Lymphoma Flashcards
General B symptoms
Weight loss
Fever
Night sweats
Types of low-grade/indolent NHL
Follicular lymphoma
Mantle cell lymphoma
Marginal zone lymphoma
Small lymphocytic lymphoma
Lymphoplasmacytic lymphoma
Most common low-grade NHL
Follicular lymphoma
Types of high-grade/aggressive NHL
Diffuse large B cell lymphoma
Burkitt lymphoma
Peripheral T cell lymphoma
Most common high-grade NHL
DCBCL
Cell of origin of follicular lymphoma
Germinal center B cells
Prognosis of follicular lymphoma
Incurable, median survival of 7-9 years
Treatment of follicular lymphoma
Cytotoxic drugs
anti-CD20 antibody (rituximab)
Translocation associated with follicular lymphoma
t(14;18)
IGH;BCL2
Lymph node biopsy characteristics in follicular lymphoma
Nodular growth pattern
Centrocytes and centroblasts
Characteristics of centrocytes
Small cells with angular cleaved nuclei
Characteristics of centroblasts
Larger cells with open chromatin, several nucleoli, and modest cytoplasm
Indicated by a higher number of centroblastic cells in follicular lymphoma
More aggressive course
Immunophenotype in follicular lymphoma cells
CD19+
CD20+
CD10+
BCL2+ –> in most cases
Cancer that arises in lymph nodes, spleen, and extra-nodal tissues
Marginal zone lymphoma
Lymphoepithelial lesions description and associated cancer
Homing of malignant B cells to the epithelium
Marginal zone lymphoma
Beginning of marginal zone lymphoma and its cells of origin
Polyclonal immune reaction - chronic inflammation
Memory B cells
Cells of origin of mantle cell lymphoma
Naive B cell in mantle zone in men over 50
Histologic characteristics of mantle cell lymphoma
Vaguely nodular or diffuse pattern
Commonly involves bone marrow
Immunophenotyping of mantle cell lymphoma
CD20+
CD19+
CD5+
sIgM
IgD
Cyclin D1
Translocation associated with mantle cell lymphoma
t(11;14)
cyclin D;IgH
Clinical presentation of DCBCL
Rapidly enlarging mass at nodal or extra-nodal site
Most common nodal site is Waldeyer ring
Most common extra-nodal site is GI tract
Infrequently involves spleen or bone marrow
Genetic mutations associated with DCBCL
Dysregulation of BCL6
t(14;18) with overexpression of BCL2
MYC mutation
Cell types in DCBCL microscopy
Large cells
Centroblasts
Immunoblasts
Multinucleated giant cells and highly anaplastic cells
Characteristics of immunoblasts
Vesicular nucleus
Single, central, prominent nucleus
Abundant basophilic cytoplasm
Immunophenotyping of DCBCL
CD19+
CD20+
Variable germinal center B cell markers: BCL6, CD10, BCL2
General treatment of DCBCL
Intensive chemotherapy regimens
Stem cell transplant in relapse
Special types of DCBCL
Immunodeficiency associated large B cell lymphoma
Primary effusion lymphoma
Mediastinal B cell lymphoma
Associated characteristics of immunodeficiency associated large B cell lymphoma
Advanced HIV
Allogeneic bone marrow transplantation
Associated with EBV infection
HHV-4
EBV
Associated characteristics of primary effusion lymphoma
Affects pleural cavity, peritoneum, and/or pericardium
Kaposi sarcoma
Immunosuppressed pts
HHV-8
Karposi sarcoma/KSHV
Associated characteristics of mediastinal B cell lymphoma
More common in young women
Spreads to abdominal viscera and/or CNS
Cancer strongly associated with EBV in endemic type
Burkitt lymphoma
Types of Burkitt lymphoma
African/endemic
Sporadic/non-endemic
AIDS-related
Molecular pathogenesis of Burkitt lymphoma
All MYC translocations
t(8;14) - MYC;IgH
t(2;8) - Ig kappa light chain;MYC
t(8;22) - MYC;Ig lambda light chain
Clinical sign of endemic Burkitt lymphoma
Mandibular mass
Prognosis of endemic Burkitt lymphoma
Very aggressive tumor that responds well to intensive chemotherapy
Microscopy of Burkitt lymphoma
Moderate amount of deeply basophilic cytoplasm with numerous lipid vacuoles
Typical starry sky pattern
High mitotic activity and numerous apoptotic cells
Macrophages with cytoplasm containing nuclear debris
Explanation of starry sky pattern
Numerous, pale, tingible-body macrophages with ingested nuclear debris in a background of deep blue neoplastic cells
Immunophenotyping of Burkitt lymphoma
CD10+
CD20+
CD22+
Positive for monoclonal surface immunoglobulin
CD5-
CD10+
BCL6+
Classic types of HL
Nodular sclerosis
Mixed cellularity
Lymphocyte rich
Lymphocyte depletion
Non-classic type of HL
Nodular lymphocyte predominant (NLP)
Characteristics of Reed-Sternberg cells
Bilobed (owl-eye) nucleus
Large, inclusion-like nucleoli
Abundant cytoplasm
HL where RS cells are most common
Mixed cellularity type
RS cell imunophenotype
Co-expression of CD15 and CD30
Negative for CD45, sIg, common B antigens, and common T antigens
Common B lineage markers
CD19
CD20
CD22
CD79a
EBV infection is mostly associated with what type of HL
Mixed cellularity type
Mechanism of EBV infection leading to HL
Activates NFkB
Preventing apoptosis
Functional property of RS cells
Release cytokines
Cytokines released by RS cells
IL-5
IL-10
M-CSF
Chemokines
Purpose of IL5 released by RS cells
Infiltration of eosinophils
Purpose of M-CSF released by RS cells
Induces accumulation of macrophages and granulocytes
Specific characteristics of nodular sclerosis HL
Bands of collagen around lymphoid cell aggregate nodule
CD45 negative
Lacunar cells
Histology of lacunar cells
Folded/multilobed nucleus surrounded by an empty space
Immunophenotype of nodular sclerosis HL
CD15 and CD30 positive RS cells
Negative for EBV
Prognosis of nodular sclerosis HL
Excellent
HL with a biphasic distribution
Mixed cellularity
Morphology of mixed cellularity HL
Lymph node is diffusely effaced by a heterogenous infiltrate
Associated with EBV
Immunophenotype of mixed cellularity HL
Classic RS cells positive for CD15 and CD30
HL associated with primarily young male pts
Nodular lymphocyte predominant
Typical presentation in nodular lymphocyte predominant HL
Cervical or axillary lymphadenopathy
Not associated with EBV
Morphology of nodular lymphocyte predominant HL
Nodular infiltrates of small lymphocytes admixed with macrophages
LH variant RS cells with multilobed nucleus
Popcorn cells
LH variant RS cells with multilobed nucleus
Immunophenotype of LH variant RS cells
Express B cells markers, like CD20
Negative for CD15 and CD30
Stage I of Ann Arbor classification
Involvement of single lymph node region or a single extralymphatic organ/site
Stage II of Ann Arbor classification
Involvement of 2+ lymph node regions on same side of diaphragm OR localized involvement of an extralymphatic organ or site
Stage III of Ann Arbor classification
Involvement of lymph node regions on both sides of diaphragm
With or without localized involvement of an extralymphatic organ or site
Stage IV Ann Arbor classification
Diffuse involvement of one or more extralymphatic organs or sites
With or without lymphatic involvement
A or B meanings in Ann Arbor classification
Absence (A) or presence (B) of symptoms –> fever, night sweats, weight loss
Disease associated with HTLV-1 infection
Adult T-cell leukemia or lymphoma
Endemic areas for HTLV-1
Southern Japan
West Africa
Caribbean basin
Pathogenesis of HTLV-1 associated T cell lymphoma
HTLV-1 encodes Tax which activates NFkB
NFkB prevents apoptosis
Clinical presentation of adult T-cell leukemia
Skin lesions - plaques/nodules
Generalized lymphadenopathy
Hepatosplenomegaly
Prognosis of of adult T-cell leukemia/lymphoma
Rapidly progressive, aggressive disease
Smear histology of T cell leukemia/lymphoma
Peripheral blood lymphocytosis
Cells with multilobulated nuclei
Clover leaf or flower cells
Cell with multilobulated nuclei
Cause of mycosis fungoides
Tumor of CD4+ helper cells that home to the skin
Most common cutaneous T cell lymphoma
Mycosis fungoides
Three stages of mycosis fungoides lesions
Erythematous patches - inflammatory premycotic stage
Plaque stage
Tumor stage
Skin histology in mycosis fungoides
Epidermotropism - propensity of lymphocytes to colonize epidermis
Atypical lymphocytes palisade along dermal-epidermal junction
Mycosis fungoides with blood infiltration
Sezary syndrome
Immunophenotype of Sezary cells
CD4+
CD8-
Aberrant loss of pan T cell markers
Histology of Sezary cells
Hyper-convoluted cerebriform nuclei