Mature Lymphoid Neoplasms Week 6

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MALT

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Mucosa associated lymphoid tissue- lymphoid tissue by the respiratory and digestive tracts

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Lymphoid cortex

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Most superficial portion consisting of primary and secondary follicles

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Microscopic aggregates of small round naive B lymphocytes

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Primary follicles

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CD 19, CD 20, CD 5

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Naive B cell

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CD 19, CD 20, surface Immunoglobulin

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B- immunoblast

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CD 19, CD 20, CD 38, CD 138, surface Immunoglobulin

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Plasma cell

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CD 19, CD 20, CD 5+/-

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B- cell of primary follicle

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CD 19, CD 20, CD 10, BCL 6

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Centroblast- large B cell in germinal center w/ round vesicular nuclei, small nuclei adjacent to nuclear membrane and basophilic cytoplasm

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Site of high proliferative activity and somatic mutations of B cell immunoglobulin variable regions

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Dark zone

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Smaller cells w/ dense chromatin and irregular nuclear outlines, which form the light zone

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Centroblasts

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CD 19, CD 20, BCL 2, sIg

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Memory B cells

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CD 38, CD 138, cytoplasmic Immunoglobulin

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Plasma cell

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What is in the mantle of secondary follicles?

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Memory B cells

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This compartment generates immunocompetent T cells and is occupied predominantly by T cells and high endothelial vessels

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Paracortex

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CD 3, CD 5, CD 2, CD 7

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T cells

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Innermost portion of the lymph node contains plasma cells, cords and sinuses

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Medulla

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This compartment generates immunocompetent T cells and is occupied by T cells, dendritic cells and high endothelial venules.

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Paracortex

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Smudge cells

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CLL/ SLL

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Derived from circulating CD5 IgM IgD B cells

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CLL/ SLL

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CD19 CD20 CD5 CD23 kappa lambda chains

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CLL/ SLL

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CLL w/ trisomy of chromosome 12

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Rapidly progressive clinical course

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CLL with 13q14

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Post germinal center (memory) phenotype - more indolent clinical course

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Rare mature lymphoid leukemia. More than 55% circulating lymphoid cells have the morphology of a prolymphocyte

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Prolymphocytic Leukemia

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Punched out nucleolus in medium sized prolymphocyte Leukopenia

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Prolymphocytic leukemia

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Internal lymph nodes

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Mediastinal, hilar, mesenteric

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Difference between CLL and SLL

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SLL involves lymph nodes and lymphoid organs. CLL involves peripheral blood and bone marrow monoclonal B lymphocytes

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CLL/SLL w/ mutated Vh gene

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Memory B cells- post germinal center

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CD3 CD5 CD2 CD7

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T cells

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CLL/ SLL w/ naive B cells w/ no mutations in the variable region of immunoglobulin heavy chain Vh gene

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Pre-germinal center phenotype

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Presence of CD23 and absence of FM7 and cyclin D1 distinguish CLL/ SLL from _________

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mantle cell lymphoma

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Median age for CLL

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65

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Median survival for CLL

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10 years

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5% of CLL pts develop

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DLBCL (Richter transformation)

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Consequence of accumulating lymphocyte mass in CLL

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Neutropenia, Anemia and Thrombocytopenia

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Altered humoral immunity in CLL pts leads to

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Ig suppression, hypogammaglobulinemia, susceptibility to infections, autoimmune diseases: ITP,-autoimmune hemolytic anemia

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Body parts associated with prolymphocytic anemia

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PB, BM, spleen

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CD20 CD19 CD22 FMC7

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B cell PLL

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Nucleus resembling sezary cells, cytoplasmic blebbing CD3, CD5, CD7, CD4

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T cell PLL

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Filled w/ macrophages or histiocytes

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Sinus

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B cell PLL median survival

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3 yr

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T cell median survival

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1 yr

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Median age for PLL

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70 yrs

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PLL high incidence of mutation in

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Tumor suppressor gene TP53

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PLL or CLL? Extreme leukocytosis >100x 10^9 /L

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PLL

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PLL or CLL? Density of CD20 and surface light chain is higher

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PLL

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____ distinguishes PLL from mantle cell lymphoma

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CD5 antigen

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T- CLL/ PLL differentiation from B cells

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Alpha naphtol acetate esterase and acid phosphatase rxn

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Small B lymphocytes with abundant cytoplasm and fine cytoplasmic projections

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Hairy Cell Leukemia

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HCL cells are found in

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Bone marrow and red pulp of the spleen

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Most common reactive lymphadenopathy

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Follicular hyperplasia

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CD19 CD20 CD22 CD11c CD25 CD103

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Hairy cell leukemia

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+TRAP tartarate resistant acid phosphatase, DBA 44 and annexin A1 stains

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HCL

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BM biopsy shows reticulun fibers due to production of cytogenetic cytokines of leukemic cells

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HCL

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Lymph node involvement is rare

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HCL

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Rare lymphproliferative disorder occurring in middle aged ppl ~55, splenomegaly, marrow fibrosis, pancytopenia

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HCL

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Durable remission w/ interferon alpha or purine analogues

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HCL

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Irregular nuclear outlines, strong CD 20 + light chain expression, immunoreactivity of cyclin D1

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Mantle cell lymphoma MCL

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Follicular lymphoma originates from

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Germinal centers

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BCL2 pan B cell markers multiple marginated nuclei, angular or indented nuclei

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Follicular lymphoma

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Treatment for follicular lymphoma

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DLBCL w/ doxorubicin regimen

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In adults, it occurs in association with autoimmune disorders: HIV, syphilis, arthritis. In children, rxn to infections

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Follicular hyperplasia

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Median age for follicular lymphoma

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59

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Associated with autoimmune disorders: Sjogren. Hashimoto thyroiditis

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MALT lymphoma

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cD20, CD19,monoclonal Ig CD43

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MALT lymphoma

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Multifocal accumulation of malignant plasma cells in BM

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Lytic bone lesions

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Monoclonal proliferation of terminally differentiated B cells- plasma cells

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Plasma cell neoplasms

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M spike in immunofixation electrophoresis. IE on urine -> 2 monoclonal bands: IgG kappa + free kappa light- Bence Jones

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Plasma cell neoplasm

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Mott cells, flame cells,

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Plasma cell neoplasm

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Bone pain, renal insufficiency, cytopenias, decreased Ig levels

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Plasma cell neoplasms

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Most common lymphoma

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Diffuse large B cell lymphoma

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Lymph nodes, g tract, cns, bm, large cell sz

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Diffuse lg B cell lymphoma

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Associated with viral infections, drug reactions, chronic skin infections- small lymphocytes and immunoblasts

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Paracortical expansion

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Medium shaped highly proliferative lymphoid cells w/ basophilic vacuolated Cytoplasm CD19 CD 20 CD10 BCL6

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Burkitt lymphoma

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Immunodeficiency associated Burkitt lymphoma is a

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Nodal disease occurs predominantly in HIV pts

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Highly proliferative due to constitutive expression of MYC gene secondary to its translocation under promotors of Ig heavy or light chains: t(8:14, t(2:8), t(8:22)

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Mature B cell lymphomas- Burkinlphoma

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Mycosis fungoides and Sezary syndrome, peripheral T cell lymphoma unspecified, anaplastic large cell lymphoma

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Mature T and Natural Killer Cell Lymphomas

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Toxoplasma gondii infection produces

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Florid Follicular hyperplasia and paracortical expansion

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Strongest risk factor for development of lymphproliferative disorder

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Altered immune function

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________ produce monoclonal light chain immunoglobulins, clonal immunoglobulin gene arrangements or both

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B cell lymphoma

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Mycosis fungoides and Sezary syndrome, peripheral T cell lymphoma unspecified, an aplastic large cell lymphoma

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Mature T and Natural Killer Cell Lymphomas

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Pan T cell markers, epidermis and dermis may spread to lymph nodes, sm to md sz lymphoid cells w/ irregular lines (cerebriform nuclei)

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Mycosis Fungoides

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Most common cutaneous lymphoma

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Mycosis Fungoides

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Pan T cell markers, epidermis and dermis may spread to lymph nodes, sm to md sz lymphoid cells w/ irregular lines (cerebriform nuclei)

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Mycosis Fungoides

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CD 30, CD15 in 80% of cases, CD20 weak to absent

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Reed Sternberg cell

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RS, Hodgkin and Lymphoma cells- fibrotic bands, background of sm lymphocytes, histiocytes and eosinophils

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Hodgkin Lymphoma- Nodular Sclerosis

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Most common cutaneous lymphoma

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Mycosis Fungoides

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Disseminated diseases w/ leukemic presentation and skin and lymph node involvement

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Sezary syndrome

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Md sz cells w/ cerebriform nuclei, at least 1000 Sezary cells/ mm^3

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Sezary syndrome

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CD4:CD8 more than 10. Loss of CD26 and/ or T cell markers on CD4

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Sezary syndrome

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Who does Hodgkin lymphoma occur in

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Young individuals

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Prognosis of nodular lymphocyte predominant Hodgkin Lymphoma

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80-90% survival rate

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B cell neoplasm w/ sm lymphocytes and scattered lymphocytic/ histiocytic or popcorn cells Lg cells w/ abundant cytoplasm + multilobulated nuclei

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Nodular lymphocytic predominant Hodgkin Lymphoma

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Heterogenous grp of lymphoid neoplasms derived from the germinal center- in peripheral lymph nodes except in nodular sclerosis which often shows mediastinal lymphadenopathy

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Classical Hodgkin Lymphoma

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Rees Sternberg cells characteristic of

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Hodgkin lymphoma

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RS, Hodgkin and Lymphoma cells+ sm lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, no fibrotic bands

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Hodgkin Lymphoma- mixed cellularity

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RS, Hodgkin and Lymphoma cells+ diffuse nodular background of sm, lymphocytes, no or few eosinophils and neutrophils

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Hodgkin Lymphoma- Lymphocyte rich

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RS, Hodgkin and Lymphoma cells+ numerous RS cells and Hogkin lymphoma cells, few background lymphocytes

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Hodgkin Lymphoma- lymphocyte depleted

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is a condition in which there are abnormal increases in the concentration of gamma globulins (antibodies) in the blood.

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A gammopathy

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Reactive conditions such as chronic infections where several bacterial antigens (flagellar, somatic wall, capsular, DNA, etc.) may be presented to the host at one time

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polyclonal gammopathy

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plasma cell dyscrasia

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monoclonal gammopathy

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results in the appearance of an “M” spike on serum electrophoretic patterns

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monoclonal Gammopathy

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a) Hyperglobulinemia with a peak in the gamma region

b) Bone marrow plasmacytosis <10%

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Monoclonal Gammopathy of Undetermined Significance (MGUS)

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A small number (10-15%) will eventually develop a plasmacytic or lymphocytic malignancy.

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Monoclonal Gammopathy of Undetermined Significance (MGUS)

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most common gammopathy

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multiple myeloma

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may present as a solitary tumor (localized) or as a systemic disease (more common) affecting many organs or areas of the body.

2. Features of the fully developed disease are related to bone destruction, replacement of normal marrow with plasma cells, and hyperglobulinemia

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Multiple myeloma

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The disease is rare before the age of 40 with the peak occurring in the 60-70s.

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Multiple myeloma

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Most patients experience an asymptomatic period during which the disease is developing but there are minimal outward signs of anything happening.

2. Nonspecific findings during this time

a)M spike

b) Increased ESR

c) Persistent unexplained proteinuria

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Multiple myeloma

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Increased susceptibility to infections: Defective normal antibody production, Increased gamma globulin catabolism, Granulocytopenia

Skeletal pain: invasion of red marrow by neoplastic cells, osteolytic lesions, Osteoporosis, Bone destruction, Renal failure, Hyperviscosity syndrome, Hypersensitivity to cold, Abnormal bleeding, Amyloidosis, Neurologic manifestations

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Multiple myeloma

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A mild normocytic-normochromic anemia, occasionally a severe anemia. folic acid deficiency. megaloblastic changes. Rouleaux formation due to hypergammaglobulinemia, ESR is increased, WBC count normal or neutropenic, Platelet count normal.

The stained blood smear frequently has a darker background than normal blood as a result of the gamma globulins “holding” some of the stain

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PB Multiple myeloma

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The bone marrow is hypercellular showing sheets and clusters of plasma cells of varying stages of maturity.

b) Plasma cells may be moderately large (15-30

microns), round or ovoid with eccentric nuclei and dark cytoplasm.

c) Variant forms of plasma cells (Mott, Morulla, grape, and flame) may be seen

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BM Multiple myeloma

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myelomas type of light chain

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kappa or

lambda, but not both.

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multiple myeloma w/ the best prognosis

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IgG

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percentage of Myelomas w/ Immunoglobulin Class IgG

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50

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percentage of Myelomas w/ Immunoglobulin Class IgA

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25

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percentage of Myelomas w/ Immunoglobulin Light chains (Bence Jones)

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25

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treatment for multiple myeloma

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keep the patient mobile and reduce the tumor load

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“cured” of multiple myeloma?

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They may enter remissions following

aggressive treatment but rarely is a patient

considered “cured” of multiple myeloma.

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individuals produced a macroglobulin similar to IgM but much heavier

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Waldenstrom’s Macroglobulinemia

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Visual problems (dilated veins, blurred vision) are

common.

d) Circulatory impairment and congestive heart failure may occur.

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Waldenstrom’s Macroglobulinemia

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A moderate to severe, normocytic-normochromic anemia is frequently present.

b) Blood shows a relative and/or absolute lymphocytosis with plasmacytoid lymphocytes often appearing atypical or immature-looking.

c) Marked rouleaux and an increased ESR are present.

d) Serum electrophoresis shows an M spike identified as IgM

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Waldenstrom’s Macroglobulinemia

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produced by plasmacytoid lymphocytes – cells that appeared to be an intermediate stage between the mature B cell and a plasma cell.

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macroglobulin w/ a high molecular
weight (>1,000,000) IgM with a Svedberg
concentration of 18-20s

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Waldenstrom’s Macroglobulinemia Survival

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months to 10 years

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proliferation of plasmacytic and reticulum cells.

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Heavy Chain Disease (HCD)

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This condition is most frequently seen in young

Mediterranean individuals and has been called

Mediterranean lymphoma.

2.The patients often present with an abdominal mass.

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Alpha HCD

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The rarest of the heavy chain diseases.
Splenomegaly + hepatomegaly are common.
peripheral blood has many circulating lymphocytes, looks like CLL

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Mu HCD