[LEC] Unit 4 Intro to Hema Neoplasm Flashcards by jade doe

Hematologic neoplasms are a group of disorders
which results from abnormal growth of

Hematologic stem cells

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T or F: Hematologic neoplasm is acquired (genetic diseases)

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HN is the first identified human _________ with consistent genetic defect

cancer

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Other name for Chronic Myelogenous Leukemia

Philadelphia Chromosome

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Philadelphia Chromosome translocation

t9:22

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Burkitt lymphoma translocation

t8:14

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published a study wherein they observed
the shortened chromosome in patients
suffering from CML

Nowell and Hungerford

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Initially referred to as preleukemias

Myelodysplastic Syndrome

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HN is the mutation in only 1 gene regulating cell
proliferation, survival, differentiation, or maturation

1 or more

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Leukemias originate from

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Solid tumors originating from ________ are called __________

lymphatic system; lymphoma

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T or F: Lymphoma can be found in the peripheral blood

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2 types of lineage of leukemia

Myeloid
Lymphoid

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Survival of acute

Fatal in weeks or
months

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The accumulation of younger forms is due to a
block in differentiation called as

Maturation arrest

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Bleeding due to thrombocytopenia (decrease in
platelet count

Acute leukemia

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Acute leukemia induces fever due to _______

neutropenia

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Acute leukemia induces fatigue due to

anemia

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Manifestations are non-specific and variable (some
patients are asymptomatic)

Chronic leukemia

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Diagnosis of chronic leukemia is often _______

incidental

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Highest incidence of HN in the US

Non-Hodgkin Lymphoma (4.3%)

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In the Philippines, the WHO 2018 data reported _____
leukemia deaths in our country

3678

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Acute Lymphoblastic Leukemia is more common in

children

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Chronic Lymphocytic Leukemia and Myelodysplastic
Syndrome are more common in

adults

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Etiology of leukemia

HUV Hereditary Unexpected Viruses

Most cases are developed to be

unkown in nature

Exception in unkown cases

Environmental toxins

Human T-cell Lymphotropic Virus-1/HTLV-1 is also known as

CD4 invaders

HTLV1 infects

Adult T cell leukemia/lymphoma

Epstein Barr virus is also known as

Burkitt lymphoma

Subser of Hodgkin lymphoma under EBV

Non-Hodgkin lymphoma

EBV affects

B cells

Increase risk of Non-Hodgkin Lymphoma

HIV-1

HIV-1 causes

Immunosuppression

4 diseases under hereditary condition of etiology

LF DAD Li-Fraumeni syndrome Fanconi's anemia Dyskeratosis congenita Ataxia telangiectasia Down Syndrome

Gene mutated in Fanconi's anemia

FA gene

mutation in the genes for telomerase maintenance

Dyskeratosis congenita

mutation in Li-Fraumeni syndrome

TP53

TP53 is also known as

Molecular policeman

Promotes cell cycle arrest and apoptosis when damaged DNA cannot be repaired

TP53

Gene mutated in Ataxia telangiectasia

ATM gene

a component of the DNA damage response signaling pathway

ATM kinase

Patients who are suffering has an increase risk of suffering of leukemia but the mechanism is not fully understood yet

Down syndrome (trisomy 21)

FAB classification basis

Morphological characteristics Cytochemical characteristics (enzymatic and non enzymatic)

Basis of WHO classification

Morphology, Immunophenotype Cytogenetics, Molecular Genetics

T or F: The WHO classification did not abandon the diagnostic criteria of fab, but there are additional bases that are used in the classification of different types of hematologic disorders.

initiation and maintenance of leukemia (“multihits”)

Leukemogenesis

These multihits can eventually form

malignant clones

Results of chromosomal aberration

uncontrolled proliferation loss of DNA repair capability and cell cycle control block in differentiation (Nuclear Transcription Factors) Continued cell survival and inhibition of apoptosis (pro-apoptotic proteins)

is responsible for the regulation of the cell cycle

cell cycle protein

responsible for the arrest of the cell cycle whenever there is damage to the DNA

checkpoint control protein

Control how genes are expressed or silenced

Epigenetic mechanisms

MI DNA is HIS DNA methylation Histone acetylation miRNAs

Excessive histone _______ closes the activity of DNA

deacetylases

Inhibits gene expression specifically binding to target mRNA that blocks translation of protein

miRNAs

Derived from protooncogenes

oncogenes

Responsible for normal cellular function

Protooncogenes

T or F: overexpression of protooncogene is a quantitative defect

Quali: Philadelphia chromosome t (9;22)

CML

Quali: JAK-2 point mutation

Polycythemia vera (PV)

Quali: FLT gene mutation

AML

t(18;14)

Follicular lymphoma

Follicular lymphoma involves the overexpression of what gene

BCL2 gene

BCL2 is normally found in chromosome #

t(11;14)

Mantle cell lymphoma

Mantle cell lymphoma involves the overexpression of what gene

CCND1

Increase copy of the gene occurs such as trisomy

Gene amplification

slows down cell division and promotes apoptosis

Tumor Suppressor Genes

Loss of function occurs when

gene deletion inactivating mutation epigenetic silencing

inactivation of TP53 gene

Li Fraumeni syndrome

inactivation of RB1 gene

Familial Retinoblastoma

Inactivation of W1 gene

Wilms tumor

Treatment

Chemotherapy Radiation Supportive therapy Targeted therapy HSC transplant

Done in combination with other types of treatment

chemotherapy

3 phases of chemotherapy

Induction Consolidation Maintenance

3 types of remission under induction phase

Hematologic remission Cytogenic remission Molecular remission

this will include a normal bone marrow picture, normalcy in the peripheral blood, and any evidence of the absence of leukemic cells.

hematologic remission

indicated by the absence of cytogenetic defects as seen when karyotyping is performed

cytologic remission

absence of leukemia cell nucleic acid sequences using very highly sensitive procedures capable of detecting a single leukemia cell among millions of normal cells

molecular remission

After the hematologic remission has been observed, a number of leukemia cells have still not been detected in the body in Consolidation phase. This condition is known as the

minimal residual disease.

Takes a long period because of less intensive agents that would destroy any remaining leukemia cells so relapse will be prevented

Maintenance phase

Unstable ion is used due to its toxic effects on cells, but there are complications.

Radiation

T or F: The toxic effect of radiation is irreversible

What is given to the patients are certain products that are naturally produced in the body

Supportive therapy

The first drug that is produced and used for the treatment of leukemia

Imatinib mesylate

Imatinib mesylate is specifically used for the treatment of

Chronic Myelogenoys Leukemia

all-trans retinoic acid is given to patients with

Acute Promyelocytic Leukemia

Aka Anti-CD 20 drug; targets lymophocytes

Rituximab

Monoclonal antibodies that are conjugated with toxins termed __________ will directly kill the target cell

immunoconjugates

anti CD-22 monoclonal antibody conjugated with an antibiotic with cytotoxic activity

Inotuzumab

The most effective treatment for malignant blood disorders

HSC transplant

from an identical twin

Syngeneic donor

usually from HLA-identical sibling or HLA-matched unrelated donor

Allogeneic donor

own patient’s bone marrow or peripheral blood stem cells are used

Autologous donor