Lymphomas and Myelomas Flashcards
affects all ages; male predominance; insidious onset; painless lymphadenopathy; w/ or w/o fever, night sweats, and/or weight loss; lymphoreticular, extranodal
Non-Hodgkin Lymphoma
nodular aggregates of neoplastic cells; several B cell subtypes recapitulate normal B cell counterparts; Ex: follicular lymphoma
nodular growth pattern
diffuse growth of neoplastic cells; many B cell and all T cell subtypes
diffuse growth pattern
2nd most common type of NHL especially in the West
usually occurs > 40 years
neoplasm of follicular center B cells
t(14;18) with BCL2 aberration in 90%
nodules of small cleaved - large B cells (small and mitotically inactive)
Grade I and II = mostly small cleaved cells
Grade III = mostly large B cells
may progress to higher grade; disseminated at presentation and is slowly progressive
Follicular Lymphoma
most common type of NHL worldwide
may occur de novo or progress from follicular lymphoma
occurs at any age
proliferation of large B cells with open vesicular chromatin, nucleoli, and variable cytoplasm; numerous mitoses
many morphologic and genetic variants; in lymph nodes or extranodal; localized at presentation
aggressive if untreated, 50-60% cure rate
Diffuse Large B cell Lymphoma
low-grade lymphoma of specialized small B lymphocyte
> 40 years; extranodal, indolent course
results from chronic hyperplasia of B cells induced by autoimmune or infectious processes
H. pylori infections can cause the most common form that often responds to antibiotic therapy
Marginal Zone, MALT Lymphoma
mostly seen in children and immunosuppressed patients (HIV)
endemic in Africa, but seen worldwide
MYC abnormality in all cases usually due to t(8;14
EBV infection is associated with most endemic cases
extranodal often in the jaw and abdomen, may become leukemic
diffuse proliferation of uniform intermediate size B lymphocytes with fine chromatin, small nucleoli, and numerous mitoses mixed with starry sky cells
very aggressive if untreated, highly curable (except in HIV patients)
Burkitt Lymphoma
cutaneous T cell lymphoma; >40 years
primary skin involvement (plaques and tumors) and late lymph node and spleen involvement
epidermal and high dermal infiltrate of small CD4 T cells with cerebriform nuclei
slowly progressive
Mycosis fungoides
cutaneous T cell lymphoma; >40 years
primary skin involvement (erythroderma) and late peripheral blood, lymph node, and spleen involvement
epidermal and high dermal infiltrate of small CD4 T cells with cerebriform nuclei
clinically aggressive
Sezary syndrome
Reed-Sternberg cells and variants surrounded by benign reactive cells
genetically abnormal B cell with abnormal NF-kB signaling, increased cytokine/chemokine production, abnormal cytokine/chemokine receptors
classical types are often associated with EBV
bimodal age distribution in classical types (15-35 and >45)
male predominance except with NS type
painless lymphadenopathy w/ or w/ofever, night sweats, and/or weight loss
lymph nodes (cervical and mediastinal), spleen , liver, BM
younger patients: localized with minimal symptoms
older: disseminated with B symptoms
Hodgkin Lymphoma
Classical HL
Variable number of R-S cell variants called lacunar cells in the nodules surrounded by broad bands of collagen
Cervical and mediastinal lymph nodes are commonly involved
Tends to occur in younger patients, especially females
Usually presents with asymptomatic stage I or II disease
Nodular Sclerosis Hodgkin Lymphoma
Classical HL
Moderate number of R-S cells (classical and variants) with a mixture of benign inflammatory cells (lymphocytes, plasma cells, eosinophils, and histiocytes)
Presents with any stage and is often asymptomatic
Mixed Cellularity Hodgkin Lymphoma
Classical HL
Moderate number of R-S cells (classical and variant) with a background of benign small lymphocytes but few other inflammatory cells
Usually presents with asymptomatic stage I or II disease
Lymphocyte-rich Hodgkin Lymphoma
Classical HL
Numerous R-S cells (classical and variants) with relatively few reactive cells; often confused with large cell NHL
Usually presents with advanced stage and prominent symptoms
Lymphocyte-depleted Hodgkin Lymphoma
Nonclassical HL
R-S cell variant (popcorn cells) with numerous reactive cells (mostly small lymphocytes with some histiocytes)
Reactive small lymphocytes are mostly B cells rather than T cells
Usually presents with asymptomatic stage I or II disease; there is a propensity for late relapse (similar to low-grade B cell NHL)
Nodular Lymphocyte Predominant Hodgkin Lymphoma
single node group or extranodal site
Lymphoma Staging: Stage I
multiple node groups or nodes and extranodal sites on the same side of the diaphragm
Lymphoma Staging: Stage II
node groups on both sides of the diaphragm with possible localized extranodal site or spleen
Lymphoma Staging: Stage III
disseminated nodal and extranodal
Lymphoma Staging: Stage IV
A = no "B" symptoms B = constitutional symptoms including fever, night sweats, and/or weight loss
Lymphoma Staging: modifiers
Plasma cell disorder
neoplastic disorders in which there are solitary or multiple collections of almost mature plasma cells usually within the bone; produce monoclonal Ig
>50 years
present with monoclonal gammopathy w/ or w/o anemia, bone pain, lytic bone lesions
varies from smoldering to aggressive
free light chain/Bence Jones proteinuria (80%)
complications: hypercalcemia, bone fractures, marrow failures, renal disease, amyloidosis, infections
Plasmacytoma/Plasma Cell Myeloma
Plasma cell disorder
associated with lymphoplasmacytic lymphoma characterized by infiltration of small lymphocytes and plasmacytoid lymphocytes in bone marrow, lymph nodes, spleen, and liver
neoplastic cells secrete IgM
serum hyperviscosity, Bence Jones proteinuria (25%), no lytic bone lesions
Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma)
Plasma cell disorder
rare neoplasms in which a single clone of plasma cells or lymphocytes produces and secretes Ig heavy chains only
structurally abnormal heavy chains cannot bind light chains
Heavy Chain Disease
Plasma cell disorder
neoplastic condition in which a single clone of plasma cells secretes excess monoclonal intact Ig or free light chain that is deposited as amyploid in many organs causing organ failure
this may be a manifestation of myeloma but is more commonly in patients with minical bone plasmacytosis and no other myeloma findings
Primary Amyloidosis
Plasma cell disorder
monoclonal Ig in serum with no demonstrable evidence of plasma cell neoplasm
prevalence increases with age
usually
Monoclonal Gammopathy of Undetermined Significance (MGUS)
excess production of monoclonal Ig by a single clone of plasma cells
IgG in 50%, IgA in 20%, light chain only in 20%
detected by serum and/or urine protein electrophoresis
heavy and light chain class determined by IFE
Monoclonal Gammopathy
