WBC Disorders V Flashcards
BCL6 dysregulation
diffuse large B cell lymphoma
most common form of NHL in US
DLBCL
t(14;18)
DLBCL
same as follicular
EBV and KSHV/HHV-8
associated with DLBCL
EBV and DLBCL
severe T cell deficiency
with DLBCL
KSHV/HHV8
primary effusion in malignant pleural effusion
with DLBCL
DLBCL demo
adults 60yo
also children and young adults
DLBCL prognosis
aggressive
-rapidly fatal without tx
chemo - good prognosis
rapidly growing mass at waldeyers ring
DLBCL
two classes of DLBCL
BCL6 and BCL2 rearrangement
BCL2 - though to be morph of follicular lymphoma
starry sky pattern
burkitt lymphoma
categories of burkitt lymphoma
african endemic
sporadic non-endemic
HIV
C-myc oncogenes
burkitt lymphoma
translocation of C-myc on chromosome 8
ALL burkitt lymphomas
mandibular burkitt
african - endemic
GI burkitt
sporadic - non-endemic
aggressive burkitt
HIV
fastest growing human tumor
burkitt
EBV and burkitt
all endemic
25% HIV
15-20% sporadic
warburg effect
in burkitt
MYC oncogene
NO BCL2
burkitt
burkitt demo
adolescents or young adults with extranodal masses
most common and deadly plasma cell dyscrasia
multiple myeloma
t(8;14)
translocation involving MYC gene - burkitt lymphoma
rouleaux formation
multiple myeloma
due to high M proteins
path fractures, hypercalcemia, renal failure, immune abnormalities
multiple myeloma
flame cells, mott cells, russel bodies (cyto inclusion), and dutcher bodies (nuclear inclusions)
multiple myeloma
bence joines proteins
light chains in urine
toxic to renal tubular cells
can lead to myeloma kidney
most common multiple myeloma
monoclonal IgG-kappa
CD138, syndecan 1, CD56
multiple myeloma
definitive diagnosis of multiple myeloma
bone marrow examination
most common plasma cell dyscrasia
MGUS
dx of MM
> 3g/dL of monoclonal Igs in serum
single mass in bone or soft tissue
solitary myeloma (plasmacytoma)
uncommon
lack of sx and high plasma M component
smoldering myeloma
multiple myeloma demo
men, african, 65-70yo
most myelomas
> 3g serum Ig
and/or >6g bence jones proteinuria
solitary plasmacytoma
solitary lesion
progress to MM 10-20 yrs
extraosseous - cure if resection
-often upper respiratory
smoldering myeloma
asymptomatic with M protein >3g serum
prgress to MM 15 yrs
middle ground of MM and MGUS
multiple myeloma prognosis
survival 4-7 yrs
untreated 6-12 months
cyclin D1 - good prognosis
deletion 13, 17, t(4;14) - bad prognosis
confusion, lethargy, constipation, renal dysfunction, bacterial infections
multiple myeloma
CNS - from hypercalcemia
monoclonal gammopathy of uncertain significance
asymptomatic and serum Ig < 3g/dL
early stage of multiple myeloma development
**should periodically assess patient for serum M component and bence-jones proteins in urine
lymphoplasmacytic lymphoma
B cell neoplasm
60-70yo
fraction to plasma cells
secretes IgM - high levels
-lead to hyperviscosity - waldenstrom
waldenstrom macroglobulinemia
hyperviscosity due to large IgM release
with lymphoplasmacytic lymphoma
hyperviscosity leads to
visual impairment - venous congestion
neuro problems - HA, dizzy, deaf - sluggish blood flow
bleeding
cryoglobulinemia - precipitate at low T - raynauds, urticaria
lymphoplasmacytic lymphoma prognosis
incurable
- IgM alleviated with plasmapharesis
- also temp tx is anti-CD20 Ab
survival 4 years
overexpression of cyclin D1
mantle cell lymphoma
t(11;14)
mantle cell lymphoma
-IgH on 14 and cyclin D on 11
overexpression of cyclin D1
-more G1 to S transition
fluorescence in situ hybridization
painless lymphadenopathy, spleen and gut sx
mantle cell lymphoma
mantle cell lymphoma prognosis
incurable - survival 3-4 yrs
organ dysfunction caused by tumor infiltration
HSC transplant - some promise
lymphomatoid polyposis
mantle cell lymphoma
cyclin D1, CD19, CD20, CD5, no CD23
mantle cell lymphoma