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Exam 5 > Myeloid Neoplasms > Flashcards

Myeloid Neoplasms Flashcards

1
Q

Give rise to common myeloid stem cell or common lymphoid stem cell

A

Multipotent stem cell

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2
Q

Cannot self renew, but actively differentiate

A

Blasts

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3
Q

AML

A

Acute myeloid leukemia

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4
Q

Acquired genetic alterations causing impaired maturation of leukemic cells

A

AML

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5
Q

Include RUNX1, RARA, and NPM1 mutations

A

AML

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6
Q

Includes mutations of genes involved in signal transduction

FLT3, JAK2, and KIT

A

AML

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7
Q

Has auer rods. Myeloperoxidase positive.

CD34/CD13/CD33

A

Myeloblast of AML

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8
Q

Myeloperoxidase negative

CD11b/CD14/HLADR

A

monoblast

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9
Q

Associated with t(8;21), inv16, t(15;17), and t(11q23)

A

AML

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10
Q

Formation of chimeric gene with dominant negative activity. Interferes with fn of transcription activator, so have daughter cells unable to differentiate

A

inv 16 of AML

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11
Q

t(15;17) causes

A

acute promyelocytic leukemia

of AML

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12
Q

RARA gene fused to PML gene. PML/RARA protein blocks ___________ differentiation at _____________ stage

Translocation?

A

myeloid
promyelocytic stage

t(15;17)

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13
Q

Use topoisomerase inhibitors (1-3yr latency), alkylating agents (5-7 yr latency). Poor prognosis

A

AML

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14
Q

Similar symptoms to ALL. BM failure. Bleeding diathesis. Opportunistic infections. Granulocytic sarcoma

A

AML

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15
Q

Associated with DIC

A

Acute promyelocytic leukemia

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16
Q

t(15;17) acute promyelocytic leukemia is different - use _______ based chemo plus _______ or ________

A

Anthracylcine
ATRA
Arsenic

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17
Q

ATRA

A

All trans retnoic acid (vit A analogue)

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18
Q

t(8;21) and inv 16

A

Better prognosis

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19
Q

11q23

A

poorer prognosis

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20
Q

Refractory anemia only is _____ grade

A

low

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21
Q

Refractory anemia with excess blasts is ____ grade

A

high

22
Q

Clonal multipotent stem cell abnormality resulting in ineffective hematopoiesis with dysplasia

A

myelodysplastic syndromes

23
Q

Symptoms related to cytopenias. Therapy related - occurs after chemos

A

myelodysplastic syndromes

24
Q

Panycotpenic with macrocytic anemia. Dysplastic neutrophils.
Trilineage hyperplasia with dysplasia in at least one line

A

Myelodysplastic syndromes

25
Q

Monosomy 5 and 7
del5q, del7q, del20q
tri 8

A

Myelodysplastic syndromes

26
Q

Effective hematopoiesis, just too much of it.

Multipotent stem cell abnormality giving rise to all myeloid lines.

A

Chronic myeloproliferative diseases

27
Q

CMPD

A

Chronic myeloproliferative diseases

28
Q

Neoplastic cell is pluripotent giving rise to myeloid and lymphoid lines

A

CML

29
Q

Marrow hypercellularity with increased peripheral counts.

Genetic abnormalities have tyrosine kinase activity

A

chronic myeloproliferative disorders

30
Q

Can have extremedullary hematopoiesis

A

Chronic myeloproliferative diseases

31
Q

Pluripotent stem cell abnormality.
t(9;22) BCR-ABL fusion
Tyrosine kinase activity

A

CML

32
Q

Leukocytosis and neutrophilia with left shift.
Eosinophils and basophilia.
thrombocytosis 50% time

A

CML

33
Q

Packed BM with prominent granulocytic hyperplasia.
Seablue histiocytes due to increased cell turnover
Small dwarf megakaryocytes

A

CML

34
Q

Middle age
Extramedullary hematopoeisis causes LUQ pain and fullness
Slow progression to AML/ALL without treatment

A

CML

35
Q

Treat with tyrosine kinase inhibitor gleevac

A

CML

36
Q

Must rule out secondary polycythemia
Decreased serum erythropoietin
Multipotent stem cell (erythro, leuko, and thrombocytosis)
Jak2 mutation

A

polycythemia vera

37
Q

PV

A

polycythemia vera

38
Q

See erythrocytosis in PB
Hypercellularity in BM with trilineage hyperplasia
Minimal reticulin fibrosis
Late stage BM can become spent

A

PVV

39
Q

Late middle age.
Vascular consequences from increased RBC mass
Abnormal blood flow on venous side with stagnation/thrombosis
DVT, abnormal plt function

A

PV

40
Q

Least common of CMPDs. Older >60 usually
Platelet count >600K with abnormal large platelets
Diagnosis of exclusion

A

Essential Thrombocyothemia

41
Q

ET

A

Essential Thrombocythemia

42
Q

Asymptomatic.
Dysfn and numerous plts. Causes bleeding and thrombosis.
Unusual sites of thrombosis.
Symptoms related to microvascular thrombosis

A

ET

43
Q

Hypercellularity with huge megakaryocytes.

Indolent

A

Essential thrombocythemia

44
Q

Increased reticulin and collagen fibrosis.

Spent marrow and extramedullary hematopoeisis

A

Primary myelofibrosis

45
Q

Neoplastic megakaryocytes release PDGF and TGF-b

JAK2 mutation 50% time

A

Primary myelofibrosis

46
Q

Hypercellular marrow with trilineage hyperplasia
Megakaryocytes are large and dysplastic
Minimal fibrosis

A

Prefibrotic of primary myelofibrosis

47
Q

Hypocellular marrow with replacement by fibrosis.
Atypical clusters of megakaryocytes
Leukoerythroblastosis with marked erythrocyte anisopoikilocytosis and nRBCs

A

Fibrotic of primary myelofibrosis

48
Q

Usually in people >60

Fatigue with progressive anemia

A

Primary myelofibrosis

49
Q

Must differentiate and self renew. Greatest capacity for self renewal

A

Pluripotent stem cells

50
Q

Leukocytosis and absolute eosinophilia.
Fusion of PDGFRalpha and FIP1LI1
Pts respond to gleevac

A

Chronic eosinophilic leukemia/hypereosinophilia syndrome

Exam 5 (8 decks)

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