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Bloc F - Defense > Mar23 A1-Hematopoiesis and Leukemias > Flashcards

Mar23 A1-Hematopoiesis and Leukemias Flashcards

1
Q

(imp) myeloproliferative diseases charact. + the prototype one

A
  • cancerous stem cell disorder of the BM
  • increased cell number but intact differentiation
  • may affect one or more cell lines
  • may affect WBC, RBC or platelets
  • may see prolif of other cells (fibroblasts for ex in myelofibrosis), in rarer entities
  • prototype = CML, driven by the bcr-abl kinase
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2
Q

(imp) treatment idea of CML and consequence

A
  • target bcr-abl

- this restores normal hematopoiesis

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3
Q

(imp) myelodysplastic diseases two types + risk with them (related to the two types)

A
  • low and high risk disease

- may transform to AML (M for myelogenous, myelocytic, myeloblastic), related to low vs high risk

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4
Q

(imp) myelodysplastic diseases charact

A
  • cancerous stem cell disorder of the BM
  • increased cell number but impaired differentiation
  • results in cytopenias (decrease in circulating cells)
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5
Q

(imp**) four major subtypes of MPNs (myeloproliferative neoplasms, disorders) AND main cell type affected in each

A
  • chronic myeloid leukemia (CML): WBCs
  • essential thrombocytosis: platelets
  • polycythemia rubra vera (PRV): RBCs
  • myelofibrosis: fibroblasts (accumulate in the BM)
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6
Q

(imp) CML: what happens and main thing

A
  • translocation event in the HSC, abl (9) and bcr (22) fusion on philadelphia chromosome
  • abl, kinase responsible for cell growth and division, is always active
  • accumulation of uncontrolled GRANULOCYTES (are WBCs)
  • differentiation is intact in early disease
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7
Q

(imp) causes or categories of MDS (myelodysplastic syndromes)

A
  • primary or de novo (no clear cause, may be certain toxins)
  • therapy-related MDS (secondary to chemo, radiation)
  • related and secondary to hematologic disorders (aplastic anemia, MPNs, paroxysmal hemoglobinuria)
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8
Q

4 broad categories of leukemias

A
  1. acute lymphoblastic leukemia (ALL)
  2. acute myeloid leukemia (AML)
  3. chronic lymphocytic leukemia (CLL)
  4. chronic myeloid leukemia (CML)
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9
Q

(imp?) 3 main acute leukemias (def of acute leukemia = blast count over 20% in BM or blood)

A
  • AML (acute myeloid leukemia)
  • ALL (acute lymphoblastic leukemia)
  • APL (acute promyelocytic leukemia (M3)) (a subtype of AML)
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10
Q

(imp?) most common acute leukemia in adults and its prognosis

A

AML. prognosis is variable and depends on cytogenetics

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11
Q

(imp?) how AML occurs + 2 most frequent initiating mutations

A
  • stepwise accumulation of mutations by a HSC leading. (early, late and relapse specific mutations)
  • clonal hematopoiesis leads to different clones with different mutations and deletions
  • DNMT3 and NPM1
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12
Q

(imp?) most common acute leukemia in children and its prognosis

A

acute lymphoblastic leukemia (ALL)

  • excellent prognosis in children, with multiagent chemo
  • less good prognosis in adults
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13
Q

(imp?) acute promyelocytic leukemia M3 (APL) how it occurs

A
  • often, translocation of chrom 15 and 17
  • gives fusion protein of PML gene (chrom 15) and retinoic acid receptor alpha (chrom 17) driving disease
  • RARa which normally binds RA in cytoplasm and then goes to nucleus as a TF doesn’t go in nucleus anymore = BLOCK IN DIFFERENTIATION
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14
Q

(imp?) APL treatment and prognosis

A
  • good prognosis with all-trans-retinoic acid (noticed you can overcome the block with more retinoic acid)
  • arsenic trioxide in relapsed disease
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15
Q

(imp?) 2 examples of leukemias where pathophysiology insights are useful for a targeted treatment decision

A
  1. bcr-abl fusion protein in CML. kinase inhibitors like imatinib
  2. PML-RARa fusion protein in APL. all-trans-retinoic acid
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16
Q

leukemia def and chronic vs acute

A
  • myeloid or lymphoid cancer starting in the BM
  • acute = aggressive
  • chronic = more indolent
17
Q

myeloid vs lymphoblastic or lymphocytic leukemia

A 
myeloid = arises in stem cells giving rise to RBCs, megakaryocytes (megakaryocyte erythrocyte progenitor lineage) and granulocytes (macrophages, mast cells, etc.) (granulocyte myeloid progenitor lineage)
lymphoid = in the lineage of the common lymphoid progenitor (gives B, T, NK)
18
Q

chronic leukemia how present most commonly

A

elevated white count, abnormal CBC

19
Q

2 chronic leukemias

A
  1. chronic myeloid leukemia (CML), is a MPN
  2. CLL (chronic lymphoid leukemia). subtype of B cell lymphoma, mature B lymphocytes expressing CD5 (atypical), a T cell marker. **remember CLL is SLL (small lymphocytic lymphoma: a low grade mature B cell NHL)’s leukemia counterpart
20
Q

exact definition of an acute leukemia (myeloid or lymphoid)

A

having a blast count (count of blast cells) of over 20% in the BM or in the blood)

21
Q

blast cell def

A
  • earliest (most immature) distinguishable hematopoietic cell
  • large size, large nucleus, ctly open DNA, prominent nucleolus
22
Q

acute leukemia how present commonly

A
  • pancytopenia
  • constitutional symptoms (fever, fatigue, bone pain, malaise)
  • tissue infiltration by blast cells
  • coagulation disturbances
  • leukostasis syndromes (accum of blasts in microcirculation with impaired perfusion. lungs and CNS affected)
  • tumour lysis syndromes
23
Q

how acute leukemia is diagnosed

A

BM study

24
Q

good, intermediate and poor risk categories in AML (means if can control disease and prevent death with chemo)

A

good risk = CML, APL and chrom 16 transloc
intermediate risk = normal chromosomes or trisomy
poor risk = abnormal chrom 5 or 7, 11q23 (chrom 11) abnormality in MLL (mixed lineage leukemia), 3 or more chrom abnormalities

25
Q

frequent mutatations in AML (also targets for therapy)

A
  • FLT3 (a TK)
  • IDH (of Kreb’s cycle)
  • DNMT3A (a DNA methyl transferase)
26
Q

APL main characteristics on presentation

A
  • pancytopenia

- coagulation disturbances

27
Q

most common malignancy in children

A

ALL

28
Q

ALL cells affected (malignant cells)

A

B cell disease (lymphoid cells)

29
Q

ALL symptoms and presentation

A
  • related to the cytopenias (anemia, neutropenia, thrombocytopenia)
  • lymphadenopathy, bone pain, fever
30
Q

goals of acute leukemia treatment

A
  • remission induction
  • blast count less 5%
  • restore normal hematopoiesis
  • WBCs above 1-1.5
31
Q

treatment that heals all acute leukemias and

A

immunotherapy. take cells out of patient, transfect with retrovirus, grow in large number, reinfuse back in pt

32
Q

one immunotherapy example for acute leukemias

A
  • blinatumumab (BITE Ab = Bi-specific, T cell engager Ab)

- bi-Ab binds CD19 and CD3 to force tumor cell and tumor cell interaction

33
Q

treatment options in MDS (myelodysplastic syndromes)

A
  • RBC and platelet transfusions
  • GF support (ESA, G-CSF)
  • demethylating agents (aza-C, decitabine)
  • immunosuppression
  • high dose chemo
  • allogeneic stem cell transplant (donor = someone else, not self)

Bloc F - Defense (57 decks)

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