follicular lymphoma - common age? - clinically at diagonsis?

- middle age is common - widespread disease at Dx - peripheral and central lymphadenopathy - splenomegaly - BM- 40-70% - PB 10% (PB usually not involved)

Mantle cell lymphoma - age group? - sex predominance? - general presentation? - lymphocytosis how often? - organs usually affects? - any other organs sometimes?

-50-60s yr -male predominance -generalized lymphadenopathy, BM involvement, and liver involvement - lymphocytosis in 30% -splenomegaly in 50% and liver common - small bowel involvement - lymphomatoid polyposis

- equatorial Africa - most common childhood malignancy - 4-7 yo, EBV+ in majority

Pathology of Lymphomas Flashcards by Art F

Anaplastic large cell lymphoma is always what kind of cell?

T-cell bro!

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b-cell or t-cell lymphoma more common?

B-cell is more common - accounts for over 90% of lymphoid neoplasms worldwide

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lymphomas that arise from germinal center?

follilular
burkitt
large cell

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lymphomas that arise from mantle zone?

naive b-cell mantle cell lymphoma

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lymphomas that arise from marginal zone?

memory b cell
CLL/SLL
marginal zone lymphoma

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follicular lymphoma

common age?
clinically at diagonsis?

middle age is common
widespread disease at Dx
peripheral and central lymphadenopathy
splenomegaly
BM- 40-70%
PB 10% (PB usually not involved)

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follicular lymphoma germinal center made up of what cells?

centroblasts & centrocytes

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follicular lymphoma low grade has what cell type?

mostly centrocytes

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follicular lymphoma high grade has what cell type?

greater than 15 centroblasts/hpf

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centrocyte appearance vs centroblast?

centrocytes are more condensed (condensed = mature)

- blasts are looser looking = immature

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***follicular lymphoma positive immunephenotype? negative staining?

+ =CD19, CD20, CD10, and bcl-2

= CD5 and CD3

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Mantle cell lymphoma

age group?
sex predominance?
general presentation?
lymphocytosis how often?
organs usually affects?
any other organs sometimes?

*-50-60s yr
*-male predominance
*-generalized lymphadenopathy, BM involvement, and liver involvement
lymphocytosis in 30%
-splenomegaly in 50% and liver common
small bowel involvement - lymphomatoid polyposis

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***mantle cell lymphoma - immunophenotypes + and -?

+ = CD19, CD20, CD5, & cyclinD

= CD23 and CD3

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Burkitt lymphoma

-3 types?

endemic BL (african)
sporadic BL (non african, non endemic)
immunodef BL

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Endemic BL etiology:

equatorial Africa
most common childhood malignancy
4-7 yo, EBV+ in majority

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sporadic BL etiology:

throughout the world
children and young adults
30-50% of all childhood lymphomas
med age of adult 30 yo

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All BL patients are at risk for:

CNS involvement

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Endemic BL common sites:

50% in jaws or facial bones

- ovaries, kidneys and breasts

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Sporadic BL common sites:

-majority: abdominal masses, ileo-cecal region
-also: ovaries, kidneys and breast
RARELY JAW

Immunodeficient-associated BL common sites?

nodal localization and BM

BL - morphology:

DIFFUSE MONOMORPHIC INFILTRATE of intermediate sized round to slightly irregular lymphoid cells with multiple peripherally placed nucleoi
high mitotic rate
TINGIBLE BODY MACROPHAGES PRESENT (STARY SKY)

***STARRY SKY APPEARANCE?

BL!

***BL - immunophenotype:

+ and -?

\+ = CD10, CD19, CD20
- = blc-2

BL - prognosis:

very aggressive disease

- responds well to therapy

Adult T-cell leukemia/lymphoma - what kind of cell? - caused by what? - where most common? - age group?

- Mature T-cell only CD4! - caused by HTLV-1 - Central africa, caribbean, southwestern japan - long latency - OCCURS ONLY IN ADULTS

Adult t-cell leukemia/lymphoma - presentation?

- not very specific: - -large LN - -large spleen & liver * ***--hypercalcemia**** - -skin lesions

Case has japanese patient and some hypercalcemia disease may be?

Adult t-cell leukemia/lymphoma

Type of cell in adult t-cell leukemia/lymphoma?

CD4 T-cell

adult t-cell leukemia/lymphoma - appearance of cells?

- appearance varies - immature are smooth even chromatin with high nucleus to cytpolasm ratio - more mature chromatin, multilobated nuclei-flower cells or cloverleaf cells

Flower cells or clover leafs cells?

adult t-cell leukemia/lymphoma | not specific but need to rule out other potential Dx

adult t-cell leukemia/lymphoma - prognosis?

-very aggressve and most die within 1 year w/ treatment

new therapy for adult t-cell leukemia/lymphoma?

anti-CD52 antibody - alemtuzumab

which adult t-cell leukemia/lymphoma is more indolent?

when skin is primarily involved

Mycosis fungoides/Sezary syndrome- presentation? what cell type? survival?

One disease with two clinical manifestations - SKIN IS ALWAYS INVOLVED*** - malignant lymphocytes are CD4*** - indolent disease - 8to9 yrs survival-ish

Mycosis fungoides/Sezary syndrome- appearance for pathology?

malignant cells have cerebriform nuclei- **highly folded nuclear membrane**

Mycosis fungoides/Sezary syndrome- | -disease progression?

- skin lesions startwith *premycotic (patch) phase* which has few neoplastic lymphoid cells - PLAQUE PHASE-increased neoplastic lymphoid cells in epidermis - TUMOR PHASE - tumor masses, predominantly in dermis Progresses to involved LN and BMT-

Sezary syndrome-presentation:

2 simulataneous manifestations: - leukemia - generalized exfoliative eryhroderma

t-lymphoblastic leukemia/lymphoma - - whats important? - general presentation?

- immature t-cell lymphomas - medical emergency - patients are short of breath (SOB) - 85% presents as a mass in ant mediastium and cervial LN - 15% as leukemia - similar to B-ALL

T-cell ALL clinical presentation?

- abrupt stormy onset - within days to a few weeks of symptoms - depression of normal marrow: fatigue due to anemia, infection, bleeding, 2ndary to thrombocytopenia - ***MEDIASTINAL MASS***, lymphadenopathy, splenomegally, hepatomegaly due to neoplastic infiltration - CNS; headache, vomit, nerve palsies due to meningeal spread

T-cell ALL - morphology:

monotonous population of blasts similar to B-ALL -may have more clumped chromatin sheets of blasts in lymph node, soft tissue or bone marrow

****T-cell ALL - immunophenotype markers:

- Positive for: CD34, TdT, CD1a (IMMATURE MARKERS) | - CYTOPLASMIC CD3 + (neg for surface CD3)

T-cell ALL - prognosis:

- most T-ALL are higher risk - immature - more factors; - --under age 2 - --presentation in adolescence or adulthood - --presence of philadelphia chromosome 9,22 or translocation involving MLL gene on ch11 - --WBC>100K

anaplastic large t-cell lymphoma (ALCL): | -etiology:

- immature t-cell lymphoma - 10-20% of childhood lymphomas-- also seen in adults - involved LN and extranodal sites, mediastinal disease is less frequent then HL - aggressive

ALCL - immunophenotype +/- & prognosis

* **-ALK-1 positive = better prognosis | * **-ALK-1 negative = poor prognosis in adults

***distinct cell in ALCL?

HALLMARK CELLS - abundant cytoplasm and kidney shaped nuclei

ALCL - immunophenotype

-ALK+/-, *****CD30+**** - EMA+, CD2, CD5, CD4 = positive 70% - CD3 negative in 75% - CD15 engative

CD30 positive?

ALCL