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Pathophysiology > Hematopathology 1 > Flashcards

Hematopathology 1 Flashcards

1
Q

How are hematopoietic malignancies classified?

A

acute (presence of blasts - very immature cells)

chronic (presence of differentiated, mature cells)

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2
Q

What 2 cell types are involved in hematopoietic malignancies?

A

lymphoid

myeloid

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3
Q

What characterizes leukemias?

A

malignant neoplasms of hematopoietic cells that replace bone marrow

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4
Q

True or false: leukemic cells usually spill over into peripheral blood

A

TRUE

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5
Q

What differentiates a leukemia from lymphoma?

A

leukemia = present with widespread involvement of bone marrow and blood

lymphoma = describes proliferation arising as discrete tissue masses

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6
Q

What 3 blood metrics are common in ALL and AML?

A

anemia
neutropenia
thrombocytopenia

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7
Q

What are some clinical features of acute leukemias?

A
  • abrupt stormy onset

- depression of normal marrow function (fatigue (anemia) fever (neutropenia) bleeding (thrombocytopenia))

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8
Q

80% of acute leukemias of in children are _____

A

ALLs

acute lymphoblastic leukemia

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9
Q

When is the peak incidence for ALL in kids?

A

4 years (but most under 15 years)

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10
Q

What kind of cells are neoplastic in ALL?

A

lymphoblasts (precursor B or T lymphocytes)

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11
Q

What are the majority of ALL neoplastic cells?

A

pre-B cell neoplasms

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12
Q

What is the main population at risk for T-ALL?

A

adolescent males

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13
Q

What are microscopic tools to diagnose ALL?

A

lymphoblasts show scant basophilic cytoplasm and fine nuclear chromatin

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14
Q

What are the markers used to diagnose ALL?

A

Terminal deoxynucleotidyl transferase (TdT)

CD19, 20, 21 (B cells)
CD1, 2, 3, 4, 5, 7, 8 (T cells)

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15
Q

TdT is diagnostic for which blood cancer?

A

acute lymphoblastic leukemia

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16
Q

True or false: 90% of ALL patients have numerical or structural changes in chromosome of leukemic cells correlating with immunophenotype and prognosis

A

TRUE

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17
Q

What are the 4 chromosomal changes found in ALL?

A

1) hyperdiploidy (>50 chromosomes)
2) TEL1-AML1 (t(12;21))
3) BCR-ABL (t(9;22))
4) AF4-MLL (t(4;11))

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18
Q

What are 3 favorable prognostic indicators in ALL?

A

1) age 2-10
2) hyperdiploidy
3) t(12;21) TEL1-AML1

19
Q

What are 3 UNfavorable prognostic indicators of ALL?

A

1) age under 2
2) adolescent or adult
3) presence of t(9,22) PHILADELPHIA chromosome

20
Q

What is the philadelphia chromosome?

A

BCR-ABL t(9;22)

first genetic link to cancer

21
Q

What is acute myeloid leukemia?

A

a disease of adults peaking after 60 years (only 20% of childhood leukemias)

22
Q

What are the neoplastic cells in AML?

A

heterogenous disease (reflects the complexities of myeloid cell differentiation)

23
Q

What AML associated genetic abnormality has the worst prognosis?

A

MLL t(11q23;v)

24
Q

What are 4 WHO classifications of AML?

A

1) AML with recurrent genetic abnormalities
2) AML arising from myelodysplastic syndrome (MDS)
3) therapy related
4) not otherwise specified)

GENERALLY ARISE FROM MDS

25
Q

Define myelodysplastic syndromes (preleukemia)

A

clonal stem cell disorders showing defective and ineffective hematopoiesis with increased risk for transformation to AML

26
Q

What are the two types of MDS?

A

1) primary/idiopathic

2) therapy related (following chemo)

27
Q

What are signs of myelodysplastic syndrome?

A

1) hypercellular marrow with peripheral cytopenia (poor hematopoiesis)
2) clonal cytogenetic abnormalities
3) MDS arise on a background of stem cell damage
4) morphologic abnormalities seen in all lineages (mostly nuclear)

28
Q

How can the bone marrow be hypercellular but still have peripheral cytopenia in AML?

A

bone marrow cells apoptose

29
Q

What are some of the morphologic findings in myelodysplastic syndrome?

A

1) highly abnormal RBCs

2) megakaryocytes with multiple, separated nuclei

30
Q

What kinds of MDS subtypes are associated with a poorer prognosis?

A

those with higher blasts in blood or marrow

leads to worse cytopenia

31
Q

Which disease, ALL or AML, has more severe signs/symptoms related to infiltration of tissues?

A

ALL (although AML does have some mild lymphadenopathy and organomegaly)

32
Q

Which disease, ALL or AML leads to CNS problems?

A

ALL

33
Q

In which type of acute leukemia is DIC found?

A

acute promyelocytic leukemia 1(15;17)

releases procoagulants

34
Q

What is the most severe acute leukemia that if you suspect it even a little bit you should begin treatment?

A

APL (need to initiate therapy in order to avoid bleeding in the brain)

35
Q

What is the main morphological hallmark of APL?

A

auer rods (HUGEly diagnostic)

little rods in the cytoplasm

36
Q

What is the fusion gene for APL?

A

abnormal retinoic acid receptor that blocks myeloid cell differentiation

37
Q

How can you treat the differentiation block in APL?

A

trrans-retinoic acid

38
Q

What cancer can you treat by giving trans retinoic acid?

A

APL

39
Q

What are 4 diagnostic signs of AML?

A
  • myeloblasts >20%
  • myeloperoxidase or alpha napthyl butyrate esterase stains
  • CD13, 33, 34, 117
  • AUER RODS
40
Q

What is the strongest support of an AML diagnosis?

A

auer rods

41
Q

What type of cancer has multiple auer rods?

A

APL

42
Q

What is the prognosis for AML

A

GOOD for t(15;17) t(8;21) of inversions of chromosome 16

POOR for chromosome 11q23 (MLL)

43
Q

What two genetic mutations are implicated in AML and what are the prognoses for them?

A

FLT3 (BAD)

NPM (good)

44
Q

What is the treatment for AML?

A

chemo (60% achieve remission)

bone marrow transplant

Pathophysiology (26 decks)

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