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Heme/Lymph 1 CP > 6b- Lymphocytosis/lymphoid leukemia > Flashcards

6b- Lymphocytosis/lymphoid leukemia Flashcards

1
Q

What is CLL/SLL?

A
  • CLL/SLL= chronic lymphocytic leukemia/small lymphocytic lymphoma
  • Most common leukemia in the western world
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2
Q

What are the demographics and presentation of CLL/SLL?

A
  • Mean age at diagnosis= 65 yo
  • Symptoms:
    • most patients asymptomatic (indolent disease)
    • worked up due to lymphocytosis
    • may have lymphadenopathy
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3
Q

What is Richter syndrome?

A

The transformation of an indolent CLL/SLL to aggressive B cell lymphoma (happens in 2-8% of cases)

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4
Q

What are some histological findings for CLL/SLL leukemia?

A
  • Leukemia= blood and BM involvement (“wet”)
    • Knotting lymphocyte chromatin
    • Smudge cells (not diagnostic for CLL/SLL but commonly seen)
    • BM lesions
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5
Q

What are some histological findings for CLL/SLL lymphoma?

A
  • Tissue based (lymph nodes, spleen, MALT)
  • Proliferation centers (don’t worry about identifying on exam)
    • pale, ill-defined nodules
    • prolymphocytes admixed with small lymphocytes with pale cytoplasm (“Soccer ball cells”)
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6
Q

How do you differentiate types of Small B cell leukemia/lymphoma?

A
  • CD5
  • CD23
  • CD10
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7
Q

Describe some good (3) and bad (4) prognostic factors for CLL/SLL

A
  • Good
    • low Rai stage
    • mutated Ig Heavy Variable (IGHV) gene
    • deletion 13q
  • Bad
    • high Rai stage
    • unmutated IGHV gene
    • CD38/ZAP-70 expression
    • deletion 11q, 17p
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8
Q

Describe Rai staging system

A

For staging CLL/SLL

(better prognosis the lower the stage)

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9
Q

What are the indications to treat CLL/SLL?

A
  • treat Rai stages III/IV
  • labs
    • rapid lymphocyte doubling time (<6 months)
    • Hgb < 10g/dL
    • Platelets < 100k/uL
  • clinical
    • autoimmune anemia/thrombocytopenia
    • richter transformation
    • enlarging symptomatic (or bothersome) lymph nodes
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10
Q

What are the treatment options for CLL/SLL?

A
  • chemotherapy
  • targeted cell therapy
    • BCL2 inh
    • PI3 kinase
    • BTK inh
  • immunotherapy
    • CD20 antibodies
  • cellular therapy
    • stem cell (BM) transplant
    • CAR-T cells
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11
Q

What is FCR treatment?

A
  • For CLL/SLL
  • Fludarabine + Rituximab + Cyclophosphamide
  • Combination of drugs for complicated course of treatment
  • Works better/worse depending on cytogenetics:
    • Better for trisomy 12, 13q and 11q deletion
    • Worse for 17p deletion
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12
Q

What is ibrutinib?

A
  • Oral irreversible BTK inhibitor
    • inhibits proliferation and survival
    • inhibits adhesion
    • modulates chemotaxes and trafficking
  • Effectiveness NOT determined by cytogenetics
  • NOT a cure (need to be on a daily pill for life)
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13
Q

What is venetoclax?

A
  • BCL2 inhibitor
    • pushes cells to commit suicide (normally BCL2 inhibits apoptotic pathway)
  • Better outcomes than chemotherapy
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14
Q

What disorders can present with atypical lymphocytes with OR without lymphocytosis?

A
  • CLL/SLL (usually presents with lymphocytosis)
  • Leukemic lymphoma (follicular, mantle, etc)
  • Hairy cell leukemia
  • Adult T cell leukemia/lymphoma
  • Sezary syndrome
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15
Q

What disease does this blood smear likely represent?

A

Hairy cell leukemia

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16
Q

Describe hairy cell leukemia

A
  • 40-60 yo males
  • splenomegaly (red pulp infiltration)
  • pancytopenia
  • “hairy” lymphocytes +/- lymphocytosis
  • TRAP + cells
  • BM fibrosis leading to “dry” spinal tap
  • Flow cytometry -> bright CD11c and CD22 expression
  • indolent disease
17
Q

What disease does this blood smear likely represent?

A

Sezary syndrome (lymphocytes with “cerebriform” nuclei)

18
Q

Describe Sezary syndrome

A
  • CD4 T cell neoplasm
  • triad: erythroderma, generalized lymphadenopathy, circulating sezary cells (cerebriform nuclei)
  • related to mycosis fungoides
  • may transform into aggressive T cell tumor
19
Q

What is mycosis fungoides?

A

Sezary cell infiltration of the epidermis

20
Q

What disease does this blood smear likely represent?

A

Adult T cell leukemia/lymphoma (“flower” cells)

21
Q

Describe adult T cell leukemia/lymphoma

A
  • CD4 T cell neoplasm
  • associated with retroviral infection (HTLV-1)
  • more common in Japan, Caribbean, and Africa
  • “flower cell” with lymphocytosis
  • hypercalcemia
  • aggressive clinical course
22
Q

What disease does this blood smear likely represent?

A

Large granular lymphocytic leukemia

23
Q

Describe large granular lymphocytic leukemia

A
  • lymphoporliferative disorder of CD8 T cells
  • indolent
  • usually presents with neutropenia, anemia, and splenomegaly
  • associated with autoimmune disease (esp rheumatoid arthritis)

Heme/Lymph 1 CP (12 decks)

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