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PATH Heme-Onc II > Myeloid Neoplasms II > Flashcards

Myeloid Neoplasms II Flashcards

1
Q

review the general characteristics of myelodysplastic neoplasms (MDNs)

A
  • hematopoiesis: ineffective
  • dyspoesis: in one or more lineages
  • blood: peripheral blood cytopenia
  • bone marrow: hypercellularity
  • manifestations: organomegally uncommon
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2
Q

compare and contrast MPNs and MDNs in terms of

  • hematopoiesis
  • dyspoesis
  • blood cellularity
  • bone marrow cellularity
  • organ presentation
A
  • hematopoiesis
    • MPN: effective
    • MDN: ineffective
  • dyspoesis
    • MPN: in megakaryocytes only
    • MDN: in 1+ lineages
  • blood cellularity:
    • MPN: increased cell counts (mostly)
    • MDS: cytopenia
  • bone marrow: both hypercellular
  • organomegally:
    • MPN: common
    • MDN: uncommon
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3
Q

what are the general clinical components of MDNs?

A
  • common in older adults (median age of 70)
  • male predominance
  • sx are:
    • largely related to cytopenia
    • generally includes anemia - to a degree that most MDN pts require blood transfusions
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4
Q

what are Aeur rods? in what situations are they seen?

A
  • rod shaped structures that are = fused lysosomes / primary neutrophilic granules
  • see in:
    • RAEB-2 (MDN)
    • acute myeloid leukemias (MPN/MDN)
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5
Q

identify this picture, note important features

A
  • Aeur rods: fused lysosomes / primary neutrophilic granules
  • seen in
    • RAEB-2 (MDN)
    • acute myeloid leukemias (MPN/MDN)
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6
Q
A

dysgranulopoiesis

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7
Q
A

hypo-granular platelets

dysgranulopoiesis

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8
Q
A

dysmegakaryopoiesis

dysgranulopoiesis

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9
Q

list the all the MDN neoplasms

A
  • refractory cytopenia with unilineage dysplasia (RCUD) - anemia, neutropenia, thrombocytopenia
  • refractory cytopenia with multilineage dysplasia (RCMD)
  • refractory anemia with ring sideroblasts (RARS)
  • refractory anemia with excess blasts: RAEB-1, RAEB-2
  • myelodysplastic syndrome, unclassified (MDS-U)
  • MDS associated with isolated del(5q)
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10
Q

describe the bone / blood of RCUD.

note blast / sideroblasts status

A

refractory cytopenia with unilineage dysplasia

  • bone & blood: unilineage dysplasia
    • RA - anemia
    • RN - neutropenia
    • RT - thrombocytopenia
  • blood: cytopenia
  • blasts & ring sideroblasts - within normal range
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11
Q

describe the blood / bone of RMCD

note blast / sideroblasts status

A

= refractory cytopenia with multilineage dysplasia

  • blood & bone - multilineage dysplasia
  • blood
    • cytopenia
    • no blasts
  • bone
    • < 5% blasts
    • < 15% sideroblasts
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12
Q

describe the bone / blood of RARS.

note blast / sideroblasts status

A

refractory anemias with ringed sideroblasts

  • blood
    • anemia, often with dimorphic pattern - (two populations of red cells)
    • no blasts
  • bone marrow
    • < 5% blasts
    • erythroid dysplasia (unilinear)
    • > 15% of erythroid precursors are ringed sideroblasts
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13
Q

describe the bone / blood of MDS associated with RAEB-1

note blast / sideroblasts status

A

refractory anemia with excess blasts-1 (RAEB-1)

  • blood
    • cytopenia
    • < 5 blasts
  • bone
    • unilineage / multilineage dysplasia
    • 5-9% blasts
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14
Q

describe the bone / blood of MDS associated with RAEB-2

note blast / sideroblasts status

A

refractory anemia with excess blasts-1 (RAEB-2) = aka preleukemia

  • blood
    • cytopenia
    • 5-19% blasts
    • +/- Auer rods
  • bone
    • unilineage / multilineage dysplasia
    • 10-19% blasts
    • +/- Auer rods
    • myeloid maturation arrested
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15
Q

compare the blood & bone characteristics of RAEB 1 & RAEB 2 in terms of

  • blasts / sideroblasts
  • auer rods
  • dysplasia
  • cell levels
  • other features
A

both MDN

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16
Q

describe the bone / blood of MDS associated with isolated del(5q)

note blast / sideroblasts status

A
  • blood
    • anemia that is often severe & transfusion-dependent
    • normal or inc platelets
    • blasts < 1%
  • bone
    • dysplasia in 2+ lineages
    • blasts < 5%
    • ringed sideroblasts < 15%
17
Q

MDS associated del(5q)

  • pathogenesis
  • demographics
  • prognosis
A
  • pathogenesis: deletion of bands 31-33 of the long arm of chromosome 5
  • demographics: elderly women
  • prognosis: good
18
Q

identify picture, note important features

A

= RARS (MDN)

  • bone marrow (blue = inc iron):
    • > 15% erythroid precursors = ringed sideroblasts
    • erythroid dysplasia
19
Q

in which MDN is myeloid maturation arrested?

A

RAEB-2

20
Q

in which MPNs are ringed sideroblasts present?

A
  • RCUD: < 15%
  • RCMD: +/- 15%
  • RARS: .>/= 15%

(all in bone marrow)

21
Q

characterized MPN/MDN disorders based on

  • peripheral blood counts
  • bone marrow cellularity
  • hematopoiesis efficacy
  • dyspoiesis
  • blasts cell presence
  • presence of organomegally
A
  • peripheral blood counts - variable
  • bone marrow - hypercellular
  • hematopoiesis effectiveness - varies, usually less
  • dyspoesis - present
  • blasts cell - either normal or increased, but always < 20%
  • organomegally - common
22
Q

discuss the genetics of MDS/MPN combination disorders

A
  • no specific abnormalities
  • are all NEGATIVE for BCR-ABL1
23
Q

which neoplasms are MPN/MDN disorders?

A
  • chronic myelomonocyte leukemia (CMML)
24
Q

chronic myelomonocytic leukemia (CMML) - clinical: pathogenesis, demographics, risk factors

A
  • pathogenesis: no specific genetic linkage
    • no Ph chromosome or BCR-ABL2 fusion gene (like in CML)
    • no rearrangement of:
      • PDGFRA or PDGRFAB
      • FGFR1
      • PMC1-JAK2
  • demographics: older people
  • risk factors:
    • environmental toxins
    • radiation
    • previous chemo therapy
25
Q

chronic myelomonocytic leukemia (CMML) - morphology in

  • blood
  • bone marrow
A
  • blood
    • absolute monocytosis
    • +/- increase / decrease in
      • inc: granulocytosis (+/-)
      • dec: thrombocytopenia, anemia (+/)
    • dysplasia present - esp granulocytes
  • bone marrow - nothing specific
26
Q

chronic myelomonocytic leukemia (CMML) - presentation

A

just like CML (MPN) except:

like CML, except also includes presence of:

  • tissue based leukemic infiltrates
  • hepatomegaly common

CML presentation:

  • malaise / fatigue
  • pallor (anemia)
  • hyper-metabolic syndrome (cancer triad)
    • weight loss / anorexia
    • night sweats
    • fever
  • splenomegaly
  • gout
27
Q

compare & contrast the blood findings of CML vs CMML

A
  • CML
    • cellular #s:
      • increase
        • granulocytosis (invariably, basophils)
        • +/- thrombocytosis
      • decrease : erythrocytopenia (suppression)
      • NO MONOCYTE ABNORMALITIES
    • dyspoesis:
      • megakaryocytes- hypo lobulated “dwarf” megakaryocytes
  • CMML
    • cellular #s
      • increase:
        • MONOCYTOSIS
        • +/- granulocytes
      • decrease
        • +/- erythrocytopenia (suppression)
        • +/- thrombocytopenia
    • dyspoesis:
      • in granulocytes
28
Q

what is necessary to make a dx of CMML?

A
  • persistent blood monocytosis: > 1x 103/uL
  • < 20% blasts in blood or bone marrow
  • genetic requirements:
    • no Ph or BRC-AB1L fusion
    • no rearrangement of
      • PDGFRA or PDGFRA
      • FGFR1
      • PCM1-JAK2
  • leukocytosis common
29
Q

acute myeloid leukemia (AML) - clinical: pathogenesis, demographics, risk factors

A
  • pathogenesis
    • known risk factors:
      • radiation
      • conditions - downs, fanconi anemia
    • if no known conditions: related to mutations in chromosomes 5 & 7 & arise in the setting of:
      • myelodysplasia
      • DNA damaging drugs:
        • topoisomerase inhibitors
        • alkylating agents
  • demographics
    • in adults - 80% of leukemias
    • in children - 20% of leukemias
    • in neonates - most frequent leukemia
30
Q

discuss the prevalence of AML in

  • adults
  • children
  • neonates
A
  • adults - 80% of acute leukemias
  • children - 20% of acute leukemias
  • neonates - most common acute leukemia
31
Q

AML - overall presentation

A
  • a lot like CML
    • pallor
    • fatigue
    • fever
  • except
    • organomegally uncommon
    • and, lineage specific presentation:
      • DIC: in acute promyelocytic
      • gingival hyperplasia; in monocytic lineage
32
Q

discuss the bone / blood of AML

A
  • blood - anemia, thrombocytopenia like CMML
  • bone
    • hypercellular
    • blasts > 20% UNLESS associated with specific cytogenic abnormalities
      • myeloblast
      • monoblasts

PATH Heme-Onc II (6 decks)

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