B-cell Neoplasms 2021

B-Cell Neoplasms
Acute Lymphoblastic Leukemia/Lymphomas
and Mature B-cell Non-Hodgkin
Belinda R. Fender, M.D.
Copyright © 2018, A.T. Still University/Kirksville College of Osteopathic Medicine. This presentation is intended for ATSU/KCOM use only. No
part of this presentation may be distributed, reproduced or uploaded/posted on any Internet web sites without the expressed written
consent from the author or ATSU/KCOM Department Chairperson.
Lymphoid Neoplasms:
Two Major Groups
•Precursor lymphoid neoplasms (acute
•Mature lymphoid neoplasms
• Non-Hodgkin lymphomas/leukemias
• Hodgkin lymphomas
Leukemia vs. Lymphoma
Acute versus Chronic Disease
B-lymphocyte maturation
From Hematopathology, E. Jaffee et. al., p.108, Saunders, 2010.
Diffuse Large B-cell
B-lymphocyte development and function. Illustration by A. Y. Chen. Blood Journal, Nov.
2008. CLP, common lymphoid progenitor; SHM, somatic hypermutation; and CSR, class
switch recombination.
NHL – General Comments
• Older age group than acute leukemia
• Arise in lymph nodes or extranodal sites
• Spread is unpredictable.
• Staging not as useful as for HL.
• +/- monoclonal protein
• Many types
Relative Frequencies of Mature B-cell
Lymphomas in Adults
From: S. Swerdlow et. al., WHO Classification of Tumours of Haematopoietic and
Lymphoid Tissues, 4th ed., 2008, IARC Press, p. 164
Chronic Lymphocytic Leukemia/Small
Lymphocytic Lymphoma
• Most common leukemia in adults.
• Older adults, M>F
• Involves peripheral blood and bone marrow (if
• +/- involvement of lymph nodes, liver, spleen and
other sites.
• Fatigue, autoimmune hemolytic anemia, infections,
+/- M-protein
CLL/SLL lymphadenopathy
CLL/SLL: Peripheral blood
Smudge cell
CLL/SLL: Lymph Node
CLL/SLL: Bone Marrow
Maslak, P. ASH Image Bank 2009
CLL/SLL Natural History
• About 1/3 never need therapy.
• About 1/3 will eventually need therapy.
• About 1/3 will immediately need therapy.
• Rai or Binet staging system.
• Prognosis depends upon stage, performance status,
molecular/cytogenetic markers, and the
presence/absence of transformation.
• 0: Lymphocytosis in blood
and marrow
• 1: Lymphocytosis and
• II: Lymphocytosis and
• III: Lymphocytosis and anemia
• IV: Lymphocytosis and
• A: No cytopenia; <3 lymphoid
areas enlarged
• B: No cytopenia; >3 lymphoid
areas enlarged
• C: Anemia or
thrombocytopenia present
CLL/SLL Staging
CLL Progression
Richter Transformation
CLL-Prolymphocytic Leukemia
Diffuse Large B-Cell Lymphoma
• 30-40% of all non-Hodgkin’s lymphomas
• Middle age and elderly, most commonly.
• Usually arises de novo.
• Secondary cases have worse prognosis.
• Underlying immunodeficiency = risk factor.
• Aggressive. Requires therapy
DLBCL Risk Factors:
• Infection
• Immunodeficiency
• Autoimmune disease
• Pre-existing low-grade B-cell lymphoma
Diffuse Large B-Cell Lymphoma
DLBCL: Morphology
Size Assessment
Centroblastic Variant
Immunoblastic Variant
Anaplastic Variant
DLBCL variants (taken at different
Follicular Lymphoma
•Second most common NHL in U.S.
•Middle age – older adults
•t(14;18)(q32;q21) in most
•Indolent but incurable.
•Most present at late stage.
•Old grading system = 3 grades
•New grading system = 2 grades
•May undergo Richter transformation.
Follicular Lymphoma: Gross
Spleen with many small nodules
Lymph node. “Fish flesh”, nodular
Scanning view of lymph node
Centroblasts (large cells) and
centrocytes (small cleaved cells)
Follicular Lymphoma
Low grade (1-2)
High grade (3)
Follicular Lymphoma
Neoplastic follicle
(white open arrow,
lower left) is positive
for Bcl-2 stain.
Normal follicle (white
closed arrow, upper
center) is negative.
Immunostain for Bcl-2
Follicular Lymphoma in Blood—”Buttock”
Plasma Cell Neoplasms
•Neoplasms of terminally-differentiated
B-lymphocytes (plasma cells)
•Middle-aged to elderly persons
•Usually secrete monoclonal
•May be preceded by monoclonal
gammopathy of uncertain significance
•Malignant cells suppress proliferation of
normal plasma cells.
•Death usually from bleeding or
•Renal failure is common.
•DX based on combination of
pathological, clinical, and radiologic
findings: bone marrow plasmacytosis,
osteolytic bone lesions, monoclonal
gammopathy, end-organ damage
Punched out appearance of skull
Lytic lesions in skull
Normal Serum Protein Electrophoresis
Serum Protein Electrophoresis with
Monoclonal Protein
Serum Protein Electrophoresis
IgG Kappa on Immunofixation
Peripheral blood: Rouleaux
Atypical plasma cells on aspirate.
Appearance on the biopsy
Anaplastic myeloma, aspirate
Anaplastic myeloma, biopsy
Anaplastic Myeloma
Amyloidosis -- Pathogenesis
Perivascular Amyloid – H&E stain
Perivascular Amyloid – Congo Red
Plasmacytomas: Solitary Neoplasms of
Monoclonal Plasma Cells
• Extraosseous. Arise outside of bone marrow. 3-5%
of plasma cell neoplasms.
• Intraosseous. Arise within bone marrow. 3-5% of
plasma cell neoplasms.
• No clinical features of myeloma.
• Normal immunoglobulins.
• No anemia, hypercalcemia, or renal failure.
• Treat both types with radiotherapy.
Extraosseous plasmacytoma
•Most are in upper
respiratory tract and
•+/- low levels of
monoclonal protein.
•Good prognosis.
About 70% are
disease-free after 10
Solitary Plasmacytoma of Bone
Small M-protein in
up to 70%.
•Frequently present
with pain at site; may
have fractures.
•Up to 2/3 evolve into
myeloma or
Lymphoplasmacytic Lymphoma and
Waldonstrom Macroglobulinemia
• Rare lymphoma
• Older adults, slight male predominance
• Majority have M-protein, usually IgM (Waldenstrom
• Hyperviscosity in <30%.
• Involves bone marrow.
• May involve other organs.
• Incurable
Lymphoplasmacytic Lymphoma
Purpuric rash
Lymphoplasmacytic Lymphoma and
Waldonstrom Macroglobulinemia
Extranodal Marginal Zone Lymphoma
of MALT Type (MALT lymphoma)
•Generally arise in chronic
inflammatory disorders.
•Associated with Helicobacter pylori in
•Usually occur in adults.
•Indolent course. Usually localized
early, widespread in late stages.
Extranodal Marginal Zone Lymphoma
of MALT Type
Mantle Cell Lymphoma
• Middle-aged to older patients
• Male predominance.
• Lymph nodes most common
• Spleen and marrow, +/- blood involvement, or GI
• Most patients present in late stage.
• Median survival 3-5 years. Incurable
• t(11;14)(q13;q32), overexpression of cyclin-D1.
Mantle Cell Lymphoma
Lymph node. Low power magnification Lymph node. High power magnification
Starry sky appearance.
Macrophages, apoptotic cells.
Blastoid Mantle Cell Lymphoma
Burkitt Lymphoma
Above: Massive involvement of
ovaries by Burkitt lymphoma
Left: Lymphomatous involvement
of the face by Burkitt lymphoma
• Equatorial Africa.
• Mainly in children
• Jaw and facial bone involvement
common. Also gonadal, breast,
abdominal sites.
• Geographic location
corresponds to malaria.
• Worldwide, low incidence
• Children and young adults
• Abdominal tumors most
common; also gonads, kidneys
and breast. Jaw tumors are
• EBV positive in about 30%
Burkitt Lymphoma
Burkitt Lymphoma, Immunodeficiencyassociated
• Mainly seen in adults.
• Mainly seen in association with HIV/AIDS.
• Post-transplant
• Iatrogenic (drugs for autoimmune dz)
• Involves lymph nodes and bone marrow in most.
• EBV positivity varies with cause.
Burkitt Lymphoma Pathogenesis
• Immunophenotype:
• Mature B-cell markers
(CD19, CD20, CD10,
BCL6, CD38)
• CD34 and TdT are
uniformly negative.
(Not blasts.)
• Possibly is a
polymicrobial disease
(malaria, arboviruses,
t(8;14) is characteristic.
Burkitt Lymphoma: “Starry Sky”
Burkitt Lymphoma/Leukemia
Bone Marrow Aspirate
• May have leukemic
• Cells look blast-like.
• Deeply basophilic cytoplasm,
usually with many lipidcontaining vacuoles
• Immunophenotype of more
mature B-cells
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