Hematological malignancies Flashcards
definition: leukemia
cancer that starts in blood forming tissue such as bone marrow and causes large number of abnormal blood cells to be produced and enter the blood
definition: lymphoma and myeloma
cancers that begin in the cells of the immune system
myeloid / myelogenous / myeloproliferative cancer lines involve which cell types?
granulocytes, RBCs, platelets, or their progenitors
lymphoid, lymphocytic, lymphoblastic, lymphoproliferative cancer lines involve which cell types?
B cells, T cells, NK cells, or their precursors
what are the two types of acute leukemia?
- acute myeloblastic leukemia (AML)
2. acute lymphoblastic leukemia / lymphoma (ALL)
AML and ALL symptoms are often caused by what condition in the bone marrow?
pancytopenia - tumor cells displace or suppress normal blood elements in the bone marrow
what is the main general feature of AML?
undifferentiated myeloid blast accumulating in bone marrow
what are the two types of ALL? what are their frequencies among total ALL cases?
- B cell type - 85%
2. T cell type - 15%
what is a major concern for myeloproliferative neoplasms / disorders?
may transition into an acute leukemia later on
what is the main pathology associated with polycythemia vera? what is the cause?
- high levels of functional RBCs
2. JAK2 mutation
what is the main pathology associated with essential thrombocythemia? what is the cause?
- overproduction of megakaryocytes
2. JAK2 or MPL mutations
what is the main pathology associated with primary myelofibrosis? what is the cause?
- fibrosis and atypical megakaryocytes
2. JAK2 or MPL mutations
what is the pathogenesis of CML? how does it present?
- BCR-ABL fusion protein from Philadelphia chromosome leads to constitutively active kinase signaling mediating hematopoietic growth
- overgrowth of granulocytic and megakaryocytic precursors in bone marrow
what determines the classification of lymphoma as Hodgkin’s or non-Hodgkin’s?
presence of Reed-Sternberg cell
RS cell - Hodgkin’s lymphoma
what is a Reed-Sternberg cell?
large cell with two or more nuclei or nuclear lobes, each of which contains a large eosinophilic nucleolus
what are the three classifications of non-Hodgkin’s lymphoma?
- indolent
- aggressive
- very aggressive
what type of cancer is characterized by the presence of smudge cells?
chronic lymphocytic luekemia
multiple myelomas are composed of what cell type?
mature B cells (plasma cells)
multiple myeloma comes from what cell origin?
lymphoid origin!!
what will multiple myeloma show on PBS?
Rouleaux formation
what are the features of solitary plasmacytoma?
- solitary mass
- clonal plasma cells
- low or no Ig in serum
what is primary amyloidosis?
clonal expansion of plasma cells in the bone marrow that secrete monoclonal light chains that are misfolded and form fibril deposits in tissue
what is the main pathology associated with primary amyloidosis?
organ problems due to amyloid fibril deposits
what is monoclonal gammopathy of uncertain significance?
- detection of monoclonal Ig in serum or urine without evidence for malignancy of the plasma or B cells
- risk of progression to multiple myeloma
what is POEMS syndrome?
plasma cell disorder characterized by
Polyneuropathy Organomegaly Endocrinopathy Monoclonal protein Skin changes
what is Waldenstrom’s macroglobulinemia / lymphoblastic lymphoma?
- IgM secreting
2. associated with hyperviscosity syndrome
leukemias are diagnosed by which tests?
PBS and/or bone marrow biopsy
lymphomas are diagnosed by which tests?
lymph node biopsy
plasma cell neoplasms are diagnosed by which tests?
bone marrow biopsy
an Auer rod is pathognomonic for for what cancer?
acute myelogenous leukemia (AML)
what is the pathognomonic structure seen in AML?
Auer rod
what technique is used to detect the Philadelphia chromosome translocation event for CML?
cytogenetics - karyotype or FISH
what technique is used to detect the serum IgGk in someone with amyloidosis?
electrophoresis
lytic bone lesions are characteristic of what type of cancer?
multiple myeloma
what is a kaplan-meier plot?
compares experimental treatment to control with respect to patient survival % during a phase III trial
what is the goal of induction therapy?
remission
what is the goal of consolidation therapy?
eliminate undetectable cancer cells
what is the goal of maintenance therapy?
maintain remission and reduce risk of relapse
