Lymphomas and Myelomas

Question 1

affects all ages; male predominance; insidious onset; painless lymphadenopathy; w/ or w/o fever, night sweats, and/or weight loss; lymphoreticular, extranodal

Answer 1

Non-Hodgkin Lymphoma

Question 2

nodular aggregates of neoplastic cells; several B cell subtypes recapitulate normal B cell counterparts; Ex: follicular lymphoma

Answer 2

nodular growth pattern

Question 3

diffuse growth of neoplastic cells; many B cell and all T cell subtypes

Answer 3

diffuse growth pattern

Question 4

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

Answer 4

Follicular Lymphoma

Question 5

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

Answer 5

Diffuse Large B cell Lymphoma

Question 6

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

Answer 6

Marginal Zone, MALT Lymphoma

Question 7

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)

Answer 7

Burkitt Lymphoma

Question 8

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

Answer 8

Mycosis fungoides

Question 9

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

Answer 9

Sezary syndrome

Question 10

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

Answer 10

Hodgkin Lymphoma

Question 11

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

Answer 11

Nodular Sclerosis Hodgkin Lymphoma

Question 12

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

Answer 12

Mixed Cellularity Hodgkin Lymphoma

Question 13

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

Answer 13

Lymphocyte-rich Hodgkin Lymphoma

Question 14

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

Answer 14

Lymphocyte-depleted Hodgkin Lymphoma

Question 15

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)

Answer 15

Nodular Lymphocyte Predominant Hodgkin Lymphoma

Question 16

single node group or extranodal site

Answer 16

Lymphoma Staging: Stage I

Question 17

multiple node groups or nodes and extranodal sites on the same side of the diaphragm

Answer 17

Lymphoma Staging: Stage II

Question 18

node groups on both sides of the diaphragm with possible localized extranodal site or spleen

Answer 18

Lymphoma Staging: Stage III

Question 19

disseminated nodal and extranodal

Answer 19

Lymphoma Staging: Stage IV

Question 20

A = no "B" symptoms
B = constitutional symptoms including fever, night sweats, and/or weight loss

Answer 20

Lymphoma Staging: modifiers

Question 21

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

Answer 21

Plasmacytoma/Plasma Cell Myeloma

Question 22

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

Answer 22

Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma)

Question 23

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

Answer 23

Heavy Chain Disease

Question 24

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

Answer 24

Primary Amyloidosis

Question 25

Plasma cell disorder
monoclonal Ig in serum with no demonstrable evidence of plasma cell neoplasm
prevalence increases with age
usually

Answer 25

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Question 26

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

Answer 26

Monoclonal Gammopathy