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01 Hematology > Mature Lymphoid Leukemia > Flashcards

Mature Lymphoid Leukemia Flashcards

1
Q

About what portion of chronic lymphoid leukemias are chronic lymphocytic leukemia?

A

90%

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2
Q

In what condition can “soccer ball” chromatin been seen?

A

Chronic Lymphocytic Leukemia

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3
Q

What is the immunophenotypic hallmark of CLL?

A

B cells expressing CD5

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4
Q

What is the immunophenotype of CLL?

A

CD19, Cd5, CD23, CD20(weak), CD11c (weak), surface light chains (weak)

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5
Q

True or False: Generally, CLL is a curable disease since the cells don’t divide rapidly.

A

False: THe low replication due to their maturity makes it hard to cure

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6
Q

What are the two categories of CLL based on somatic hypermutation? Which has a better prognosis

A

Unmutated IGHV genes (worse prognosis); Mutated IGHV genes (better prognosis)

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7
Q

What are the independent prognostic markers from IGHV mutations?

A

CD38 and ZAP70

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8
Q

What kind of prognosis is associated with CD38 and ZAP70 mutations? Why?

A

Poor prognosis bcause expression of those proteins increases BCR- mediated signaling and cell proliferation (anti-apoptosis)

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9
Q

What are the clinical features of Hairy Cell Leukemia?

A

Hepatosplenomegaly, fatigue, weakness, and recurrant infections

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10
Q

What lab finding is unique to hairy cell leukemia?

A

Peripheral monocytopenia

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11
Q

The presence of Tartrate-resistance acid phosphatase (TRAP) is characteristic of what condition?

A

Hairy cell leukemia

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12
Q

What is the potential effect of hairy cell leukemia on the spleen?

A

Hairy cells concentrate in the red pulp and cause red pulp disease causing loss of distinction between red pulp and white pulp

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13
Q

What is the characteristic histologic feature of red pulp disease?

A

Red cell “lakes”

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14
Q

What is the cell morphology of Hairy Leukemic Cells?

A

Halo or fried egg appearance

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15
Q

What is the characteristic immunophenotype of hairy cell leukemia?

A

CD11c+, CD103+, CD25+

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16
Q

What is the ultimate conequence of bRAF mutations in Hairy Cell Leukemia?

A

Activation of MAPK pathway leading to transcriptional activation of cyclin D1

17
Q

What specific chemotherapy is targeted against mutant b-Raf?

A

Vemurafenib

18
Q

What is mycosis fungoides

A

Epiderotropic, primary cutaneous T cell lymphoma (CTCL)

19
Q

What is the morphology of mycosis fungoides histology?

A

IN the early phase there are band-like (lichenoid) lymphocytic infiltrate in superficial epidermis, with plaques there is increased intraepidermal collections of lymphocytes

20
Q

What is the most important prognostic factor for mycosis fungoides?

A

Extent of cutaneous/ extracutaneous disease

21
Q

What is Sezary Syndrome?

A

Mycosis fungoides’ cells but in the peripheral blood making the condition a leukemia

22
Q

What are Sezary cells?

A

CLonally related neoplastic T cells with cerebriform nuclei

23
Q

What is the triad of clinical features associated with sezary syndrome?

A

Erythroderma, generalized lymphadenopathy, presence of sezary cells in skin, lymph nodes, and peripheral blood

24
Q

True or False: Generally, Sezary Syndrome is an indolent neoplasm with long life expectancies?

A

False- aggressive disease, overall 5 yr survival 10-20 %

25
Q

What is the cause of Adult T cell Leukemia/ Lymphoma?

A

Human T-cell leukemia virus type 1 (HTLV-1)

26
Q

What are the clinical variants of Adult T cell Leukemia/ Lymphoma? Which is most common/? Which has the worst prognosis?

A

Acute (most common, worst prognosis), Lymphomatous, Smoldering, and Chronic

27
Q

In which clinical variant of ATLL is hypercalcemia seen?

A

Acute

28
Q

What is the characteristic morphology of ATLL cells?

A

Flower cells

29
Q

How can chronic/ smoldering forms of ATLL Lbe differentiated from MF?

A

ATLL has strong CD25 expression

01 Hematology (16 decks)

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