nuclei

56
Q

Cytochemical staining in acute myeloid vs lymphocytic leukaemia

A

Myeloperoxidase: +AML, -ALL
Sudan black: +AML, -ALL
Non-specific esterase: +AML, -ALL

57
Q

Definitive Ix for diagnosis of ALL

A

Immunophenotyping

ALL:
Precursor B cell = CD19, CD20, TdTm CD10+/-
B cell: CD19, CD20, surface Ig
T cell: CD2, CD3, CD4 or 8, TdT

AML: MPO, CD13, CD33, CD14, CD15, glycophorin (E), platelet antigens

58
Q

Important platelet proteins/receptors

A

glycotprotien 1a - direct adhesion to exposed subendothelium

glycoprotein 1b - binds with assistance of vWF to exposed subendothelium (more important)

glyocpotein 2b 3a ‘Fibrinogen receptor’ - allows platelet aggregation

59
Q

Clotting pathway

A

TF binds F7 –> F7a
TF-Factor 7a complex activates F10 and 9 –> F10a and F9a
F10a activates F5 on cell surface

F10a and F5a convert prothrombin –> thrombin (small amount)
Thrombin activates F8, F5, F11, local platelets
Factor 11a activates Factor 9
Platelets bind Factor 5, 8 and 9

Factor 9a + Factor 8a together activate more Factor 10
Activated factor 10 and 5 convert more prothrombin –> thrombin ‘thrombin burst’ which forms stable fibrin clot

60
Q

Most thrombogenic quality/condition

A

Anti-thrombin deficiency (quantitative or qualitative)

61
Q

Treatment of Immune-mediated Idiopathic Auto ITP

A

Platelets >50,000 + No Sx = no Tx

Platelets 20-50,000 + No Sx = no Tx
Platelets 20-50,000 + bleeding = steroids + IV Ig

Platelets <20,000 + No Sx = steroids
Platelets <20,000 + bleeding = Steroids, IV Ig, hospital admission

62
Q

Factor deficiency coagulation disorders - name & defect/mechanism

A

Haemophilia

  • congenital deficiency of Factor 8 (A) or 9 (B)
  • X linked

Von Willebrand disease

  • autosomal domiannt
  • Type 1 = partial quantitative deficiency
  • Type 2 = qualitative deficiency
  • Type 3 = total quantitative deficiency
63
Q

Affected proteins in RBC Membrane disorders

A

Hereditary spherocytosis

  • Band 3
  • Protein 4.2
  • Ankyrin
  • beta spectrin

Hereditary elliptocytosis

  • alpa spectrin
  • beta spectrin
  • protein 4.1
64
Q

Indications for Splenectomy in Haemolytic anaemia

A 
Transfusion dependence
Growth delay
Physical limitation Hb <80
Hypersplenism
Age not <3 years but BEFORE 10 years - maximise prepubertal growth
65
Q

Blood film features during acute episode of RBC oxidative haemolysis (G6PD deficiency)

A 
Contracted cells
Nucleated RBCs
Bite cells - cytoplasm removed
Hemighosts?
Heinz bodies!!!! (using Methyl violet stain)
66
Q

Morphological feature of Pyrimidine 5-nucleotidase deficiency AND lead poisoning

A

Basophilic stippling - fine and course

67
Q

PROthrombotic effects in vessel wall after injury/inflammation

A

Anticoagulant molecules e.g. thrombomodulin DOWNregulated
tissue factor becomes expressed
Prostacyclin (anti platelet factor) production DECREASES
Adhesion molecules UPREGULATED
Von Willebrand factor release

68
Q

Key diagnostic feature of Paroxysmal cold haemoglobinuria (PCH)

A

Presence of Donath-Landsteiner Ab

Stick to RBC in cold –> complement mediated haemolysis when re-warmed

69
Q

TTP Pentad of Signs/Sx

A 
Microangiopathic haemolytic anaemia (MAHA)
Thrombocytopenia
AKI
Neurological impairment
Fever
70
Q

Aetiology of TTP

A

Mutation in ADAMTS13

Causes deficiency of vWF cleaving protease = fragmentation of RBCs in vessels

71
Q

Measuring response to Tx in CML

A

Haematological - WBC <10

Cytogenetic (on 20 metaphases)

  • Partial = 1-35% Philadelphia Chr +
  • Complete = 0% Philadelphia Chr + after 6 months Tx

Molecular - measure % reduction in BCR-ABL transcripts

72
Q

Diagnostic criteria for MGUS

A

Serum M protein <30g/L
BM clonal plasma cells <10%
No lytic bone lesions
No myeloma-related organ or tissue impairment
No evidence of other B cell proliferative disorder

73
Q

Risk factors for progression of MGUS to lymphoma/myeloma

A

Non IgG M spike
M spike >15g/L
Abnormal serum free light chain ratio

74
Q

Criteria for smouldering myeloma

A

Must meet both:

1) serum monoclonal protein (IgG or IgA) 30+ g/L OR urinary monoclonal protein 500mg + per 24 hours AND/OR clonal BM plasma cells 10-60%
2) Absence of myeloma defining events or amyloidosis

75
Q

Risk factors for progression of smouldering myeloma

A

BM myeloma cells >20%
M spike >20g/L
Serum free light chain ratio >20

High risk (2+ factors) may need Tx

76
Q

Mechanism of Myeloma kidney disease

A

MAIN = Cast nephropathy

  • normally taken up within proximal tubule –> return to circularion
  • BUT very high serum free light chains + Bence Jones proteinuria overwhelms cells
  • triggers inflammatory + fibrotic pathways
  • light chains able to travel further down tubule - interact with uromodulin (THP)
  • block tubules –> AKI

Other causes:

  • hypercalcaemia
  • loop diuretics
  • infection
  • dehydration
  • nephrotoxics
77
Q

Treatment of myeloma kidney disease

A

Hydration

Bortezomib-based therapy

78
Q

Diagnostic workup for multiple myeloma

A

Immunoglobulin studies

  • serum protein electrophoresis
  • serum free light chain
  • 24h Bence jones protein

BM aspirate + biopsy

  • immunohistochemistry for CD138 - myeloma specific!
  • kappa / lambda light chain staining

FISH analysis
Flow cytometry immunophenotyping

79
Q

Multiple myeloma treatment regimen if fit + <65 years old

A

Induction:

  • ‘VTD / VRD’ = proteasome inhibitor e.g. Bortezomib + immunomodulatory drug e.g. Lenalidomide + Dexamethasone
  • +/- Anti CD38 mAb e.g. Daratumumab

Autologous stem cell transplant
+/- consolidation: 2 x VTD/VRD regimen

Maintenance: low dose lenalidomide 2 years

80
Q

Multiple myeloma treatment regimen if frail and/or over 65 yrs

A

Lenalidomide + Dexamethasone

OR Bortezomib (proteasome inhibitor) + Cyclophosphamide (alkylator) + Dexamethasone

OR Daratumumab (anti CD38 mAb) + Bortezomib + Cyclophosphomide + Prednisolone

81
Q

Subtypes of Classical Hodgkin’s lymphoma

A

Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted

81
Q

Subtypes of Classical Hodgkin’s lymphoma

A

Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted

82
Q

ABVD Hodgkin’s Lymphoma Treatment Regimen

A

Adriamycin
Bleomycin
Vinblastine
DTIC (Dacarbazine)

83
Q

R-CHOP Non-Hodgkin’s Lymphoma Tx regimen

A

R = Rituximab (anti CD20)

CHOP:

  • Cyclophosphamide
  • Adriamycin
  • Vincristine
  • Prednisolone
84
Q

Indications to start Tx in (indolent) follicular Non-Hodgkin’s lymphoma

A

Nodal extrinsic compression e.g. bowel, bile duct, ureter, vena cava
Massive painful nodes
Recurrent infections

85
Q

Active Treatment of Chronic Lymphocytic Leukaemia

A

Ibrutinib = bruton tyrosine kinase inhibitor, suitable for refractory p53 mutation

Venetoclax = anti Bcl-2 oral agent, suitable for refractory p53 mutation

Allogenic stem cell transplant

CAR-T - complete B cell depletion so must give IV Ig!

86
Q

Indications for active Tx in CLL

A

Progressive lymphocytosis (count doubling in <6mths)

Progressive BM failure: Hb <100, Plts <100, Neutrophils <1

Massive or progressive lymphadenopathy / splenomegaly

Systemic/B symptoms

87
Q

Staging systems for CLL prognosis

A

Binet
A = <3 lymphoid areas
B = >3
C = Hb or Plts <100

Rai
0 = lymphocytosis only
1 = lymphadenopathy
2 = hepatosplenonegaly +/- splenomegaly
3 = Hb <110
4 = Plts <100
88
Q

Rank severity of Acute blood transfusion reactions

A

Severe/fatal

  • Acute haemolytic ABO incompatibility
  • Anaphylaxis
  • Infection (bacterial)
  • Acute lung injury (TRALI)

Moderate-severe-fatal
-Transfusion associated circulatory overload (TACO)

Mild/moderate

  • Allergic
  • Febrile non haemolytic
89
Q

Severity of delayed blood transfusion reactions

A

Always fatal
- Transfusion associated Graft versus Host disease

Mild-moderate

  • Delayed haemolytic transfusion reaction
  • Infection (from donor blood)
  • Post-transfusion purpura (BUT sometimes causes life-threatening bleeding)
90
Q

Criteria for neutropenic sepsis

A

Temp 38+ for 1 hour OR single fever >39

in patient with neutrophils <1.0 x 10^9/L

91
Q

EBMT risk score Post BM transplant

A

Age <20=0 20-40=1 >40=2
Disease phase Early=0 Intermediate=1 Late=2
Gender of rec/donor. Female to Male =1
Time to transplant <1year=0 >1 year =1
Donor Sibling=0 VUD=1

92
Q

Type of leukaemia seen in Down syndrome infants

A

Transient abnormal myelopoeisis

form of congenital leukaemia
Involves megakaryocytic lineage
Spontaneous remission common

93
Q

Typical hypersplenism bacteria

A

Pneumococcus
Meningococcus
H. influenzae

94
Q

Factors considered in WHO classification of Myelodysplastic syndrome (2016)

A 
Number of dysplastic cell lineages
% blasts in BM and peripheral blood
Cytogenetic
% ringed sideroblasts
Number of cytopenias
Hb <100
Plts <100
Neutrophils <1.8
Monocytes <1.0
95
Q

Outcomes of Myelodysplastic syndrome

A

1/3 die from infection
1/3 die from bleeding
1/3 die from transformation to acute leukaemia

96
Q

Drugs that can cause BM failure

A

Dose dependent - cytotoxics

Idiosyncratic - gold salts, phenylbutazone, NSAIDs

Other:
Abx - chloramphenicol, sulphonamide
Thiazide diuretics
Carbimazole (anti thyroid)

97
Q

Camilla Criteria for Idiopathic Aplastic Anaemia

A

2 out of 3:

  • Reticulocytes <1% (<20 x 10^9/L)
  • Neutrophils <0.5
  • Platelets <20

AND BM cellularity <25%

98
Q

Classical triad of Dyskeratosis Congenital

A

Skin pigmentation
Nail dystrophy
Leukoplakia

99
Q

Key features of Fanconi Anaemia

A

Congenital malformations - up to 70%

  • short stature
  • cafe au lait spots
  • thumb abnormalities
  • microcephaly/hydrocephaly
  • hyogonadism

+ Aplastic anaemia

Path 2022 (12 decks)

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