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Pathology 1 Block 3 > WBC & LN Part, 3: Chronic Leukemias > Flashcards

WBC & LN Part, 3: Chronic Leukemias Flashcards

1
Q

what are the chronic Leukemias?

A

Chronic Lymphocytic Leukemia (CLL)

Hairy Cell Leukemia

HTLV-1

Mycosis Fungoides / Sézary syndrome

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2
Q

what is the most common type of leukemia in the Western world?

What increases the risk of this leukemia progressively?

what sex is more susceptible to this leukemia?

this leukemia is characterized by what?

what is the pathogenesis of this leukemia?

A

CLL (chronic lymphocytic leukemia)

age (older than 60 yrs), more in males, and insidious onset

accumulation of B cells that don’t function

clonal B cells get arrested in the B-cell differentiation pathway, intermediate between pre-B cells and mature B cells

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3
Q

what can we expect in the lab report for CLL?

Hb?

White cell count?

peripheral blood smear?

bone marrow?

immunoglobin?

markers?

A

Hb = anemia in late stage disease

White cell count = very elevated

peripheral blood smear = lymphocytosis (smudge cells)

bone marrow = involved due to neoplastic B cells

immunoglobin = low level of surface Ig expressed

markers = CLL expressed pan B cell markers (CD19, CD 20)

  • CD 5 and CD 23 will be positive
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4
Q

what is this? how do you know?

A

CLL, look at the smudge cells

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5
Q

what is this?

how do you know?

A

CLL

smudge cells

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6
Q

what is this?

how do you know?

A

Chronic Lymphocytic Leukemia: peripheral blood smear showing numerous small lymphocytes with condensed nuclei and scant cytoplasm.

‘smudge’ cells (red arrow). This is due to the cell fragility. These cells smudge while preparing the smear.

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7
Q

what are the clinical features of CLL?

A
  • Asymptomatic (incidental)
  • AIHA (autoimmune hemolytic anemia
  • Hepatosplenomegaly
  • Hypogammaglobulinemia (which predisposes to infections)
  • Thrombocytopenia
  • Generalized lymphadenopathy
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8
Q

CLL may transform to what?

What is Richter syndrome?

what is the its prognosis?

what causes it?

if there is transformation, what is the prognosis?

A

aggressive lymphoma

  • rapidly enlarging mass within lymph node/spleen which is a “diffuse large B cell lymphoma”
  • transformation is because of additional mutation
  • transformation is associated with poor prognosis (survival less than a year following transformation)
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9
Q

what is Hairy Cell Leukemia?

how do you spot hairy cell leukemia in a microscope?

On who does it happen the most?

what is the pathogenesis?

A
  • chronic lympho-proliferative disorder of B lymphocytes
  • cells have prominent cytoplasmic hair-like projections
  • Middle age, Male, age of 55 years
  • mutation in BRAF causing overexpression of cyclin D1 (cell cycle regulator)
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10
Q

what can be expected in a lab report in hairy cell leukemia?

Hb

total WBC count

platelet count

peipheral blood smear

Bone Marrow

A

Hb = decreased (anemia)

total WBC = decreased

platelet count = decreased (pancytopenia)

peripheral blood smear = neutropenia, presence of hairy cells

bone marrow = (dry tap)

  • leukemic cells have hair-like structure on surface of cytoplasm
  • involved, where they are enmeshed in extra cellular matrix
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11
Q

what is this?

A

hairy cell leukemia (hairy cells)

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12
Q

what is this?

A

hairy cell leukemia

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13
Q

what is this?

what is it for?

A

TRAP (tartrate-resistant acid phosphatase) stain

hairy cell leukemia

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14
Q

what are the Clinical features & Treatment: Hairy cell leukemia?

What type of infection are patients with Hairy cell leukemia more prone to?

what is the survival rate?

what is the treatment for hairy cell leukemia?

A
  • massive splenomegaly, consistent feature
  • lymphadenopathy is rare
  • pancytopenia from marrow involvement, and splenic sequestration

prone for infection: Mycobacterium avium intracellular

  • this may be due to unexplained monocytopenia

indolent course, survival rate is high

  • Cladribine (2-CDA), which inhibits adenosine deaminase (ADA) and increases the level of toxic deoxyadenosine
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15
Q

what is Adult T cell Leukemia / Lymphoma?

what is the latency for this disease?

what countries have the highest incidence of this disease?

how is it transmitted?

A

lympho-proliferative disorder seen in adults, associated with HTLV 1 infection

long latency

Japan, West Africa, Caribbean

blood, sexual, transplacental, breast milk (low risk)

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16
Q

What cell is affected in HTLV-1?

what is Adult T cell Leukemia’s pathogenesis?

What does TAX activate?

what does that do?

what will TAX inhibit?

A
  • it selects (tropism) CD4+ T cells
  • HTLV-1 has a region referred to as TAX (essential for viral replication) which can activate transcription of several host cell genes and at the same time inactivates cell cycle inhibitor (p16/INK4a) as well as activating cyclin D1
  • TAX activates NF-kB (transcription factor that helps by making cell anti-apoptotic)
  • Inhibits p53
17
Q

what can be expected in a lab report of Adult T cell Leukemia / Lymphoma?

CBC?

Peripheral blood smear?

biochemical test?

skin biopsy?

serum?

radiology?

A

CBC = leukocytosis

Peripheral blood smear = lymphocytosis, abnormal lymphoid cells described as‘flower cells’

biochemical test = hypercalcemia

skin biopsy = T-cell markers positive (CD4)

serum = HTLV-1

radiology = lytic bone lesions

18
Q

what is this?

how do you know?

A

adult T-cell leukemia/lypmhoma

because of ‘flower cells’ seen

19
Q

what are the clinical features of adult T-cell leukemia/lymphoma (HTLV-1)?

A
  • lymphadenopathy
  • hepato-splenomegaly
  • skin lesions
  • hypercalcemia
  • lytic bone lesions
20
Q

what is this?

A

adult HTLV-1

Note: Nodular skin tumors

21
Q

what is Mycosis Fungoides / Sézary syndrome?

A

Mycosis Fungoides and Sézary syndrome are different manifestations of tumor arising from CD4+ T helper cells

* they are a common type of cutaneous T-cell lymphoma

22
Q

what are the clinical features of Mycosis Fungoides / Sézary syndrome?

What will a immunophenotype reveal about Mycosis Fungoides / Sézary syndrome?

A
  • Mycosis fungoides progresses from patch to plaque to tumor phase
  • but patients may simultaneously have more than one type of lesion
  • indolent disease

mature T cell markers: CD2, CD3, CD4, on skin biopsies

23
Q

what is this?

what disease?

A

patches

Mycosis Fungoides

24
Q

what disease?

what is this?

A

Mycosis Fungoides

patches and plaque

25
Q

what disease?

what is this?

A

Mycosis Fungoides

tumors

26
Q

what disease is this?

what is this?

A

Mycosis fungoides

Leonine Facies

27
Q

what will histology reveal in Mycosis Fungoides / Sézary syndrome?

where may it spread?

A

histology shows infiltration of epidermis and dermis by neoplastic T cells, typically a “band-like and patchy infiltrate” in upper dermis of atypical lymphocytes

may spread to lymph nodes

28
Q

what disease is this?

what should be noted?

A

Mycosis Fungoides / Sézary syndrome

Note: ‘band-like’ infiltrate along dermo-epidermal junction

29
Q

what is this?

what should be noted?

A

Mycosis Fungoides

Note: disruption of the epidermal-dermal junnction. Abnormal lymphocytes are seen within the epidermis, and on surface.

30
Q

what disease is this?

what should be noted about this?

A

mycosis fungoides

Pautrier abscesses (collections of neoplastic lymphocytes) arrow

31
Q

what is Sézary syndrome characterized by?

how are sézary cells characterized?

A
  • is characterized by tumor dissemination to other organs and spill over into peripheral blood (Mycosis fungoides with a leukemic phase)
  • “Sézary cells” are characterized by cerebriform nuclei
32
Q

what are these?

A

Sézary cells

33
Q

what are Sézary cells?

A

They are medium to large lymphocytes with characteristic cerebriform nuclei and scant cytoplasm.

Pathology 1 Block 3 (7 decks)

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