Sloka WBC malignancies Flashcards
Neoplastic proliferations etiology
Mutations in Stem cells/Block in differentiation/Maturation
Lymphoid - B, T cells, NK cells
Myeloid - Hematopoietic cells
Histiocytoses - Macrophages/Dendritic cells
Lymphoid Neoplasms
Acute lymphoblastic leukemia/lymphoma (ALL)
Chronic Lymphocytic leukemia/lymphoma (CLL)
- Adult T cell leukemia
-Hairy cell leukemia
Plasma cell neoplasms
Hodgkin lymphoma/ Non-Hodgkin lymphoma
HL characteristic presentation & predictable spread pattern
Lymphoid neoplasms Pathogenesis risk factors
(Most) Acquired mutations, Inherited mutations
Chromosomal TRANSLOCATIONS (MC in leukemia/lymphoma)
Chronic inflammation (EBV)
Ionizing radiation
Chemicals (benzene)
Viruses (EBV)
smoking
Lymphoid neoplasms Pathogenesis
Pro-growth mutation in pleuripotent cell
Goes toward Warburg metabolism (anaerobic)
Pro-survival mutation(BCL2)
Increased self-renewal (IL-2?)
WHO classification of Lymphoid Neoplasms
- Precursor B-cell neoplasms (immature B-cells)
- Peripheral B-cell neoplasms (mature B-cells)
- Precursor T-cell neoplasms (immature T-cells)
- Peripheral T-cell and NK-cell neoplasms (mature T cells and NK cells)
- Hodgkin lymphoma (Reed-Sternberg cells and variants)
General Principles of lymphoid Neoplasms
- May be suspected from clinical features but biopsy is required
- Antigen receptor rearrangement generally precedes transformation
- Most resemble some recognizable stage of B or T-cell differentiation
- Often associated with immune abnormalities
- Tend to recapitulate behavior of their normal counterparts
- Hodgkin lymphoma spreads in a predictable fashion
pre-B Lymphoblast
Acute
Precursor B lymphoblastic lymphoma/leukemias
Naive B cell
Small lymphocytic lymphoma
Chronic lymphocytic leukemia
Plasma cell
Multiple myeloma
Mantle zone LN
Mantle cell lymphoma
Germinal center LN
Follicular lymphoma
Burkitt lymphoma
Diffuse large B cell lymphoma
Hodgkin’s lymphoma
Marginal zone
Diffuse large B cell lymphoma
Small lymphocytic lymphoma
Chronic lymphocytic leukemia
Immature Thymus
Precursor T lymphoblastic lymphoma/leukemias
T cell neoplasm in LN
Peripheral T cell lymphoma
CD1
Thymocytes Langerhans cells Histiocytes - Macrophages Dendritic cells
CD3
Thymocytes
Mature T cells (forms part of receptor)
CD4
Helper T cells
CD 8
Cytotoxic T cells
some NK cells
CD10
Pre-B cells
Germinal center B cells
(lymphoblasts)
CD 19
Pre-B cells
Mature B cells
NOT plasma cells
Antibodies made in what type of neoplasms
Plasmacytoid?
CLL
Macroglobinemia
CD20
Pre-B cells after CD19
Mature B cells
NOT plasma cells
CD21
EBV receptor
Mature B cells
Follicular dendritic cells
CD14
Monocytes
CD15
Granulocytes
Reed-Sternberg cells
Hodgkin’s Lymphoma
CD30
Reed-Sternberg cells
Hodgkin’s lymphoma
activated B, T cells, and monocytes
CD16
NK cells
granulocytes
CD56
NK cells
subset of T cells
B-cell acute lymphoblastic leukemia/lymphoma
Bone marrow precursor B cell
t(12:21) RUNX1 and ETV6 - 25%
3 y.o. children
Aggressive
T-cell acute lymphoblastic leukemia
Precursor T cell (thymus mc)
translocations
-NOTCH1?
Adolescent Males
Aggressive - difficult to tx
ALL epidemiology
MC (B>T) cancer and leukemia in children
-
Early Pre-B cell ALL
Common ALL ag: CD10*
TdT+
t(12:21), hyperdiploidy
-Prognosis
95% remission
75-85% cures
Early Pre-B cell ALL Laboratory & sx
WBC - (lymphoids^)
NN anemia*
Thromocytopenia*
Lymphoblasts - Peripheral &/or BM
Aching sternum
Pre-T cell ALL
CD10 neg
TdT+*
Anterior medistinal mass/acute leukemia
Adolescent males
Usually lymphoma only
NN anemia*
Thromocytopenia*
Small Lymphocytic lymphoma/ CLL
MC naive B cell
some memory B cells
Olderadults w/ BM, LN, spleen, and liver disease
Minority: autoimmune hemolysis & thrombocytopenia
CLL
MC leukemia 15000 - adults
>60 y.o.
MC generalized LAD
Virgin B-cell* maturation
MC leukemia
CLL
MC generalized LAD
CLL
CLL Laboratory
WBC count - depends when found -5000 dx -2000 early Smudge cells* NN anemia 50% Thrombocytopenia 40% Hypogammaglobulinemia - may have Ig spike Ab for hemolysis BM -
Bilateral axillary LAD suggests
CLL
Burkitt lymphoma book chart
Germinal center B-cell
t(8:14) MYC, IgG
-EBV associated
Adolescents or young adults w/ extranodal masses
-rare leukemia presentation
t(8:14)
Burkitt lymphoma
MYC + Ig
Diffuse large B-cells lymphoma book chart
Germinal center or postgerm B cell
All ages
- older adults
Rapidly growing mass
Aggressive
HIV brain, EBV …
Burkitt’s lymphoma variants
African - jaw
North American - abdominal mass
30% of all child NHL in US (3-7 y.o.)
Burkitt lymphoma
-B-cell non-Hodgkins lymphoma