Mature B cell lymphomas

Question 1

CLL/SLL

Answer 1

• CD19/CD5/CD23
• Dim: CD20 and surface light chain

Richter syndrome (diffuse large cell)

Question 2

most common NHL

2nd most

Answer 2

DLBCL

Follicular

Question 3

most common leukemia of adults in western world

Answer 3

CLL

Question 4

Follicular lymphoma

Answer 4

CD19/CD20/CD10
Bright surface light chain restricted
bcl2+ (in GC where normally shut off)
bcl6+ (indicates cells have passed through GC stage of maturation)
t(14;18)
IgH (14) and BCL2 (18) 
bcl2: anti-apoptotic

Question 5

Mantle Cell lymphoma

Answer 5

CD19/CD20/CD5
Bright CD20 and surface light chain
often CD23 negative (found in CLL/SLL)

t(11;14)
cyclin D1 (11) with IgH (14)
Cyclin D1: promotes progression of cell cycle from G1 to S

Detection: FISH, PCR, immunohistochemistry

Lymphomatoid polyposis = GI involvement

Question 6

Marginal zone lymphoma

Answer 6

polyclonal → oligoclonal → monoclonal theory : reactive T-cell dependent for growth/survival
acquisition of t(11;18) or t(1;14) : independent of extrinsic stimuli. No response to antibiotics
Bcl10
MALT1

Question 7

Lymphoplasmacytic lymphoma (LPL)

Answer 7

secrete monoclonal IgM (>3gm/dL)
hyperviscosity syndrome: Waldenstorm macroglobulinemia

nvolves BM
+/- spleen, nodes
high viscosity causes: visual sx (blurried vision), cns sx, bleeding (interference with coag factors and platelets), autoimmune hemolytic anemia (cold agglutinins - IgM), cryoglobulinemia - precipitation of IgM at low temps (fingers/toes)

MYD88

Question 8

HCL

Answer 8

older male
splenomegaly
pancytopenia 
BRAF mutation 
involves blood, BM, splenic red pulp 

diffuse “fried eggs” in BM
round to reniform nuclei (kidney beaned nuclei) with moderate amnt of pale blue cytoplasm w/ “hairs”
reticulin fibrosis - “dry tap” of BM
splenic red pulp w/ obliteration of white pulp

CD19/CD20/surface light chain restriction
CD11c/CD25/CD103

different type of treatment from other NHL
purine analogs → long remission
BRAF inhibitors

Question 9

Multiple myeloma

Answer 9

anemia → weakness, fatigue
decreased Ig → recurrent infection
hypercalcemia → polyuria, constipation, confusion
tumor load → bone pain, pathologic fractures
Bence jones protein → renal insufficiency
light chain deposition in organs → amyloidosis

One class will be elevated, rest will be decreased: IgG > IgA&raquo_space;»> IgM, IgE, IgD

PB rouleux (inc Ig in serum → RBC adhesion)
PB: excess Ig in serum → acidic, so takes up blue (basophilic) stain
PB plasma cell leukemia = end stage 

translocations with IgH
deletions of 17p(TP53)
rearrangements of MYC
IL-6 - GF produced
tumors also influence activation of osteoclasts and inhibition of osteoblasts (no repair of bone → inc serum calcium

Question 10

most common cause of monoclonal gammopathy

Answer 10

MGUS

Question 11

Plasmacytoma

Answer 11

Solitary bone/soft tissue clonal plasma cell mass w/ no evidence of BM or organ disease - spine, lung and oronasopharynx
local
can progress to myeloma

Question 12

Diffuse large B-cell lymphoma

Answer 12

High grade
heterogenous
lack specific risk factor
immunodeficient 
EBV
MOST COMMON NHL

Rapidly enlarging - single nodal/extranodal mass
“B-type” symptoms
BM - minority of ptns - late in disease

mitotically active

nondescript

bcl6 mutation (3q27) - cell has been through GC maturation
bcl2 mutation - t(14;18), transformed follicular lymphoma
cMYC mutation/translocation
Double hit lymphoma - translocation of both bcl2 and myc - aggressive, poor prognosis

Question 13

Burkitt Lymphoma

Answer 13

High grade - HIGHEST
Extranodal
30% childhood NH
Tumor lysis syndrome - uric acid, k+, calcium
“starry sky”
basophilic cytoplasm with vacuoles 
CNS sx
highly mitotic

CD19/CD20/CD10
surface light chain restriction
bcl6

grows fast - glycolysis 
translocation involving c-MYC(8q24)
t(8;14)
t(2;8)
t(8;22)

Question 14

Burkitt Lymphoma

Endemic vs sporadic

Answer 14

endemic EBV: precedes neoplasm. Mandibular mass, abdominal viscera
sporadic: abdominal mass in ileocecum and peritoneum