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Pathology > Myeloid Neoplasms > Flashcards

Myeloid Neoplasms Flashcards

1
Q

Characterization of acute myelogenous (myeloid) leukemia? (AML)

A

Accumulation of immature myeloid precursors in the BM and suppression of normal hematapoiesis

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2
Q

Characteristics of Myelodysplastic syndromes?

A

Ineffective hematopoiesis with cytopenias

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3
Q

Characterization of chronic myeloproliferative disorders (CML)?

A

Increased production of terminally differentiated myeloid cells

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4
Q

What is found in the BM in AML?

A

Undifferentiated blasts

(results in anemia, neutropenia, and thrombocytopenia)

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5
Q

Diagnosis of AML?

A

Myeloid blasts >20% of marrow cells

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6
Q

M1 myeloblast characteristics in AML?

A
  • Delicate chromatin
  • 2-4 faint nucleoli
  • Cytoplasm with azurophilic peroxidase positive granules
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7
Q

M3 acute promyelocytic leukemia cell characteristics?

A
  • Auer rods (definitive evidence)
  • Peroxidase positive
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8
Q

M5 monoblast cell characteristics in AML?

A
  • Folded/lobulated nuclei
  • Esterase positive
  • NO auer rods or peroxidase
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9
Q

AML cell markers?

A
  • +CD34 (multipotent stem cell marker)
  • +CD33 (immature myeloid marker)
  • -CD64 (mature myeloid)
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10
Q

What is this? What can you distinctively see that indicates that?

A

Acute promyelocytic leukemia, M3

Can clearly see the Auer Rods

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11
Q

What is this?

A

AML, M1

Can see the faint nucleoli

Dispersed chromatin

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12
Q

What is this?

A

AML M5

The nuclei are lobulated/folded.

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13
Q

AML signs and symptoms?

A
  • Pancytopenia
  • Weakness, fatigue, infections, and bleeding
  • Less extramedullary tissue infiltration in comparison to ALL
  • M4/M5 differentiation:
    • Infiltration of the skin and gingiva may occur

(60% remission with therapy; only 15-30% disease free after a year)

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14
Q

Bone marrow in Myelodysplastic Syndromes? (MDS)

What are the two forms?

A

Marrow is partly or completely replaced by the clonal progeny of a defective stem cell

  • 2 forms
    • Idiopathic
    • Therapy related (2-8 yrs after cancer therapy)
      • More rapid transformation to AML
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15
Q

MDS clinical presentation? Survival?

A
  • Weakness
  • Infections
  • Hemorrhages
  • Survival = 1-2 years
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16
Q

MDS:

Describe A?

B?

C?

D?

A
  • A
    • nucleated RBC progenitors
  • B
    • Ringed sideroblasts
    • Iron in mitochondria (stains blue)
  • C
    • Neutrophils with only 2 nuclear lobes
  • D
    • Megakaryocytes with multiple nuclei
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17
Q

4 most common syndromes of chronic myeloproliferative disorders?

A
  • Chronic myelogenous leukemia
  • Polycythemia vera
  • Essential thrombocytosis
  • Primary myelofibrosis
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18
Q

Simmilar characteristics in the chronic myeloproliferative disorders?

A
  • Stem cells circulate and initiate extramedullar hematopoiesis
    • Organomegaly
  • All can progress to acute leukemia
19
Q

Chronic myelogenous leukemia (CML):

Cause?

A
  • Philadelphia chromosome 9:22 translocation
    • c-abl proto-oncogene (9)
    • bcr 22
    • transposition yields tyrosine kinase activity
20
Q

CML leukocytosis cells?

A

Neutrophils

Metamyelocytes

Myelocytes

21
Q

CML presentation?

Treatment?

A
  • Presentation
    • anemia, fatigue, weight loss, anorexia
    • splenomegaly
  • Treat
    • imatinib
22
Q

CML types of blast crises?

A
  • 70% myeloblast
    • AML-like
  • ALL-like
    • Tdt
    • CD10
    • CD19
23
Q

Polycythemia vera:

Cells produced?

A

Increased production of erythroid granulocytic, and megakaryocytic progenitors.

24
Q

Presentation of polycythemia vera?

A
  • Erythrocytosis, granulocytosis, and thrombocytosis in peripheral blood
  • Erythromelalgia
    • burning pain in feet/hands with erythema, pallor, or cyanosis
  • Abnormal blood flow: bleeding and thrombosis
  • Marrow fibrosis
    • Extramedullary hematopoiesis –> hepatosplenomegaly
25
Q

What do progenitor cells proliferate so quickly/much?

A

Decreased requirements for EPO and other growth factors

(serum EPO levels are very low)

26
Q

A FYI CARD IF YOU WANT TO LEARN IT:

Hemoglobin levels of PV?

Hematocrit levels?

WBC levels?

Platelet count?

A

Hb: 14-28 (normal 12-16)

Hct: >60% (normal 31-52%)

WBC: 12-50,000 (normal 4-11,000)

Platelets: >500,000 (normal 140-450,000)

27
Q

Essential thrombocytosis:

Proliferation of?

A

Megakaryocytic progenitors (with reduced requirement for growth factors)

28
Q

Platelets of essential thrombocytosis?

Marrow?

Peripheral blood?

A
  • Platelets
    • dysfunctional –> thrombosis and bleeding
  • Marrow
    • megakaryocytes
  • Peripheral blood
    • thrombocytosis with giant platelets
29
Q

Characteristic symptom of essential thrombocytosis?

A

Erythromelalgia

30
Q

Primary myelofibrosis:

What is this disease characterized by?

A

Obliterative marrow fibrosis that then results in suppression of hematopoiesis and causes cytopenias

(this leads to more extramedullary hematopoiesis; yet, is inefficient and cytopenia persists)

31
Q

What occurs to the bone marrow in primary myelofibrosis?

A
  • Collagen deposition (by normal cells)
  • Megakaryocytes are large and clustered
  • Later: fibrotic marrow may be converted to bone (osteosclerosis)
32
Q

Cell characteristics in primary myelofibrosis?

A
  • Nucleated erythroid progenitors in peripheral blood
  • Granulocytic progenitors in the peripheral blood
  • RBCs are teardrop shaped from being pushed through fibrotic marrow
    • Dacryocytes
33
Q

Signs and symptoms of primary myelofibrosis?

A
  • Anemia
  • Splenomegaly
  • Splenic infarcts
  • Infections
  • Thrombosis and bleeding
  • 5-20% transform to AML
34
Q

Langerhans cell histiocytosis:

Cell involved?

Categories?

A

Langerhans cell histiocytosis:

  • Involves immature dendritic cells
  • Letterer-Siwe syndrome
  • Hand-Schuller-Christian disease
  • Eosinophilic granuloma
35
Q

Cell markers in Langerhans cell histiocytosis?

A
  • Cell markers
    • CD1
    • S-100
    • HLA-DR
    • Birbeck granules
      • characteristic areas of dilation in the cytoplasm
36
Q

Multifocal multisystem Langerhans cell histiocytosis (letterer-siwe syndrome):

Tumor masses are composed of?

Organs involved?

Presentation?

A
  • Tumor masses
    • CD1+ langerhans cells
  • Organs
    • Spleen, liver, lung, and eventually BM
  • Presentation
    • Anemia, thrombocytopenia, and infections
37
Q

Unifocal and multifocal unisystem Langerhans cell histiocytosis (eosinophilic granuloma):

Characterized by?

A
  • Characterized by erosive accumulations of langerhans cells
    • Often in medullary cavities of bone
    • Eosinophil is the dominant infiltrate
      • Also plasma cells, lymphocytes, and neutrophils
38
Q

Unifocal lesion of Langerhans cell histiocytosis (eosinophilic granuloma)

Characterization?

A
  • Asymptomatic or local pain
  • Usually indolent
39
Q

Multifocal unisystem lesions of Langerhans cell histiocytosis (still eosinophilic granuloma):

Gross findings?

Signs/symptoms?

A
  • Gross findings
    • erosive growths in bone that may expand into soft tissue
  • Signs/symptoms
    • 50% have invasion of the posterior pituitary stalk
      • leads to diabetes insipidus
40
Q

What is the Hand-schuller-christian traid in Langerhans cell histiocytosis?

A
  • Involvement of:
    • The calvaria
    • Diabetes insipidus
    • Exopthalmos
41
Q

What is this?

A

Essential thrombocytosis:

large platelets

42
Q

What is this?

What is the black arrow pointing at?

A

This is primary myelofibrosis

Black arrow: teardrop shaped RBC

43
Q

What disease is this found in?

What in this image is distinctive?

A

This is in Langerhans cell histiocytosis

The birbeck granules with characteristic areas of dilation in the cytoplasm

Pathology (11 decks)

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