Lymphocytosis and Lymphoid Leukemias Flashcards

1
Q

Similar to the myeloid counterparts (i.e. neutrophils, eosinophils), lymphocytes can be expanded in the blood in either reactive or neoplastic processes and this is referred to as:

A

lymphocytosis

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2
Q

A lymphocytosis is defined as an absolute lymphocyte count of

A

>4k/uL.

Clinical scenario, morphology, duration, and WBC count are important in differentiating benign (reactive) from neoplastic lymphocytoses.

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3
Q

In general, reactive increases in lymphocytes are_________, while neoplastic
proliferations are_______

A

transient

chronic (persisting for >6months)

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4
Q

Reactive expansions in general do not exceed
_______, while neoplastic lymphocytoses can cause extremely elevated WBC counts.

A

10k/uL

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5
Q

________expansions tend to have heterogeneous-appearing lymphocytes, which vary in size and cytoplasm

________proliferations tend to be composed of monotonous-appearing lymphocytes.

A

reactive

neoplastic

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6
Q
A
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7
Q

Most common causes of reactive leukocytosis are:

A

Infectious

transient stress lymphocytoses

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8
Q

List common infectious causes of lymphocytosis

A

Mononucleuosis (from EBV), cytomegalovirus (CMV), hepatitis, varicella,
adenovirus, toxoplasmosis, and pertussis

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9
Q

What are common causes of transient stress lymphocytoses?

A

trauma, myocardial infarctions, or seizures and rapidly reverse within
hours

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10
Q

Describe the morphology of lymphocytes seen in mononucleosis

A

heterogeneous, large, and have abundant, lightly basophilic cytoplasm, which
encroaches on neighboring red blood cells

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11
Q

In mononucleosis, lymphocytes represent ______, which are
responding to the EBV-infected B cells.

A

cytotoxic T cells

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12
Q

A presumptive diagnosis of infectiousmononucleosis can be made from peripheral blood smear examination by meeting the following 3 criteria:

A

1) >50% mononuclear cells in the differential (monocytes and lymphocytes)
2) marked lymphocytic heterogeneity
3) >10% reactive lymphocytes (>10/100 leukocytes).

(Confirmation of the diagnosis is made with identification of
heterophil antibodies in the “monospot” test.)

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13
Q

Neoplastic proliferations of mature lymphocytes include:

A

chronic lymphocytic leukemia (CLL), hairy cell leukemia, splenic marginal zone lymphoma, large granular lymphocytic leukemia, adult T-cell leukemia (ATLL), and Sezary syndrome

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14
Q

In neoplastic lymphocytosis, do we expect to see all disorders to present with elevated lymphocyte counts?

A

No, but abnormal lymphocyte morphology is certainly observed in the majority of these diseases.

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15
Q

In unexplained lymphocytosis, morphology may not be enough to identify the disease thus we should perform:

A

Flow cytometry

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16
Q

________will identify what
predominant cell type is present, as_______ does not allow definite distinction
between T cells, B cells, or NK cells.

A

Flow cytometry

morphology

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17
Q

By definition, the neoplastic proliferations will show
evidence of clonality how?

A

by monoclonal light chain expression

or immunophenotypic aberrancy
(abnormal antigen expression, such as CD5 on B cells).

(kappa or lambda-restricted populations of B cells)

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18
Q

would be polyclonal and demonstrate an admixture of kappa- and lambda-expressing B cells and CD4(+) and CD8(+) T cells.

A

Reactive expansions

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19
Q

Be able to recognize a histogram and what it is telling you

A

note the X and Y axsis. These will tell you if you have just kappa, just lambda or mixed

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20
Q

When neoplastic lymphoid cells involve the blood predominately, the process is termed a

A

leukemia

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21
Q

When neoplastic lymphocytes are predominately tissue-based (lymph nodes, spleen, liver), the process is termed

A

lymphoma.

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22
Q

How does WHO classify lymphoid neoplasms?

A

By cell of origin, cell size/morphology, genetics, and clinical features

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23
Q

Acute Lymphoblastic Leukemia/Lymphoma
(ALL) is predominately a ______ neoplasm defined by increased lymphoblasts

ALL is as immature lymphoid neoplasm***

A

pediatric

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24
Q

What is the precursor cell in ALL?

A

Precursor B cell in the bone marrow

25
Q

Salient Clinical Features of ALL

A

Predominantly children; symptoms relating to marrow replacement and
pancytopenia; aggressive

26
Q

What genetic abnormalities in ALL are associated with a good prognosis?

A

Hyperdiploidy

t(12;21)

27
Q

What genetic abnormalities in ALL are associated with a BAD prognosis?

A

 Hypodiploidy
 t(9;22)
 11q.23 abnormalities (MLL)

28
Q

What is the immunophenotype for ALL?

A

CD34(+)
CD10(+)
TDT(+)
CD19(+)
CD20(-)

29
Q

Cell of origin for T-cell acute lymphoblastic leukemia/lymphoma

(T-ALL)

A

Precursor T cell (often of thymic origin)

30
Q

Predominantly a_dolescent male_s; thymic/mediastinal masses and variable bone marrow involvement;
aggressive

A

Classic T-ALL

*board question

31
Q

what specific chromosomal abnormalities are associated with T-ALL?

A

None

32
Q

What is the immunophenotyping for T-ALL

A

CD3(+)
CD4-8 dual (+)
TDT(+)

33
Q

T-ALL and B-ALL are both examples of

A

immature lymphoid neoplasms

34
Q

Lymphocytosis composed of small lymphs with round nuclei and “soccer
ball” or“gingersnap” chromatin

A

Chronic lymphocytic leukemia/small lymphocytic lymphoma

(CLL/SLL)

35
Q

Clinical picture of individuals with Chronic Lmphocytic Leukemia

A

Older adults with bone marrow and lymph node disease; usually
asymptomatic; autoimmune hemolysis and thrombocytopenia in a minority; indolent

36
Q

What two cytogenetic abnormalities are associated with a good prognosis in CLL?

A

Good prognosis:
 Deletion 13q
 Mutated IgHV

37
Q

What two cytogenetic abnormalities are associated with INTERMEDIATE prognosis in CLL?

A

Trisomy 12

38
Q

What two cytogenetic abnormalities are associated with BAD prognosis in CLL?

A

Bad prognosis:
 Deletion 11q
 Deletion 17p
 Unmutated IgHV

39
Q

Immunophenotype:

CD5(+)
CD19(+)
CD23(+)

A

CLL

40
Q

Describe the morphology of hairy cell leukemia

A

Lymphocytes with “hairy” cytoplasmic projections and reniform nuclei

41
Q

Classic clinical picure of pt with Hairy cell leukemia

A

40-60y/o males with pancytopenia and
splenomegaly; indolent

42
Q

Chormosomal abnormality in hairy cell leukemia

A

No specific
chromosomal
abnormality

43
Q

Immunophenotype of hairy cell leukemia

A

CD19, CD20(+), CD11c, CD22 bright (+)

44
Q

What 2 mature B cell leukemias do we need to know

A

Chronic lymphocytic leukemia

Hairy Cell leukemia

45
Q

What is the cell of origin for Adult T-cell leukemia/ Lymphoma (ATLL)

A

Helper T cell
CD4(+),
CD25(+)

46
Q

HTLV-1 provirus
present in tumor cells

A

Adult T-cell
leukemia/Lymphoma (ATLL)

47
Q

Adults with skin lesions, hepatosplenomegaly, lymphadenopathy, and
hypercalcemia; endemic in Japan, West Africa, and the Caribbean due to HTLV
prevalence; aggressive

A

ATLL

adult T cell leukemia/lymphoma

48
Q

A pathologist points out the “Flower” cells on the HE smear, what is this indicitive of and what does it mean?

A

lymphocytes with lobulated nuclei; often lymphocytosis

Seen in ATLL

49
Q

What is the cell of origin in Mycosis fungoides/ Sézary syndrome

(MF/SS)

A

Helper T cell CD4(+)

50
Q

What genetic abnormality is present in Mycosis fungoides/
Sézary syndrome (MF/SS)?

A

No specific
chromosomal
abnormality

51
Q

What is the clinical picture of patient with mycosis fungoides/Sezary syndrome?

A

Adult patients with cutaneous patches, plaques, nodules (MF),

Sezary generalize erythema adn lymphadenopathy and is more aggressive

52
Q

A pathologist points out lymphocytes on a slide with “cerebriform” nuclei and powdery chormatin. The smear was taken from the blood.

Dx?

How would the Dx change if the sample was obtained from the skin?

A

Sezary Syndrome (blood)

Mycosis Fungoides from skin

53
Q

What is the clinical picture in a patient with Sezary Syndrome?

A

or generalized erythema and lymphadenopathy (SS); SS has a more aggressive behavior than MF

54
Q

What is the cell of origin in a patient with T-cell Large granular lymphocytic leukemia

(LGLL)

A

Cytotoxic T cell CD8(+)

55
Q

What is the genetic abnomality in a patient with T-cell Large granular lymphocytic leukemia

(LGLL)

A

No specific
chromosomal
abnormality

56
Q

What is the clinical picture in a patient with LGLL?

A

Adult patients with neutropenia, anemia, splenomegaly; may be
associated with autoimmune disease (i.e. rheumatoid arthritis); indolent

57
Q

Lab findings in patient with T-cell Large granular lymphocytic leukemia

(LGLL)

A

Lymphocytes with eosinophilic granules

58
Q

What are the three neoplasms of mature T cells?

A

Adult T cell leukemia/lymphoma (ATLL)

Mycosis fungoides/Sézary syndrome (MF/SS)

T-cell Large granular lymphocytic leukemia (LGLL)