Hem & Onc - Pathology (Leukemias & Myelodysplastic syndromes ) Flashcards Preview

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Flashcards in Hem & Onc - Pathology (Leukemias & Myelodysplastic syndromes ) Deck (47)
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1
Q

What phrase characterizes leukemia?

A

Unregulated growth of leukocytes in bone marrow

2
Q

What general effects can leukemia have on hematologic components?

A

Unregulated growth of leukocytes in bone marrow –> (1) Increased or decreased number of circulating leukocytes in blood & marrow failure –> (2) Anemia (decreased RBCs) (3) Infections (decreased mature WBCs) (4) Hemorrhage (decreased platelets)

3
Q

Where can leukemic cell infiltrates be found?

A

Leukemia cell infiltrates in liver, spleen, and lymph nodes are possible

4
Q

What are the 3 major kinds of lymphoid neoplasms in leukemia?

A

(1) Acute lymphoblastic leukemia/lymphoma (ALL) (2) Small lymphocytic lymphoma (SLL) / Chronic lymphocytic leukemia (CLL) (3) Hairy cell leukemia

5
Q

In what age group do you typically see Acute lymphoblastic leukemia/lymphoma (ALL)?

A

Age: < 15 years

6
Q

How can T-cell ALL present, and why?

A

Mediastinal mass (leukemic infiltration of thymus)

7
Q

What effect does ALL have on peripheral blood and bone marrow?

A

Very significant increase (3 arrows up) of lymphoblasts (blood smear shows large nucleus - about 2x size of RBCs instead of the normal standard of being same size)

8
Q

What are significant markers for ALL?

A

TdT+ (marker of pre-T cand pre-B cells); CD10+ (pre-B cells only).

9
Q

What is the ALL response to therapy like? What leads to a better prognosis?

A

Most responsive to therapy; t(12;21) => better prognosis

10
Q

Where might ALL spread?

A

CNS & testes

11
Q

In what age group do you typically see SLL/CLL?

A

Age: > 60 years

12
Q

How does SLL/CLL often present? What is its progression? What is found on peripheral blood smear?

A

Often asymptomatic; Progresses slowly; Smudge cells in peripheral smear

13
Q

With what hematologic condition is SLL/CLL associated?

A

Autoimmune hemolytic anemia

14
Q

What is the difference between SLL and CLL, if any?

A

SLL same as CLL, except CLL has increased peripheral blood lymphocytosis or bone marrow involvement

15
Q

What age group does hairy cell leukemia typically affected? How is it characterized/defined?

A

Age: Adults; Mature B-cell tumor in the elderly

16
Q

How does hairy cell leukemia appear on blood smear?

A

Cells have filamentous, hair-like projections

17
Q

What stain was used for hairy cell leukemia? What change has been made related to this diagnosis technique?

A

Stains TRAP (tartate-resistant acid phosphatase) positive; TRAP stain largely replaced with flow cytometry.

18
Q

How is hairy cell leukemia treated, and what kind of compound is this?

A

Cladribine (2-CDA), an adenosine analog (resistant to adenosine deaminase degradation)

19
Q

With what condition is Acute lymphoblastic leukemia/lymphoma (ALL) associated?

A

Associated with Down syndrome.

20
Q

What type of cell neoplasm is associated with SLL/CLL? What are its cell markers?

A

CD20+, CD5+ B-cell neoplasm

21
Q

What is found on blood smear in SLL/CLL?

A

Smudge cells in peripheral blood smear

22
Q

What effect does Hairy cell leukemia have on the marrow, and how does this manifest clinically?

A

Causes marrow fibrosis –> dry tap on aspiration.

23
Q

What are 2 types of myeloid neoplasms?

A

(1) Acute myelogenous leukemia (AML) (2) Chronic myelogenous leukemia (CML)

24
Q

What is the age of median onset of Acute myelogenous leukemia (AML)?

A

Age: median onset 65 years; Adults

25
Q

What histological/lab finding is associated with AML? Describe it and give the context in which it is mostly found.

A

Auer rods = peroxidase + cytoplasmic inclusions seen mostly in M3 AML

26
Q

What is the significant smear finding found in AML?

A

Markedly increased circulating myeoblasts on peripheral smear

27
Q

What are 4 risk factors for AML?

A

Risk factors: (1) prior exposure to alkylating chemotherapy (2) radiation (3) myeloproliferative disorders (4) Down syndrome

28
Q

What is the chromosomal translocation associated with a particular subtype of AML? What is that specific type? To what does it respond, and what effect does this have?

A

t(15;17) –> M3 AML subtype responds to all-trans retinoic acid (vitamin A), inducing differentiation of myeloblasts

29
Q

What is a common presentation in M3 AML?

A

DIC is a common presentation in M3 AML (Think: Auer rod release may induce it, but mechanism not understood)

30
Q

What is another name for a t(9;22) chromosomal translocation? With what disorder is it associated? What is its phenotypic change?

A

t (9;22) (Philadelphia chromosome); CML (bcr-abl hybrid); Think: “Philadelphia CreaML cheese”

31
Q

With what disorder is the t(8;14) chromosomal translocation associated? What is its phenotypic change?

A

Burkitt lymphoma (c-myc activation)

32
Q

With what disorder is the t(11;14) chromosomal translocation associated? What is its phenotypic change?

A

Mantle cell lymphoma (cyclin D1 activation)

33
Q

With what disorder is the t(14;18) chromosomal translocation associated? What is its phenotypic change?

A

Follicular lymphomas (bcl-2 activation)

34
Q

With what disorder is the t(15;17) chromosomal translocation associated? To what therapy is it responsive?

A

M3 type of AML (responsive to all-trans retinoic acid)

35
Q

What are the peak incidence and median diagnosis ages for Chronic myelogenous leukemous (CML)?

A

Age: peak incidence 45-85 years, median age at diagnosis 64 years.

36
Q

What characteristic defines CML?

A

Defined by the Philadelphia chromosome (t[9;22], bcr-abl)

37
Q

What kind of proliferation occurs in CML?

A

Myeloid stem cell proliferation

38
Q

How does CML present in terms of cell findings?

A

Presents with increased neutrophils, metamyelocytes, basophils

39
Q

What physical exam finding is associated with CML?

A

Splenomegaly

40
Q

What is the relation between AML or ALL and CML?

A

CML may accelerate and transform to AML or ALL (“blast crisis”)

41
Q

How is CML distinguished from leukemoid reaction?

A

Very low leukocyte alkaline phosphatase (LAP) as a result of low activity in mature granulocytes (vs. leukemoid reaction, in which LAP is high)

42
Q

To what therapy does CML respond? What is the mechanism of this therapy?

A

Responds to imatinib (a small-molecule inhibitor of the bcr-abl tyrosine kinase)

43
Q

What kind of disorders are myelodysplastic syndromes, and what pathophysiological effects do they have?

A

Stem cell disorders involving ineffective hematopoiesis –> defects in cell maturation of all non-lymphoid lineages

44
Q

What are 2 causes of myelodysplastic syndromes?

A

Causes by de novo mutations or environmental exposure (e.g., radiation, benzene, chemotherapy).

45
Q

What are 3 examples of environmental exposures that may cause myelodysplastic syndrome?

A

Environmental exposures (e.g., radiation, benzene, chemotherapy)

46
Q

What risk results from myelodysplastic syndrome?

A

Risk of transformation to AML

47
Q

What is Pseudo-Pelger-Huet anomaly, and when is it typically seen? With what disorder is it associated?

A

Pseudo-Pelger-Huet anomaly - neutrophils with bilobed nuclei (two nuclear masses connected with a thin filament of chromatin) typically seen after chemotherapy; Myelodysplastic syndromes