Flashcards in Pathology of Lymphomas Deck (47):
Anaplastic large cell lymphoma is always what kind of cell?
b-cell or t-cell lymphoma more common?
B-cell is more common - accounts for over 90% of lymphoid neoplasms worldwide
lymphomas that arise from germinal center?
lymphomas that arise from mantle zone?
naive b-cell mantle cell lymphoma
lymphomas that arise from marginal zone?
memory b cell
marginal zone lymphoma
-clinically at diagonsis?
-middle age is common
-widespread disease at Dx
-peripheral and central lymphadenopathy
-PB 10% (PB usually not involved)
follicular lymphoma germinal center made up of what cells?
centroblasts & centrocytes
follicular lymphoma low grade has what cell type?
follicular lymphoma high grade has what cell type?
greater than 15 centroblasts/hpf
centrocyte appearance vs centroblast?
-centrocytes are more condensed (condensed = mature)
-blasts are looser looking = immature
***follicular lymphoma positive immunephenotype? negative staining?
+ =CD19, CD20, ***CD10***, and ***bcl-2***
- = CD5 and CD3
Mantle cell lymphoma
-lymphocytosis how often?
-organs usually affects?
-any other organs sometimes?
**-generalized lymphadenopathy, BM involvement, and liver involvement
-lymphocytosis in 30%
*-splenomegaly in 50% and liver common
-small bowel involvement - lymphomatoid polyposis
***mantle cell lymphoma - immunophenotypes + and -?
+ = CD19, CD20, **CD5**, & **cyclinD**
- = CD23 and CD3
-endemic BL (african)
-sporadic BL (non african, non endemic)
Endemic BL etiology:
-most common childhood malignancy
-4-7 yo, EBV+ in majority
sporadic BL etiology:
-throughout the world
-children and young adults
-30-50% of all childhood lymphomas
-med age of adult 30 yo
All BL patients are at risk for:
Endemic BL common sites:
-50% in jaws or facial bones
-ovaries, kidneys and breasts
Sporadic BL common sites:
-majority: abdominal masses, ileo-cecal region
-also: ovaries, kidneys and breast
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 - 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?
-Mature T-cell only CD4!
-caused by HTLV-1
-Central africa, caribbean, southwestern japan
-OCCURS ONLY IN ADULTS
Adult t-cell leukemia/lymphoma - presentation?
-not very specific:
--large spleen & liver
Case has japanese patient and some hypercalcemia disease may be?
Adult t-cell leukemia/lymphoma
Type of cell in adult t-cell leukemia/lymphoma?
adult t-cell leukemia/lymphoma - appearance of cells?
-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-
what cell type?
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-
-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-
2 simulataneous manifestations:
-generalized exfoliative eryhroderma
t-lymphoblastic leukemia/lymphoma -
-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
---under age 2
---presentation in adolescence or adulthood
---presence of philadelphia chromosome 9,22 or translocation involving MLL gene on ch11
anaplastic large t-cell lymphoma (ALCL):
-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
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
-EMA+, CD2, CD5, CD4 = positive 70%
-CD3 negative in 75%