Ch 13 Key Concepts Flashcards
most common type of cancer in children, may be derived from either precursor B or T cells
- highly aggressive tumors manifest with signs and sx of bone marrow failure, or as rapidly growing masses
- tumor cells contain genetic lesions that block differentiation, leading to the accumulation of immature, nonfunctional blasts
acute lymphoblastic leukemia/lymphoblastic lymphoma
most common leukemia in adults
- tumor of mature B cells that usually manifests with bone marrow and lymph node involvement
- indolent course, commonly associated with immune abnormalities, including an increased susceptibility to infection and autoimmune disorders
small lymphocytic lymphoma/chronic lymphocytic leukemia
most common indolent lymphoma in adults
- tumor cells recapitulate the growth pattern of normal germinal center B cells; most cases are associated with a (14:18) translocation that results in the over-expression of BCL2
follicular lymphoma
most common lymphoma of adults
- heterogenous group of mature B cell tumors that share a large cell morphology and aggressive clinical behavior
- rearrangements or mutations of BCL6 gene are recognized associations; one third carry a (14:18) translocation involving BCL2 and may arise from follicular lymphomas
diffuse large B cell lymphoma
very aggressive tumor of mature B cells that usually arises at extranodal sites
- strongly associated with translocations involving the MYC proto-oncogene
- tumor cells often are latently infected by EBV
burkitt lymphoma
plasma cell tumor that manifests with multiple lytic bone lesions associated with pathologic fractures and hypercalcemia
- neoplastic plasma cells suppress normal humoral immunity and secrete partial immunoglobulins that are nephrotoxic
multiple myeloma
multiple myoloma is associated with diverse translocations inolving what locus?
IgH
- leads to frequent dysregulation and over-expression of D cyclins
plasma cell neoplasm
- common in older adults, progresses to myeloma at a rate of 1% of patients per year
- M spike, but no other plasma cell neoplasm characteristics
monoclonal gammopathy of unknown significance
plasma cell neoplasm
- disseminated disease that pursues an usually indolent course
smoldering myeloma
plasma cell neoplasm
- solitary bone lesion identical to disseminated myeloma
- most progress to myeloma within 7-10 years
solitary osseous plasmacytoma
plasma cell neoplasm
- solitary mass, usually in the upper aerodigestive tract
- rarely progresses to systemic disease
extramedullary plasmacytoma
B cell lymphoma that exhibits plasmacytic differentiation
- clinical sx dominated by herviscosity related to high levels of tumor-derived IgM
- highly associated with mutations in the MYD88 gene
lymphoplasmacytic lymphoma
tumor of naive B cells that pursues a moderately aggressive course and is highly associated with translocations involving the cyclin D1 gene
mantle cell lymphoma
indolent tumors of antigen-primed B cells that arise at sites of chronic immune stimulation and often remain localized for long periods of time
marginal zone lymphoma
morphologically distinct, very indolent tumor of mature B cells that is highly associated with mutations in the BRAF serine/threonine kinase
hairy cell leukemia
peripheral NK/T cell lymphoma:
- aggressive T cell tumor, associated in a subset with activating mutations in the ALK tyrosine kinase
anaplastic large cell lymphoma
peripheral NK/T cell lymphoma:
- aggressive tumor of CD4 T cells that is uniformly associated with HTLV-1 infection
adult T cell leukemia/lymphoma
peripheral NK/T cell lymphoma:
- indolent tumor of cytotoxic T cells or NK cells that is associated with mutations in the transcription factor STAT3 and with autoimmune phenomena and cytopenias
large granular lymphocytic leukemia
peripheral NK/T cell lymphoma:
- aggressive tumor, usually derived from NK cells, that is strongly associated with EBV infection
extranodal NK/T cell lymphoma
unusual tumor consisting mostly of reactive lymphocytes, macrophages, eosinophils, plasma cells and stromal cells mixed with rare tumor giant cells called Reed-Sternberg cells
Hodgkin lymphoma
what are the two broad types of Hodgkin lymphoma?
- classical
2. lymphocyte predominant
which form of Hodgkin lymphoma is frequently associated with acquired mutations that activate transcription factor NF-kB and EBV infection?
classical
- Reed-Sternberg cells make multiple cytokines and chemokines that influence the host response, and the host response in turn makes factors that support the growth of tumor cells
lymphocyte predominant Hodgkin lymphoma expresses what type of markers?
B cell markers
- NOT associated with EBV
aggressive tumors comprised of immature myeloid lineage blasts, which replace the marrow and suppress normal hematopoiesis
- associated with diverse acquired mutations that lead to expression of abnormal transcription factors, which interfere with myeloid differentiation
- often associated with mutations in genes encoding growth factor receptor signaling pathway components or regulators of the epigenome
acute myeloid leukemias (AMLs)
tumors in which production of formed myeloid elements is initially increased, leading to high blood counts and extramedullary hematopoiesis
- commonly associated with mutations that lead to constitutive activation of tyrosine kinase, which mimic signals from normal growth factors
myeloproliferative disorders
- all can transform to acute leukemia and to a spent phase of marrow fibrosis associated with anemia, thrombocytopenia, and splenomegaly
what are the most common pathogenic kinases of myeloproliferative disorders?
- BCR-ABL (associated with CML)
- mutated JAK2 (associated with polycythemia vera and primary myelofibrosis)
poorly understood myeloid tumors characterized by disordered and ineffective hematopoiesis and dysmaturation
- recently shown to frequently harbor mutations in splicing factors and epigenetic regulators
- manifest with one of more cytopenias and progress in 10-40% of cases to AML
myelodisplastic symdromes
