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Haem-onc Flashcards

1
Q

Myeloma: prognostic results:

A
  • age
  • PS
  • beta2 microglobulin
  • Albumin
  • creatinine
  • Ca
  • LDH
  • CRP
  • BMAT % plasma cells
  • cytogenetics/FISH
  • gene expression profile
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2
Q

Myeloma:adverse cytogenetic

A 
T(4;14)
T(14;16)
T(14;20)
17p-
1p-
1q+
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3
Q

Syndromes increased risk of acute leukaemia.

A 
Blooms
Fanconi anaemia
Ataxia telangiextasia
Neurofibromatosis
Klinefelters
Wiskott aldrich
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4
Q

What is MLL gene

A

11q23

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5
Q

Chemical exposure leading to AML or myelodysplasia

A

Benzene
Chlorambucil
Melphalan
(+RT)

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6
Q

Viruses associated with cancer

A

HTLV1 —> T cell Leukaemia/lymphoma
EBV - Burkitts and PTLD AND hodgkins
HHV8 Kaposi sarcoma and primary effusion lymphoma
HIV - lymphoma in unusual site eg CNS. Likely high grade

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7
Q

Myeloid cell markers

A

CD13
CD33
CD 117

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8
Q

Stem cell markers

A

CD34

TdT defines lymphoid lineage

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9
Q

T-ALL cell/immunological markers

A

CD7
cCD3
CD2

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10
Q

B All cell surface markers

A

CD10,
CD19
cCD22 (cytoplasmic)

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11
Q

AML favourable cytogenetic

A

t(15;17)
t(08;21)
Inv (16)

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12
Q

AML unfavourable cytogenetic

A

Del 5 or 7
Tp53 mutation
Complex rearrangements

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13
Q

AML molecular genetics favourable

A

NPM1
CEBPA (Biallelic)
T(8;21) RUNX1-RUNX1T1
Inv(16) core binding factor-myh11

Intermediate risk
NPM1 with FLT3 normal karyotype
WT NPM1 without FLT3
Other cytogenetic abnormality

High risk
Complex karyotype
Inv(3)
-5 (del 5q)
-7 (del7p)
WT NPM1 With FLT3
TP53
RUNX1
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14
Q

AML Unfavourable molecular genetics

A 
Complex karyotype (>4 abnormality)
Inv(3)
-5 (del 5q)
-7 (del7p)
WT NPM1 With FLT3
TP53
RUNX1
MLL
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15
Q

APML genetics

A

T(15;17) PML RAR alpha

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16
Q

AML subtypes 2016 WHO classification

A

1AML with recurrent genetic abN

  1. AML with myelodysplasia features
  2. Therapy related AML
  3. Myeloid sarcoma
  4. Myeloid associated with Downs
  5. AML NOS
17
Q

AML precursor cells

A 
CD34 
Cd38
Cd117
Cd133
HLA DR+
18
Q

AML granulocyte cell surface markers

A 
CD13 
Cd15
Cd16
CD33
Cd65
cMPO
19
Q

AML monocytes cell surface markers

A 
NSE
Cd11
Cd14
Cd64
Lysozyme
Cd4
Cd11b
Cd36
20
Q

AML megakaryocytes cell surface markers

A

Cd41
Cd61
Cd42

21
Q

AML erythroid cell surface markers

22
Q

Mechanism of imatinib, dasatinib, nilotinib and posatinib.

A

Liver - P450 cytochrome CYP3A4

Induced by ketoconazole, itraconazole,erythromycin,clarithromycin.

Inhibited by Dex, phenytoin,carbamazepine, rifampacin, St. John’s wort)

Cf warfarin CYP2C9

Closely monitor INR & thyroid

23
Q

Blood film features of dysplasia:

A

Red cell anisocytosis, poikilocytosis, basophilic stippling
Myeloid nuclear hypolobulation, pseudo Pelger Huet, hypogranulation
Myeloblasts
Platelet anisocytosis

24
Q

BMAT features of dysplasia

A

Erythroid binuclearity, internuclear bridging, irreg nuclear edge, megaloblastoid changes, ring sideroblasts, cytoplasmic inclusions, cytoplasmic bridging, incomplete haemoglobinisation, fringed cytoplasm or vacuolisation.
Myeloid bizarre nuclear shapes, pseudo pelger huet, nuclear hypersegmentation, Chédiak–Higashi granules, cytoplasmic hypogranulation, anisocytosis
Megakaryocytes - large monolobular forms, small binuclear forms, micromega, degranulation
Excess myeloblasts

Diagnostic = pseudo pelger huet and micro mega

Iron: >5 siderotic granules 1/3 nuclear circumference in >15% erythroid cells.

25
Recurrent chromosomal abnormalities presumptive diagnosis of MDS
-5 del5q -7 del7q i(17q) t(17p) Del of 11q 12p 13q
26
Commonly mutated genes in MDS
``` SF3B1 TET2 RUNX1 ASXL1 SRSF2 TP53 NRAS KRAS U2AF1 ```
27
Syndromes assoc with a plastic anaemia
Schwann diamond (pancreatic insuff) Fanconi anaemia (short, pigmentation, radial Ray) Dyskeratosis congenita (nails, leukoplakia, skin pigmentation) TAR DIamond Blackfan (RSP19 gene) Congenital amegakaryocytic thrombocytopenia (MPL gene mutation)
28
Aa cytogenetic abnormality
Del 13q Trisomy 8 Monosomy 7
29
Hairy cell leukaemia cell markers on flow
CD11c CD25 CD103 CD123 3/4 distinguishes from other B LPD
30
Systemic mastocytosis surface markers
CD2+ CD25 mast cells
31
Systemic mastocystosis
KIT 816V
32
Diagnostic criteria of systemic mastocytosis
Major: multi focal dense infiltrates of mast cells Minor: KIT816v point mutation >25% Bm mast cells with abnormal morphology - spindle/atypical CD2 or 25 on mast cells Serum rephrase more than 20
33
Favourable and unfavourable markers in mantle cell lymphoma
``` the following features are associated with a favourable prognosis in mantle cell lymphoma (MCL): Non-nodal presentation Hypermutated IGVH Lack of genomic complexity Negative SOX11 expression ``` A high Ki-67 proliferation index, or p53 mutations and p16 deletions, is closely related to the more aggressive MCL subtypes, such as the blastoid variants.
34
Blastocyst plasmacytoid surface markers
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) has a classical immunophenotype (CD4+/CD56+/CD123+) and dermatological manifestations. The flow cytometry results show a mixture of positive myeloid (CD33, HLADR) and T-cell (CD2, CD4) markers. CD123 is a specific marker for BPDCN in this context. There are negative B-cell (CD10) and monocytic (CD14 and CD64) markers. TdT, cCD3 and CD3 are all negative making acute lymphoblastic leukaemia less likely. Sezary syndrome usually shows positivity for both CD3 and CD4. Sezary cells also typically have a cerebriform nuclei morphology.
35
AL amyloidosis poor prognosis
``` Liver inv Cardiac inv MM FISH normal Autonomic neuropathy ```
36
Transient Leuk of Downs Syndrome
Immunophenotyping of the blast cells show variable expression of CD34, CD117, CD33/CD13, and platelet glycoproteins (i.e. CD36, CD42 and CD61), and aberrant expression of CD56 and CD7. GATA1

Haematology (2 decks)

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