Predominantly in the immunocompromised host - Burkitt - Hodgkins - DLBCL - PCNSL - Plasmablastic lymphoma

Haematology Flashcards by Laura Dryburgh

Therapy related AML/MDS

1) Alkylating agents
- Cyclophosphamide
- Melphalan
- Busulfan
2) Topoisomerase II inhibitors
- Etoposide (testicular cancer)
- Mitoxantrone

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EBV and lymphomas

Predominantly in the immunocompromised host

Burkitt
Hodgkins
DLBCL
PCNSL
Plasmablastic lymphoma

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BCR ABL - Philadelphia chromosome

translocation 9:22 –> deregulated tyrosine kinase activity

Note PCR of BCR-ABL can be used to monitor for molecular response.

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Polycythemia driver genes

1) JAK 2
- V617F (96%)
- exon 12 (4%)

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Lab findings in PCV

1) Elevated HB (16.5 male/16.0 female)
2) Suppressed endogenous EPO
3) Iron deficiency (functional iron deficiency)

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Low risk PCV

1) <65
2) No hx associated arterial or venous thrombosis

Aspirin only

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High risk

1) >65
2) Prior PV associated arterial or venous thrombosis

Aspirin 
Cytoreductive therapy 
- Venesection 
- Hydroxeurea 
- Interferon

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Essential thrombocytopenia

1) Jak 2 (60-65%)
2) CALR
3) MPL

Triple negative = low risk of vascular events

Mgmt Aspirin

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Primary myelofibrosis genetics chanegs

1) Jak 2 (60-65%)
2) CALR
3) MPL

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Clinical findings for Primary Myelofibrosis

1) Anaemia
2) Leukocytosis
3) raised LDH
4) Splenomegaly

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Management of PM

1) Supportive
2) JAK 2 inhibitors (ruxolitinib)
3) Allogenic stem cell transplant

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Treatment for CML

1) TKI: imatanib, dasatanib

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Main adverse effect of dasatanib

Pleural and pericardial effusions

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CMML Chronic myelomonocytic leukaemia

1) Leukocytosis + monocytosis

2) Absence of driver mutations (JAK2/BCR-ABL)

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Treatment for CMML

1) Supportive
2) Cytoreductive therapy i.e hydroxyurea
3) Hypomethylating agents i.e azacitadine

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What do you treat 5q deletion MDS with?

Lenolidamide

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MDS treatment (non-5q) for poor ECOG/older patients

1) Tranfusion support
- Blood transfusion
- Platelet tranfusion
2) Growth factors
- Erythropoiesis stimulating agents
- Granulocyte colony stimulating factor

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GATA 2 germline mutation (not complete)

Note all will develop MDS therefore early transplant

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Disease modifying agents for low risk MDS

1) Activin receptor type II ligand traps: Luspatercept/Sotatercept

MOA; fusion protein that blocks transforming growth factor - TGF B- that inhibits erythropoiesis

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Disease modifying agents in high risk MDS

1) Azacitadine: hypomethylating agent

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AML diagnosis

1) Pancytopenia
2) > 20% blasts in aspirate
3) Blasts with Bauer rods

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Flt 3 ITD mutation in AML good or poor prognosis

Confers poor prognosis

NPM1 + FLT 3 = better prognosis

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Treatment for AML (low ECOG)

Induction
3 days anthracycline
7 days cytarabine

Allogenic stem cell transplant

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Treatment for AML (high ECOG or age >70)

Study These Flashcards

Low dose cytarabine

Azactiadine - less than 30% blasts (due to slow cytoreduction)

Targeted therapies in AML

1) FLT 3 mutation: Midostaurin | 2) ABT-199: Venetoclax + Azacitadine

APML

PML RARA fusion --> blockage of pro-myelocytes non differentiation and no maturation 15:17

Blood film findings on APML

Bilobed nucleoli | Hypo/hyperganulated cytoplasma

Treatment for APML

1) Atra --> degrades fusion gene --> allows differentiation 2) Arsenic 3) Identify and monitor for DIC

ALL diagnostic criteria

20% blasts in bone marrow No auer rods in blasts Nb immunophenotyping via flow cytometry

Poor prognostic factors in ALL

``` B-ALL - BCR-ABL - Philadelphia like (no BCR-ABL) T-ALL - Early thymocyte precursor phenotype ``` In all lineages older age = poorer prognosis

Chronic lymphocytic leukaemia diagnosis

1) Lymphocytosis (B-cell lymphocytes) +/- cytopenias | 2) CD5 and CD19 clonal B cells

Good prognosis in CLL

13q deletion ("the lucky deletion)

Poor prognosis in CLL

11q/17p deletion Zap-70 CD38+ Unmutated heavy chain gene

When to treat CLL

1) Cytopenias 2) Lymphocyte doubled in <6 months or >50% increase in 2 months 3) Disease related symptoms 4) Autoimmune complications

Richters transformation in CLL

Tranformation to lymphoma ie DLBCL

Treatment CLL

1) Without deletion of 17p (TP53 mutation) - Chemotherapy 2) With deletion of 17p - Immunotherapy; Ibrutinib/Venetoclax

MOA of Venetoclax

Inhibits BCL-2 which is an anti-apoptotic protein that is over expressed in CLL

Diagnosis of Systemic AL Amyloidosis

Biopsy with Congo red stain

Criteria for Myeloma

1) Bone marrow >10% clonal plasma cells 2) End organ damage - CRAB - SLIM

SLIM criteria for MM

S: Sixty percent of=r greater clonal plasma cells on bone marrow LI: Free Light chain ratio >100 M: MRI demonstrate one focal lesion that is at least 5mm or greater in size

Prognostic markers for MM

``` B2 microglobulin < 3.5 good > 5 bad Albumin > 35 good < 35 bad ```

Main adverse effects of carlifilzomib

Heart failure

Pathogenesis of Burkitt Lymphoma

Translocation of the MYC oncogene from chromosome 8 - T(8:14) - T(2:8) - T(8:22)

Follicular Lymphoma translocation

T14:18 | Leads to BCL over expression

GELF criteria for commencement of chemo-immunotherapy

Symptomatic disease Threatened end organ damage Bulky disease

Which gene mutation has been identified in WM

MYD88

Complications associated with IGM MGUS

1) Perhiperal neuropathy 2) Cryoglobulinaemia 3) Hyperviscosity syndrome 4) Malabsorption - from depositing in GIT

Histological findings in Hodgkins Lymphoma

1) Reed Sternberg cells | 2) CD30 positive

Brentuximab MOA and use

Anti-CD30 | Used for relapsed refractory Hodgkins lymphoma

Emicizumab Mechanism of action

Monoclonal antibody that binds activated IX to X thus bypassing the need of activated factor VIII Used for haemophilia A

What does prothrombinex contain in Australia?

coagulation factors II, IX and X and low levels of factors V and VII

TTP

Headaches | Plasmapharesis

HLH

Elevated Ferritin | Haemophagocytosis on blood film

Felty's

RA/Splenomegaly/Neutropenia

Spur cell haemolytic anaemia

Cholesterol deposition in RBC cell membranes from Apo-lipoprotein A2 Spur cell --> Extravascular Haemolysis

B12 Deficiency

Megaloblastic Anaemia Hyper-segmented neutrophils Post gastrectomy common

Promthrombinex inclusion

2/7/9/10

Erlenmyer Flask Abnormality

Gaucher Disease (Inherited disease of storage)

CART cells

CD 19 | Cytokine release syndrome

Haematology Flashcards

(59 cards)