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PATH Heme-Onc II > Myeloid Neoplasms I > Flashcards

Myeloid Neoplasms I Flashcards

1
Q

what are the four main categories of myeloid neoplasms?

what are the defining characteristics of category?

A
  • myeloproliferative neoplasms (MPN)
    • effective hematopoiesis with increased production → cells (both mature & immature) released into blood, thus
      • increased peripheral blood counts
  • myelodysplastic syndromes (MDS)
    • ineffective hematopoiesis: abnormal cell maturation → cells die before release into peripheral blood, thus:
      • cytopenia
  • MPN / MDS syndromes:
    • overlapping MPN & MDS characteristics
  • acute myeloid leukemias (AML)
    • accumulation of immature myeloid forms in marrow → normal hematopoiesis suppression, thus
      • cytopenia
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2
Q

neoplasms within which four myeloid neoplasm categories result in cytopenia?

A
  • MDS (MPN / MDS if MDS predominant)
  • AML
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3
Q

contrast myeloid neoplasms within the MPN and MDS categories based on

  • efficacy of hematopoiesis
  • bone marrow status
  • peripheral blood cell status
  • presence of dyspoiesis
  • presence of organomegally
A

both: bone marrow hypercellularity

MPN

  • hematopoiesis effective
  • increases in peripheral blood counts (both mature + immature)
  • no dyspoiesis except for megakaryocytes
  • organomegally common

MDS

  • hematopoiesis ineffective
  • decreases in peripheral blood counts (cytopenia)
  • dyspoiesis in 1+ blood lines
  • organomegally uncommon
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4
Q

neoplasms in each in each myeloid neoplasm category have what in common?

A

= MPN, MDS, MPN/MDS, AML

  • involve some loss of regulation
  • involves bone marrow + blood & maybe secondary lymphoid tissues
  • can ALL progress to AML:
    • suppression of hematopoiesis → peripheral blood cytopenias
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5
Q

what are the general features of chronic myeloproliferative neoplasms (MPN)? note:

  • pathogenesis
  • blood marrow status
  • peripheral blood status
  • key manifestations
A
  • pathogenesis: loss of regulation d/t tyrosine kinase abnormalities → factor-independent proliferation
    • maturation / differentiation = normal
  • bone marrow:
    • hypercellular
    • no dyspoesis EXCEPT megakaryocytes*
  • peripheral blood status:
    • increased cell counts with one type predominating → granulocytes m/c
  • manifestations: organomegally (spleen, hepato)
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6
Q

list the major types of chronic myeloproliferative neoplasms (MPNs)

A
  • chronic myelogenous leukemia (CML)
  • polycthemia vera
  • primary myelofibrosis
  • essential thrombocytosis (ET)
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7
Q

chronic myelogenous leukemia (CML) - pathogenesis

A

type of MPN.

  • like all MPNs, characterized by tyrosine kinase overactivity d/t defect.
    • defect: abnormal chromosome 22 (Ph chromosome). results in
      • a t(9,22) recipricol translocation - specifically, translocation of ABL gene (on chromosome 9) to BCR region of chromosome 22, producing BCR-ABL fusion gene.
        • the resulting BCR-ABL protein is a constitutively active tyrosine kinase, which leads to uncontrolled proliferation of myeloid progenitors:
          • granulocytes > megakaryocytes
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8
Q

chronic myelogenous leukemia (CML) - comprehensive presentation

A

= type MPN

  • divided into 3 stages of presentation:
    • chronic phase
    • accelerated phase
    • blast phase
  • overall findings:
    • peripheral blood
      • increased counts of
        • granulocytes ALWAYS (specifically, basophils)
        • +/- megakaryocytes,
        • NOT RBCS!!
    • bone marrow
      • no dyspoesis apart from megakaryocytes → dwarf megakaryocytes (hypolobulated)
    • clinical
      • splenomegaly common
      • NO lymphadenopathy
      • anemia sx: pallor, tachy / spnea (RBC suppression)
      • hyperuricemia → gout
      • bleeding OR thrombus depending on platelet #
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9
Q

chronic myelogenous leukemia (CML) - chronic phase. how does of the bone, blood and clinical presentation of CML appear during this phase?

A

= type of MPN

  • peripheral blood
    • increased counts of
      • granulocytes:
        • BASOPHILS INVARIABLY.
        • eosinophils commonly
      • +/- megakaryocytes,
      • NOT RBCS!!
    • blasts normal (< 2%)
  • bone marrow
    • hypercellular
    • +/- marrow reticulin fibrosis
    • no dyspoesis apart from megakaryocytes
      • dwarf megakaryocytes (hypo-lobulated)
    • blasts normal (<5%)
    • presence of Psuedo-Gaucher cells
  • clinical
    • splenomegaly common
    • NO lymphadenopathy
    • anemia sx: pallor, tachy / spnea (RBC suppression)
    • hyperuricemia → gout
    • bleeding (thrombocytopenia) OR thrombus (thrombyocytosis) depending on platelet #
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10
Q

chronic myelogenous leukemia (CML) - accelerated phase: how does of the bone, blood and clinical presentation of CML appear during this phase?

A

type of MPN;

overall, appears just like the chronic phase (with worsening sx) except:

  • blasts are increased above normal: though < 20% of peripheral blood or bone marrow cells
  • definite presence of reticulin fibrosis in bone marrow
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11
Q

chronic myelogenous leukemia (CML) - accelerated phase: how does of the bone, blood and clinical presentation of CML appear during this phase?

A

type of MPN:

overall, appears just like the chronic phase (with worsening sx) except:

  • blasts are increased over >20% of the peripheral blood or bone marrow
  • presence of granulocytic sarcoma: collections of malignant blasts cells
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12
Q

describe the presence of blast cells during each phase of chronic myelogenous leukemia (CML)

A

CML = type of MPN.

  • chronic phase: blasts normal
    • peripheral blood: < 2%
    • bone marrow: < 5%
  • accelerated phase: blasts abnormal but < 20% of peripheral blood or bone marrow
  • blast phase: blasts > 20% of peripheral blood or bone marrow → can form granulocytic sarcoma
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13
Q
A

abnormally large “buffy coat”: layer of leukocytes comprosing the tope of centrifuged peripheral blood

seen in CML: first during the chronic phase

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14
Q
A

dwarf megakaryocytes: hypo-lobulated nuclei

CML (type of MPN) - first seen in chronic phase.

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15
Q
A

BM aspirate smear showing blast cells > normal but under 20%

CML (type of MPN) - accelerated phase.

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16
Q
A

bone marrow with blast cells > normal but under 20%

CML (type of MPN) - accelerated phase.

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17
Q
A

pseudo-gaucher cell: sea-blue histiocytes

seen in CML (type of MPN) first in the chronic phase: d/t increased hemophagocytic activity in response to increased turnover of hematopoietic cells

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18
Q

polycythemia vera - pathogenesis

A

= type of MPN.

  • like all MPNs, characterized by tyrosine kinase overactivity d/t defect.
    • somatic gain of function mutation of JAK-2 VG17-F → unregulated proliferation independent of growth factors (in particular - erythropoietin, which will actually be low d/t negative feedback), resulting in panmyelosis: inc of all myeloid cell lines
      • granulocytic
      • erythroid
      • megakaryocytic
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19
Q

polycythemia vera - comprehensive presentation

A
  • occurs in three phases
    • pre-polycythemic phase
    • polycythemic phase
    • post-polycythemic (spent) phase
  • overall presentation
    • bone marrow
      • hypercellular - panmyelosis: hyperplasia of all 3 cell lines
      • no dypoiesis except for megakaryocytes → hyperlobulated
      • extensive reticulen / collagen fibrosis
      • tear-drop cells
    • peripheral blood
      • erythrocytosis + mild granulocytosis /megakaryocytosis that evolves to → cytopenia
  • clinical presentation: largely d/t polycythemia
    • plethora (red face)
    • HTN / headache / dizziness
    • erythromelalgia: paroxysmal severe burning pain in the skin of extremities
    • splenomegaly common
20
Q

polycythemia-pre-polycythemic phase: how does of the bone, blood and clinical presentation appear during this phase?

A

= type of MPN

  • peripheral blood: mild erythrocytosis (polycythemia)
  • usually asymptomatic
21
Q

polycythemia- polycythemic phase: how does of the bone, blood and clinical presentation appear during this phase?

A

= type of MPN

  • peripheral blood:
    • borderline / mild erythrocytosis (polycythemia) that results in increased
      • RBC
      • hemoglobin
      • hematocrit
    • mild granulocytosis, thrombocytosis
  • bone marrow:
    • hypercellular, d/t panmyelosis
    • +/- hyper-lobulated megakaryocytes
22
Q

polycythemia- post-polycythemic phase: how does of the bone, blood and clinical presentation appear during this phase?

A

= type of MPN

  • peripheral blood: cytopenia
  • bone marrow: prominent reticulin / collagen fibrosis
    • tear-drop cells
    • extramedullary hemopoiesis & splenomegaly
23
Q

what are the findings in the peripheral blood during each phase of polycythemia?

A
  • pre-polycythemia: mild erythrocytosis (polycythemia)
  • polycythemia: erythrocytosis (polycythemia) that results in increased → RBC, Hb, Hematocrit + mild granulocytosis / thrombocytosis
  • post-polycythemia: cytopenia
24
Q

identify picture, note important features

A

hypercellular bone marrow + hyper-lobulated megakaryocytes

polycythemia vera (type of MPN) - polycythemic phase

25
Q

identify picture, note important features

A

plethora (red face) - d/t polycythemia, which causes stagnation of blood flow

seen in polycythemia (MPN) - all phases, more likely as phases progress

26
Q

essential thrombocytosis (ET) - pathogenesis

A

= type of MPN

  • like all MPN, due to overactivity in tyrosine-kinase d/t some defect:
    • unlike CML & polycythemia - no genetic abnormalities specific for ET. it is associated with
      • JAK - V617 mutations - in 60%
      • CALR - in 20%
      • MPL mutations - in 3%
    • resulting in cell proliferation independent of growth factors:
      • megakaryocytes (thrombocytosis > 450,000)
27
Q

essential thrombocytosis - comprehensive presentation

A

type of MPN

  • peripheral blood
    • increased cell count of
      • megakaryocytes:
        • thrombocytes > 450,000
        • are LARGE & hyper-lobulated
      • NOT erythrocytes
      • NOT granulocytes
  • bone marrow:
    • normal cellularity
    • absent or minimal reticulin fibrosis
    • LARGE & hyper-lobuted megakaryocytes
  • clinical
    • NO SPLENOMEGALY
    • bleeding or clotting depending on function of thrombocytes
28
Q

identify picture, note important features

A

peripheral blood smear showing large, hyper-lobulated megakaryocytes + nucleated megkaryocyte fragments

in ET (type of MPN)

29
Q
A

bone marrow with LARGE & hyper-lobulated megakaryocytes

in ET (type of PMN)

30
Q

primary myelofibrosis - pathogenesis

A

= type of MPN

  • like all MPN, due to overactivity in tyrosine-kinase d/t some defect:
    • unlike CML & polycythemia - no genetic abnormalities specific for primary myelofibrosis it is associated with
      • JAK - V617 > CALR > MPL
    • leads to proliferation independent of growth factors:
      • marrow megakaryocytes
      • +/- granulocytes
      • NOT erythrocytes
31
Q

identify picture, note important features

A

peripheral blood showing thrombocytopenia with large, bizzare platelets + many tear-drop cells

primary myelofibrosis - fibrotic phase

32
Q
A

bone marrow: hypocellular with reticulin/collagen fibrosis & dilated sinuses

primary myelofibrosis - fibrotic phase

33
Q
A

bone marrow: hypocellular with reticulin/collagen fibrosis & dilated sinuses

primary myelofibrosis - fibrotic phase

34
Q

list the pathogenesis of each MPN

A

all: d/t overactive tyrosine kinase activity

  • CML: BCR-ABL1 fusion gene d/t t9,22 reciprocal translocation
  • polycythemia vera: JAK2 V617F gain of function (t9,22 negative)
  • ET: no specific genetic abnormalities
    • associated with
      • JAK - V617 > CALR > MPL mutations
  • primary myelofibrosis: no specific genetic abnormalities
    • associated with
      • JAK - V617 > CALR > MPL mutations
35
Q

how does each each MPN affect erythrocytes?

A
  • CML: no erythrocytosis & usually suppressed → anemia
  • polycythemia: erythrocytosis → cytopenia
    • pre-polycythemia: mild erythrocytosis
    • polycythemia: severe erythrocytosis → inc RBC / Hb / Hct
    • post-polycythemia (spent phase) cytopenia
  • ET: no erythrocytosis (normal)
  • primary myelofibrosis: no erythrocytosis & usually suppressed (anemia)
36
Q

how does each MPN affect megakaryocytes?

A

all 4 types often show megakaryocyte dyspoesis

  • peripheral blood:
    • CML - possible thrombocytosis
    • polycythemia: - mild thrombocytosis
    • ET - severe thrombocytosis: > 450,000 platelets, LARGE & hyper-lobulated
  • bone marrow:
    • CML: dwarf (hypo-lobulated) -1st seen in chronic phase
    • polycythemia: hyper-lobulated - 1st seen in polycythemic phase
    • ET: LARGE & hyper-lobulated + nucleated megakaryocyte fragments
    • primary myelofibrosis: atypia: clusters, hyperchromatic nuclei, “cloud like” nuclei
37
Q

how does each MPN affect granulocytes?

A
  • CML: invariable, marked granulocytosis → LEUKOCYTIC AND GRANULOCYTIC LEFT SHIFT + buffy coat
    • specifically:
      • BASOPHILIA ALWAYS
      • neutropenia, eosinophilia commonly
  • polycythemia: mild granulocytosis
    • d/t marrow pancytopenia !!
  • ET: no/ mild granulocytosis → NO LEFT SHIFT
  • primary myelofibrosis: no / mild granulocytosis → NO LEFT SHIFT
38
Q

how does each MPN affect bone marrow in terms of

  • cellularity
  • fibrosis
  • other features
A
  • cellularity
    • CML - hypercellular (M:E 10:1)
    • polycythemia - hypercellular (panmyelosis)
    • ET - normal cellularity
    • primary myelofibrosis - hypercellular → hypocellular
  • fibrosis
    • CML - yes: EMH in accelerated phase
    • polycythemia - yes: EMH in spent phase
    • ET - NO
    • primary myelofibrosis - yes: EMH in fibrotic phase
  • other features
    • CML - psuedo-gaucher cells (sea-blue histiocytes)
    • primary myelofibrosis - dilated sinuses in fibrosis
39
Q

which MPNs result in extramedullary hematopoiesis? during which phases?

A
  • CML - EMH in accelerated phase
  • polycythemia - EMH in post-polycythemic /spent phase
  • primary myelofibrosis - fibrotic phase
40
Q

which MPNs result in organomegally?

A
  • CML - splenomegaly > hepatomegaly: chronic phase
  • polycythemia - splenomegaly > hepatomegaly: spent phase
  • ET - NO ORGANOMEGALLY
  • primary myelofibrosis: SEVERE splenomegaly
41
Q

contrast the clinical presentation of each MPN

A
  • ALL can result in vascular abnormalities (thrombocytopenia or thrombocytosis) depending on megakaryocyte function
  • ALL EXCEPT primary myelofibrosis: splenomegaly
  • CML - anemia sx
    • palor
    • fatigue
    • tachycardia / dyspnea
  • polycythemia - polycythemia sx:
    • plethora
    • HTN
    • HA
    • dizziness / paresthesia
42
Q

aspirate from which MPN often shows a “dry tap”?

A

primary myelofibrosis

43
Q

which MPNs can result in peripheral tear-drop cells?

A
  • polycythemia
  • primary myelofibrosis
44
Q

which MPNs can result in peripheral tear-drop cells?

A
  • polycythemia
  • primary myelofibrosis

PATH Heme-Onc II (6 decks)

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