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A3 ME3034 SPECIALTIES > W04 - HAEM: Haematological Malig.; Myeloid Malig.; Lymphoid Malig.; > Flashcards

W04 - HAEM: Haematological Malig.; Myeloid Malig.; Lymphoid Malig.; Flashcards

1
Q

Understand the principles of leukaemogenesis.

A

Proliferation of a mutant clone dt advantagenous acquired mut., mutation accum. over time, resulting in domination of tissue eg. BM or LN.
(HStemCell)
=> myeloid malignancies
= chronic phase => accelerated => blast crisis

*AMLeukemia = myeloid progenitor => downstream cells

=> lymphoid malignancies
= chronic phase => accelerated => leukemic transformation

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2
Q

Understand the presentations of leukaemias

A

M>F

  • AML = more common in adults

*ALLeukemia = more common in children
- contained in BM

CLL – chronic lymphocytic leukaemia – this is mainly a disease of the elderly, often incidentally
- often contained in nodes

CML – chronic myeloid leukaemia; often present in chronic phase

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3
Q

Discuss the principles of chemotherapy and bone marrow transplantation.

A

a

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4
Q

MPN

A

Myeloproliferative neoplasms (MPNs) are types of blood cancer that begin with an abnormal mutation (change) in a stem cell in the bone marrow. The change leads to an overproduction of any combination of white cells, red cells and platelets.

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5
Q

LN Structure and associated cell pops.

A
  • B cells in follicles
  • T cells in paracortex
  • Plasma cells in medulla
  • B cell differentiation and expnsion in germinal centres = become memory or plasma cells and exit GC
  • naive cells = mantle zone
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6
Q

Lymphoma presentation and dx modes

A

= nodal disease = lymphadenopathy

= extranodal disease (40% of NHLymph)

= systemic symptoms = fever, sweats, wt loss, pruritis, fatigue

*BIOPSY Dx
* Staging: imaging etc.

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7
Q

Significance of lymphadenopathy

A

1) LOCALISED
+pain
INFECTION

-pain
INFECTION, HARD METS., RUBBERY LYMPHOMA, REACTIVE

2) GENERALISED
+pain
VIRAL INFECTIONS

-pain
LYMPHOMA, LEUKEMIA, CONNECTIVE TISS. DISEASE, SARCOIDOSIS, REACTIVE, DRUGS

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8
Q

Outline the symptoms, diagnostic tests and treatment of:
Chronic myeloid leukaemia

A
  • cells differentiate and proliferate w/o BM failure thus survival for a few years
    anemia, splenomegaly, wt loss, gout, hyperleukostasis in fundus and venous congestion
    + ⇪⇪WCC, platelet
    + philadelhpia chromosome

(1)
> TKInhibitors: imatinib, daatibin (-ib endings)

(2)
> allogenic transplantation - when TKIs fail

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9
Q

Outline the symptoms, diagnostic tests and treatment of:
AML

A
  • nil differentiation thus BM failure, fatal
    anemia, thrombocytopenic bleeding (petechiae), infection (dt neutropenia)
  • blood count, blood film, BM aspirate = 20%+ blasts makeup
  • cytogenetics, immunophenotyping, CSF
  • gene panels

AUER RODS

> supportive
anti-leukemic ChTx = remission
- DAUNORUBICIN & CYTOSINE ARABINOSIDE
- GEMTUZUMAB OZOGAMICIN = targetted abs
- CPX-351

> allogenic SCell transplant

> Acute Promyelocytic Leukemia = ALL-TRANS RETINOIC ACID

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10
Q

Outline the symptoms, diagnostic tests and treatment of:
Myelodysplastic syndromes

A

MDS are generally considered precursors to leukaemia. 30% of all individuals suffering with MDS will go on to develop acute myoblastic leukaemia (AML).

acquired, and generally occur in the elderly.
pancytopaenia (nil rbc, wbc, plt.): anaemia, infection, and/or bleeding

*⇪blast cells in BM

> low dose ChemoT: azacytidine / High dose

> BM transplant

> conservative
Red cell infusions
Platelet infusions
Antibiotics for infection
Hemopoietic growth factors

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11
Q

Outline the symptoms, diagnostic tests and treatment of:
CHRONIC Myeloproliferative disorders/neoplasms

A

POLCYTHEMIA VERA: JAK2 V617F muts
* headaches, vac. occ, itch, TIA stroke, thrombosis, splenomegaly
+⇪Hb, haematocrit, ⇪WCC, platelet, ⇪ uric acid

> Venesection
Aspirin
Hydroxcarbamide / alpha interferon
JAK2 inhibitor

=> !thrombotic compl., BM failure, transformation to AML

ESSENTIAL THROMBOCYTHAEMIA: JAK2 V617F muts, CALR muts.
* significantly raised platelet count, thrombotic pres., gout, splenomeg.

> aspirin, hydroxycarb. or anagrelide
! progression to AML or myelofibrosis

IDIOPATHIC MYELOFIBROSIS
Marrow fibrosis and splenomegaly, de novo or following transformation of PV or ET
Usually >50 years old

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12
Q

Signs of BM Failure

A

anemia, thrombocytopenic bleeding (petechiae), infection: bacterial fungal (dt neutropenia)

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13
Q

Lymphoma Classification

A

HODGKIN LYMPHOMA

Vs

NH-LYMHPOMA: more common than H-Lymphoma
=> HIGH-GRADE
-diffuse large B cell lymphoma

=> LOW-GRADE
- follicular, marginal zone

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14
Q

Outline the symptoms, diagnostic tests and treatment of:
Acute lymphocytic leukaemia

A

nil differentiation, uncontrolled accum. often in BM
-children, B cell lineage
presents: BM failure (2/3w) or bone/joint pain + WCC increase
* 17yo male, 1mos impaired vision, 1/2stone wt loss, breathless on minimal exertion

  • large immature B cells with CD19

> ChemoT aim remission
Consolidation therapy
CNS tx
Maintenance tx for 18mos

> SC transplantation high risk

(newer)
> Blinatumumab = T cell engagers
> CAR-t Tx = Chimeric antigen receptor T cells
! cytokine release syndrome = fever, hypoT, dyspnoea
! neurotox.

*90% remission in adults and children 5y survival

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15
Q

Outline the symptoms, diagnostic tests and treatment of:
Chronic lymphocytic leukaemia

A

CLL commonest, M>F, familial
* matured cells, low grade condition, B cells with normal markers
* >5 lympjocyte count: doubling lymphocyte time = poor prog.
BM failure, lymphadenopathy, splenomeg., fever+sweats
+immune paresis
+ haemolytic anemia

> watch and wait
Cytotox ChemoT: fludarabine, bendamustine
monoAb: Rituximab, obinatuzumab
TK inhibitor

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16
Q

Outline the symptoms, diagnostic tests and treatment of:
Hodgkin lymphoma

A

30% of al lymphomas, bimodal age curve
1st pak: 15-35y
2nd peak: later in life
M>F, EBV, familial and geog clustering

*PET scanning for assessment to response to Tx

> Combination chemotherapy (ABVD)
+/- radiotherapy
Monoclonal antibodies (anti-CD30)
Immunotherapy (checkpoint inhibitors)

17
Q

Outline the symptoms, diagnostic tests and treatment of:
Non-Hodgkin lymphoma HIGH GRADE

A

Diffuse large B-cell lymphoma, commonest lymphoma

agressive, fast
* combo. chemoT
* curable

> anti-CD20 monoclonal antibody + chemo

18
Q

Outline the symptoms, diagnostic tests and treatment of:
Non-Hodgkin lymphoma LOW GRADE

A

Follicular lymphoma (watch and wait if nil symptoms), 2nd commonest lymphoma subtype

  • indolent, often asymptomatic, responsive to tx but incurable

> anti-CD20 monoclonal antibody + chemo

19
Q

Outline the symptoms, diagnostic tests and treatment of:
Myeloma

A

CRAB
Hypercalcaemia
Anaemia
Renal impairment
Bone pain

MONOCLONAL BANDS dt overprod of antibodies dt malignant proliferation of plasma cells in BM
* Bence-Jones proteins.
- elderly

Ab = hyperviscotic pic
hyperviscosity, amyloidosis and renal failure.

> autologous sc transplant
! neutropenic sepsis

> . induction rx: CHEMOT, STEROIDS, THALIDOMIDE

> supportive

20
Q

CLL Staging

A

BINET: based off of LN and clinical features
A - <3 LN
B - 3+ LN, ~8y survival
C - +Anemia or thrombocytopenia, ~6y survival

21
Q

Staging of Lymphoma

A

I to IV => I & II (contained in diaphgramic border) vs III vs IV (organ involvement)
A = absence of B symptoms
B = fever, night sweats, wt loss

*biopsy, CT scan, BM aspirate, trephine

22
Q

Understand what is meant by the term paraprotein

A

Monoclonal Ig present in blood or urine, indicating monoclonal proliferation of a B lymphocyte/plasma cell somewhere in the body.

  • useful for serum protein electropheresis
    = separating proteins based on size and chage = Ab diversity
23
Q

Know what diseases paraproteins are associated with

A

IgM paraproteins = lymphoma association
maturing b-lymph make IgM @ start of immune response

IgG, IgA = myeloma association
matured cells generate Ig after isotype switching

*amyloidosis
* renal failure
* hyperviscosity
* hypogammaglobulinemia

IgM myelomas dont exist but associated with low-grade lymphomas
- BM failure, lymphadenopathy, hepatosplenomeg., B symptoms

24
Q

Understand how myeloma commonly presents

A

Plasma cells: excessive prod of Ig. (BM)
*commoner in black population than white, 7th decade

  • bone disease: lytic lesions, fractures, cord compr., hypercalc.
  • BM failure
  • infections

CRAB
calcium (hyper.)
renal failure: dt cast nephropathyy
anemia
bone disease

+bleeding dt hyperviscocity, also causes cardiac failure, pulmonary congestion, confusion, renal failure
+ HYPOGAMMAGLOBULINEMIA

25
Q

Understand principles of diagnosis and treatment of myeloma

A

Paraproteins: monoclonal gammopathy pf uncertain icance (MGUS)

  • myeloma dx via excess plasma cells in BM
    = >10% of total BM cell pop.
  • IgG predominates, with IgA, light chain only, then other.
  • staging based on ALBUMIN & BETA-2 microglobulin

> chemoT:
carfilzomib, bortezomib (proteasome inhibitors)
lenalidomide, pomalidomide
monoclonal ab.

> BISPHOSPHONATE THERAPY: zoledronic acid

> RT

> Steroids

> Sx: long bone pinning, SC decompression

> autologous SC transplant

26
Q

Primary role of Ig

A

Recognise and bind pathogens, impeding processes or direct other components via tagging the antigen

*2 Heavy Chains
Fc = constant
- IgG, IgA, IgM, IgD, IgE
*2 Light Chains
kappa or lambda

=> variable domains on both chains

27
Q

Role of IgM

A

Initial phase of Ab prod.
Pentamer

0.5-2.0g/l

28
Q

Role of IgG

A

Most prevalent Ab subclass

6-15g/l

29
Q

IgA

A

Mucous membrane immunity

1-4.5g/l

30
Q

IgE

A

Parasite immune response, hypersens

31
Q

Other immunological tests

A

Total Ig levels = measure of Ig subclass by heavy chain

Immunofixation = identifies paraproteins present

Light chains = assess imbalance/excess ofd light chains in urine/serum

A3 ME3034 SPECIALTIES (27 decks)

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