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Heme/Lymph Exam > Pathology of Non-Hodgkins Lymphomas > Flashcards

Pathology of Non-Hodgkins Lymphomas Flashcards

1
Q

B cell lymphoblastic leukemia/lymphoma (B-LBL or B-ALL) - clinical features

A

*children > adults
*abrupt stormy onset; days-weeks of first symtpoms
*predominantly leukemic:
-peripheral blood/bone marrow involvement
-sx related to marrow dysfunction: fatigue, infections, bruising
-bone pain: marrow/subperiosteum infiltration
*extramedullary involvement frequent:
-CNS: headache, vomiting, and nerve palsies resulting from meningeal spread
-organomegaly: lymph nodes, spleen, liver, gonads

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2
Q

B cell lymphoblastic leukemia/lymphoma (B-LBL or B-ALL) - clinical course

A

*typically aggressive
*prognosis depends on cytogenetics
*80% cure rate in children

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3
Q

B cell lymphoblastic leukemia/lymphoma (B-LBL or B-ALL) - morphology

A

*primitive lymphoblasts replace tissue (Tdt+)
-no auer rods
-sites: peripheral blood/bone marrow; nodes and extranodal sites

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4
Q

B cell lymphoblastic leukemia/lymphoma (B-LBL or B-ALL) - phenotype

A

*IMMATURE B cells:
-Tdt, CD34
-CD19, CD10
-negative for CD20, Sig kappa/lambda
-Ki-67 proliferation > 90%

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5
Q

B cell lymphoblastic leukemia/lymphoma (B-LBL or B-ALL) - genotype

A

t(9;22) - BCR/ABL = favorable
t(4;11) AF4/MLL = unfavorable

hyperdiploidy > 50 = favorable
hypodiploidy < 44 = unfavorable

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6
Q

T cell lymphoblastic leukemia/lymphoma (T-LBL or T-ALL) - clinical features

A

*adolescents
*predominantly lymphomatous:
-mediastinal mass: compression of large vessels and airways; pleural effusions
-CNS: headache, vomiting, and nerve palsies resulting from meningeal spread
-organomegaly: lymph nodes, spleen, liver, gonads
*peripheral blood/bone marrow frequent:
-symptoms related to marrow dysfunction: fatigue, infections, bruising
-bone pain: marrow/subperiosteum infiltration

note - aggressive clinical course; good prognosis

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7
Q

T cell lymphoblastic leukemia/lymphoma (T-LBL or T-ALL) - morphology

A

*primitive lymphoblasts replace tissue
*no auer rods
*sites:
-anterior mediastinum/nodes
-peripheral blood/bone marrow
*thymus replaced by lymphoblasts

note - morphologically indistinguishable from B-ALL

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8
Q

T cell lymphoblastic leukemia/lymphoma (T-LBL or T-ALL) - phenotype

A

*IMMATURE T cells
-Tdt, CD34
-CD7, CD3
-CD4/8 CO-EXPRESSION (b/c immature)
-Ki-67 proliferation > 90%

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9
Q

T cell lymphoblastic leukemia/lymphoma (T-LBL or T-ALL) - genotype

A

*TCR genes rearranged
*30% cases translocations TCR loci

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10
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - clinical features

A

*90% of chronic lymphoid leukemias
*adults > 40 years; M>F
*USUALLY ASYMPTOMATIC
*lymphadenopathy +/- organomegaly

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11
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - peripheral blood

A

*absolute lymphocytosis > 5K
*small mature cells (scant cytoplasm, condensed, clumped “soccer ball” chromatin)
*“SMUDGE CELLS” (arrows on image)
*prolymphocytes < 55%
*anemia
*thrombocytopenia

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11
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - indolent course

A

*prognosis generally very good:
-asymptomatic pt: median survival > 10 years, even without treatment
-symptomatic patients are treated

note - “Richter transformation” = aggressive tumor progression, low median survival

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12
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - bone marrow

A

*diffuse interstitial lymphoid infiltrate or nodular lymphoid infiltrate

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13
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - lymph nodes

A

*diffuse effacement:
-monotonous round small lymphocytes
-clumpy “soccer ball” chromatin
*“pseudofollicular” proliferation centers

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14
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - phenotype

A

*MATURE B cells
-CD19, CD20, sIg kappa/lambda
-CD5+ (IMPORTANT - this is a T cell marker)
*Ki-67 proliferation < 20%

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15
Q

chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) - genotype

A

*IGH, IGL genes clonally rearranged
*karyotypic/genetic abnormalities prognostic:
-TP53 mutations = worse prognosis

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16
Q

follicular lymphoma - clinical features

A

*most common indolent NHL in US (25-30% of NHL; adults)
*often ASYMPTOMATIC
*“waxing/waning” lymph nodes - painless, generalized lymphadenopathy
*usually higher stage:
-multiple lymph node sites (III,IV)
-bone marrow involved
-may be extranodal: spleen, liver, GI, skin

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17
Q

follicular lymphoma - clinical course

A

*indolent, INCURABLE course
*prognosis generally good:
-“gentle” chemo
-median survival 7-9 years
*up to 30% progress to a more aggressive tumor

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18
Q

follicular lymphoma - morphology

A

*nodular growth pattern:
-effacing node
-“back-to-back” follicles
-extracapsular extension

*mixed cell composition:
-small “rasinoid” centrocytes with cleaved nuclei
-larger centroblasts

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19
Q

follicular lymphoma - phenotype

A

*MATURE B cells:
-CD19, CD20, sIg kappa/lambda
-CD10 (germinal center marker)
-BCL2 protein!!
-Ki-67% proliferation < 20%

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20
Q

follicular lymphoma - genotype

A

*IGH, IGL genes clonally rearranged
*t(14;18) BCL2/IgH genotype

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21
Q

diffuse large B-cell lymphoma - clinical features

A

*most common lymphoma in US
*all ages, more common in adults > 50 years
*RAPIDLY ENLARGING, SYMPTOMATIC MASS:
-nodal
-extranodal common
*may be de novo or secondary to other B-NHL

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22
Q

diffuse large B-cell lymphoma - clinical course

A

*aggressive, CURABLE course
*different distinct molecular subtypes have differing clinical outcomes
*intensive combination chemo & anti-CD20 immunotherapy

23
Q

diffuse large B-cell lymphoma - morphology

A

*diffuse, destructive growth
*LARGE LYMPHOID CELLS:
-pleomorphism
-irregular nuclear contours
-may have prominent nucleoli
*mitoses and necrosis common

24
Q

diffuse large B-cell lymphoma - phenotype

A

*MATURE B cells:
-CD19, CD20, sIg kappa/lambda
-other variable expressions (CD5, CD10, BCL2, etc)
-Ki-67 proliferation 50-100%
*MAY BE EBV ASSOCIATED

25
Q

diffuse large B-cell lymphoma - genotype

A

*IgH, IgL genes clonally rearranged
*non specific chromosomal or genetic abnormalities

26
Q

anaplastic large cell lymphoma - clinical features

A

*children and young adults
*rapidly enlarging, symptomatic mass
-nodal, extranodal common (skin, bone, soft tissue, liver, lung)
*may be advanced stage with B-symptoms (but it is a T cell lymphoma)

27
Q

anaplastic large cell lymphoma - clinical course

A

*aggressive, curable course
*>80% survival

28
Q

anaplastic large cell lymphoma - morphology

A

*complete to partial nodal effacement
*sinusoidal infiltrate
*large pleomorphic cells:
-“Hallmark” cells = reniform nuclei
-multi-nucleated “wreath” cells
-“donut” cells
-Reed-Sternberg cells

29
Q

anaplastic large cell lymphoma - phenotype

A

*MATURE T cells
*T-cell or “null” phenotype:
+/- CD3, CD4
+CD30
+/- ALK-1 protein

30
Q

anaplastic large cell lymphoma - genotype

A

*TCR genes clonally rearranged
*t(2;5) → ALK/NPM fusion protein
*genotype is important for distinguishing from Hodgkin’s Lymphoma: anaplastic large cell lymphoma has a 2;5 TRANSLOCATION; Hodgkin’s does NOT
-AND Hodgkin’s is B-cell; anaplastic is T-cell

31
Q

Classic Hodgkin Lymphoma (CHL) - clinical features

A

*bimodal age distribution: young adults (2nd and 3rd decade); elderly (>60 years)
*PAINLESS LYMPHADENOPATHY: mediastinal, cervical, para-aortic
*typically low stage I/II
*may have B-symptoms
*may be EBV associated

32
Q

Classic Hodgkin Lymphoma (CHL) - clinical course

A

*indolent, CURABLE course
*tumor stage most important prognostic factor
-stages I/IIA cure > 90%

33
Q

Classic Hodgkin Lymphoma (CHL) - morphology

A

*REED-STERNBERG CELLS
-multi/bi/mono-nuclear
1-10% of tumor cell population

*inflammatory background:
-eosinophils, neutrophils
-lymphocytes, plasma cells

*collagen fibrous bands (+/- nodular growth pattern)
*lymph node capsular fibrosis

34
Q

Classic Hodgkin Lymphoma (CHL) - phenotype

A

*“broken” mature B cells
*“null” lineage phenotype (negative: CD19, CD20, sIg kappa/lambda)
*positive for CD30, CD15 (Reed Sternberg cells)
*+/- EBV

34
Q

Classic Hodgkin Lymphoma (CHL) - genotype

A

*IgH, IgL genes clonally rearranged
*no specific chromosomal/genetic abnormalities

35
Q

plasma cell myeloma (multiple myeloma) - clinical features

A

*bone marrow involvement (bone fractures, pain):
-radiographically: multiple PUNCHED-OUT, LYTIC BONE LESIONS (spine, ribs, and skull especially)

*immunoglobulin protein secretion:
-MONOCLONAL SERUM GAMMOPATHY
-Bence Jones proteins found in the urine:
~monoclonal immunoglobulin kappa/lambda light chains

36
Q

plasma cell myeloma (multiple myeloma) - peripheral blood

A

*normochromic, normocytic anemia
*ROULEAUX FORMATION - correlates with magnitude of gammopathy
*occasional plasma cells
*leukopenia
*thrombocytopenia

37
Q

plasma cell myeloma (multiple myeloma) - bone marrow

A

*plasmacytosis with atypia (hypercellular marrow with replacement by plasma cells - see image):
-patchy, interstitial, focal, or diffuse
-multinucleation
-nucleoli
-cytoplasmic or nuclear inclusions

> 10% plasma cells for multiple myeloma (<10% for MGUS)

38
Q

plasma cell myeloma (multiple myeloma) - phenotype

A

*unique “post-mature” B cells in bone marrow:
-CD38, CD138, cytoplasmic Ig kappa/lambda
-CD56
-negative for BD19, CD20, sIg kappa/lambda

39
Q

plasma cell myeloma (multiple myeloma) - genotype

A

*IGH, IGL genes clonally rearranged
*IGH TRANSLOCATIONS [ex. t(11;14)]

40
Q

Burkitt Lymphoma - morphology

A

*“starry sky” histiocytes with apoptotic debris on biopsy of tumor and/or bone marrow
*effacing (destroying) underlying tissue architectures

41
Q

Burkitt Lymphoma - phenotype

A

*MATURE B-cells:
-CD19, CD20, sIg kappa/lambda
-CD10
*Ki-67 proliferation ~100%
*may be EBV-associated

note - negative for CD34, TdT

42
Q

Burkitt Lymphoma - genotype

A

*MYC (8q24) REARRANGEMENT:
t(8;14) MYC/IGH rearrangement !!!!

43
Q

Burkitt Lymphoma - prognosis

A

*potentially curable with intensive chemo
*treatment begins ASAP due to rapid doubling time or tumor
*include CNS prophylaxis

44
Q

hairy cell leukemia (HCL) - clinical features

A

*rare distinctive B-cell non-Hodgkin Lymphoma
*PANCYTOPENIA (weakness/fatigue, easy bruising, recurrent opportunistic infections)
*SPLENOMEGALY

45
Q

hairy cell leukemia (HCL) - clinical course

A

*indolent, CURABLE course
*sensitive to alpha-interferon, purine analogs 2-cda
*BRAF inhibitors

46
Q

hairy cell leukemia (HCL) - peripheral blood

A

*lymphocytosis:
-large, round, oval, reniform nuclei
-moderate/abundant pale cytoplasm with “hairy” or “villous” projections

*cytopenias: anemia, neutropenia, monocytopenia, thrombocytopenia

47
Q

hairy cell leukemia (HCL) - bone marrow

A

*lymphocytic infiltrate:
-“fried egg” cells (round cells with pale cytoplasm and distinct cell borders)

*“dry tap”:
-increased reticulin fibrosis
-inaspirable

48
Q

hairy cell leukemia (HCL) - phenotype

A

*MATURE B cells
-CD19, CD20, sIg kappa/lambda
-HCL markers: CD11c, Cd103, CD25

49
Q

hairy cell leukemia (HCL) - genotype

A

BRAF MUTATIONS !!!

50
Q

mycosis fungoides - clinical featuers

A

*rare, mainly affects adults/elderly
*CUTANEOUS INVOLVEMENT:
-widespread distribution +/- ulceration
-plaques/patches/papules
-tumors

note - it is a T cell lymphoma of the skin

51
Q

mycosis fungoides - clinical course

A

*indolent, INCURABLE course:
*long natural history:
-slow dissemination & long-term survival
-median survival 8-9 years
*prognosis depends on clinical stage: small may transform to more aggressive subtype

52
Q

mycosis fungoides - morphology

A

*dermal infiltration of medium-sized “cerebriform” cells
*epidermotropism with Pautrier microabscess

53
Q

Sezary Syndrome - clinical features

A

*systemic generalized disease
*LEUKEMIC FORM OF MYCOSIS FUNGOIDES (peripheral blood with sparing of bone marrow)
*generalized exfoliative erythroderma (pruritis, alopecia, etc)

54
Q

Sezary Syndrome - morphology

A

*peripheral blood:
-atypical lymphocytosis
-circulating Sezary cells and variants

*skin/other tissues:
-skin: histology similar to mycosis fungoides
-lymph nodes: paracortical expansion with atypical cerebriform cells

55
Q

Sezary Syndrome - phenotype

A

*MATURE T cells:
-CD3, CD4, also CD2, CD5
-loss of pan T-cell antigen: CD7
-CCR4, CCR10

Heme/Lymph Exam (38 decks)

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