mature lymphoid neoplasm Flashcards
(51 cards)
disorders in which the localization of disease
is in the
___ are
considered
lymphomas
Lymphoproliferative disorders in which the
primary site of disease is the _______ are classified as leukemias;
Lymphoproliferative disorders in which the
primary site of disease is the blood or bone
marrow are classified as leukemias;
tend to be bone
marrow based, although extramedullary
involvement may be an important part of the
clinical presentation
Plasma cell neoplasms
are recognized as B cell disorders,
although the normal counterpart of the
transformed cells remains elusive.
Hodgkin lymphoma and hairy cell leukemia
“cobblestone” or likened to a
soccer ball chromatin pattern
Chronic Lymphocytic Leukemia (CLL)
Smudged lymphocytes
cll
CLL lymphocytes exhibit__________.
B cell markers
= CD19, CD20, and CD23
T cell antigen = CD5.
abnormalities are
among the worst prognostic indicators and
foretell
resistance
to
chemoimmunotherapy approaches
Del(17p) and TP53
expressions are cell
markers that generally correlate with IGHv
mutational status but also have independent
prognostic value
CD38 and ZAP70
most powerful prognostic factor of CLL is the
immunoglobulin genes (IGHv)
Chronic Lymphocytic Leukemia (CLL)
● oral alkylating agents such as chlorambucil or
cyclophosphamide.
● nucleoside analog fludarabine and rituximab
● BTK inhibitors (ibrutinib), PI3k inhibitors
(idelalisib), and BCL2 inhibitors (venetoclax)
asymptomatic, without any lymphadenopathy,
organomegaly, cytopenias, or systemic
symptoms with less than 5x10^9/L circulating
B lymphocytes
MONOCLONAL B CELL LYMPHOCYTOSIS
(MBL) AND SMALL LYMPHOCYTIC LYMPHO
disease of the elderly, with a median age of
approximately 70 years AND AN equal
distribution between the sexes.
PROLYMPHOCYTIC LYMPHOMA (PLL)
characterized by massive splenomegaly and
marked absolute lymphocytosis, often more
than 100 10^9/L
PROLYMPHOCYTIC LYMPHOMA (PLL)
the presence of at least 5 10^9 cells/L of
circulating B lymphocytes for more than 3
months to establish the diagnosis of ____,
with confirmation of clonality performed by
flow cytometry
CLL
pll tx
● PLL has been treated with a standard
lymphoma regimen, such as CHOP
(cyclophosphamide, doxorubicin, vincristine,
and prednisone).
● The anti-CD52 monoclonal antibody alemtu
zumab has been used for the T cell subtype
of PLL
● presence of marrow fibrosis
● Pat terns of bone marrow involvement may
be interstitial or patchy
● Hairy cells are found in the peripheral blood
and bone mar row aspirate,
HAIRY CELL LEUKEMIA
rare disease characterized by an increase in
circulating LGLs in excess of 2 10^9/L
LARGE GRANULAR LYMPHOCYTIC LEUKEMIA
● may trigger a variant/atypical lymphocyte flag
or blast flag
● Most patients present with neutropenia,
anemia, or both
LARGE GRANULAR LYMPHOCYTIC LEUKEMIA
is a clonal expansion of
cytotoxic T cells, expressing CD3,
CD8, and CD57.
T cell LGL
in creased numbers of
clonal NK cells in circulation. they/re
typically negative for CD3 but do
express CD56
The other form of LGL leukemia is categorized as a chronic lymphoproliferative disorder of NK cells,
LARGE GRANULAR LYMPHOCYTIC LEUKEMIA
● Myeloid growth factors
● immunosuppressive agents, such as low
doses of methotrexate, cyclophosphamide,
or cyclosporin A,
post-thymic neo plastic disorder of T cells
associated with retroviral infection by the
human T lymphotropic virus type 1 (HTLV-1)
ADULT T CELL LEUKEMIA/LYMPHOMA
● latency period of 55 to 70 years.
ADULT T CELL LEUKEMIA/LYMPHOMA
