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218 Cards in this Set
- Front
- Back
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what are the 3 characteristics of hematologic neoplasms?
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1. lineage (myeloid vs. lymphoid), 2. survival (aggressiveness - acute vs. chronic), 3. predominant site of involvement (blood and marrow vs. tissues)
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what type of cells are predominant in acute neoplasms?
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immature cells - blasts or very early precursors
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what type of cells are predominant in chronic neoplasms?
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mature cells
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what is the predominant site of involvement in leukemias?
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blood and bone marrow
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what is the predominant site of involvement in lymphomas?
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lymphoid tissue
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what is the predominant site of involvement in granulocytic sarcoma?
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myeloid tissue
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what is the predominant site of involvement in plasmacytoma or myeloma?
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plasma cells - tissue
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what are the 4 chronic myeloproliferative neoplasms?
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1. chronic myeloid leukemia (CML), 2. PV, 3. essential thrombocytemia, 4. primary myelofibrosis
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what are the characteristics of myelodysplastic syndromes?
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cytopenias, dysplasia (abnormal cell appearance), impaired funciton; tendency to transform to acute leukemia
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define NHL
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neoplastic, clonal proliferation of lymphcytes; cells may retain properties of normal cells - produce Ig or cytokines; have systemic circulation or selective circulation
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what is the demographics of NHL?
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males, 60-70s, whites > blacks
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what are the characteristics of myeloproliferative disorders?
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clonal stem cell disorders which retain the capacity to differentiate, all lineages can be involed but a single one usually dominates in a pt
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define myelodysplastic syndrome
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stem cell disorders characterized by abnormal and inefficient maturation and differentaion
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what are the predisposing factors of NHL?
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chemicals - pesticides, fertilizers, 'agent orange'; ionizing radiation; viruses (HTLV-1, EBV, HCV); H.pylori; immunosuppression, autoimmune disease
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what type of cytogenic alterations are found in NHL?
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usualy reciprocal translocations - with proto-oncogenes and ither Ig genes or TCR genes
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what is the most common locus involved in HNLs?
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14q - Ig heavy chain gene
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t(8;14)
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NHL: Burkitt lymphoma - c-MYC/IgH
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t(14;18)
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NHL: follicular and diffuse large cell lymphoma - IgH/BCL-2
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t(11;14)
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NHL: mantle cell lymphoma - Cyclin D1/IgH
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what is the function of 8q24?
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MYC oncogene - drives cell proliferation
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what is the function of 14q32
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Ig heavy chain gene (IgH)
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what is the function of 18q21?
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bcl-2: prevents cell death due to apoptosis
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what is the funciton of 11q?
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gene for cyclin D1 - involvedin regulation of the cell cycle
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what is the most common presentation of HNL?
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lymphadenopathy
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what hematologic diseases are associated with NHL?
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lymphocytosis, leukopenia, anemia, thrombocytopenia
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what are the advantages and disadvantes of WF classification
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pro: simple, clincially useful; con: ignores phenotpe, cytogenetics and molecular tests
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what are the 3 prognostic groups of WF classification?
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1. low grade - survival > 5 years; 2. intermediate - survival 2-3 years; 3. high grade - survival<1.5 years
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what are the histology characteristics that are used in the WHO classification?
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architecture: follicular vs. diffuse and cell size: small vs. large
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what type of NHL histology is more aggressive?
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diffuse architecture with large cell size have a more aggressive clincial behavior
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what is the % of HNL made up by histological grade?
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50% made up of intermediate grade (diffuse large B-cell); 25% low grade (follicular lymphomas), true high grade lymphomas (burkitt and lymphoblastic lymphomas) are rare
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what are the clinical characteristics of indolent NHL?
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majority diseminated, predominantly nodal +/- liver, spleen and BM, long median survival, relentless relapse, incurable; can transform to high grade
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what are the clinical characteristics of aggressive NHL?
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may be localized at diagnosis, extranodal not common, short median survival if not treated, long disease free-survival possible, significant population curable
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which indolent NHLs most often transform into aggressive ones?
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follicular lymphomas and small lymphocytic lymphomas
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what is the most common NHL?
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diffuse large B-cell lymphoma - aggressive
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what factors aid in prognosis of diffuse large B-cell lymphoma?
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germinal center B-cell type - better prognosis, activated B-cell type - worse prognosis
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what is the cure rate of diffuse large B-cell lymphoma?
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40% with combo chemo
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what is the second most common NHL?
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follicular lymphoma
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what is the most common indolent lymphoma?
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follicular lymphoma
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what is the histology like of follicular lymphomas?
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the follicles are uniform in size and shape and uniformly distributed throughout the node; reactive - are usually different size and shape and not uniformily distributed
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what are the grades of follicular lymphomas?
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grade 1: fewest large cells; grade 2 and grade 3: most large cells (treated as aggressive)
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what is the most common extranodal lymphoma?
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MALT (marginal zone) lymphoma
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where do marginal zone lymphomas occur?
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50% in GI - mostly stomach
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what is the behavior of marginal zone lymphomas?
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indolent - POTENTIALLY CURABLE if they are localized
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what type of cells make up marginal zone lymphomas
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B-cells
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what is a predisposing factor for gastric MALT lymphomas?
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H. pylori - lymphoma goes away if H.pylori is eradicated
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what is small lymphocytic lymphoma?
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Idental to CLL - but lack lymphocytosis; low grade, long medial survival; 3-5 % evolve into large cell lymphoma
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histology: complete effacement of normal lymph node architecture by a diffuse proliferal of small lymphocytes with some clusters of slightly larger cells
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SLL
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what is unique about mantle cell lymphomas
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one of the worst lymphomas: short survival time and incurable
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what are the demographics of mantle cell lymphomas?
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older men; 5-10% of all lymphoma; advanced stage at diagnosis
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what lymphoma has the fastest doubling time?
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Burkitt lymphoma; very common HIV associated lymphoma
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what is associated with Burkitt lymphoma?
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EBV - in the african variant; presents with huge jaw mass +/- abdmominal disease
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which lymphoma presents as an intra-abdominal disease?
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Burkitt lymphoma - often ileo-cecal area (mimics appendicits)
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which lymphoma has a starry sky histology with cattered large histiocytes with clear cytoplasms that are phagocytizing the neoplastic cells
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burkitt lymphoma
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what is the clinical behavior of T-cell lymphomas?
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aggressive; EXCEPT - mycosis fungoides, a low grade cutaneous lymphoma with a long survival rate
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what are the characteristics of T-cell lymphomas at diagnosis?
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often disseminated, associated with systemic symptoms and extranodal involvement
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what is the most common type of T-cell lymphoma?
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peripheral T-cell lymphoma, unspecified (50%)
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what Ann Arbor stage has single site involvement?
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Stage 1
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what Ann Arbor stage has more than one site but on the same side of the diaphragm?
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stage 2
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what Ann Arbor stage has diseae on both sides of the diaphragm?
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Stage 3
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what Ann Arbor stage has disseminated extranodal disease +/- nodal disease?
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stage 4
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what sites of involvement always indicate stage 4 on the Ann Arbor staging scale?
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liver and bone marrow
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what does "A" denote in the Ann Arbor staging system?
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absence of systemic symptoms
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what does "B" denote in the Ann Arbor staging system?
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presence of systemic symtoms
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what is very important in classifying T-cell lymphomas?
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site of involvment is key
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how are low grade NHL treated?
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palliatively
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how are high grade NHLs treated?
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aggressively with combo chemo with intent to cure
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what is a common regimen for treating high grade NHLs?
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CHOP- often combined with Rituxan (anti-CD20 mab)
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what are the 6 clinical prognostic factors for NHL?
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histology,
age (older, worse), stage (localzied, better), LDH (elevated, worse), perfoamce status of pt, number of extranodal site |
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what types of NHL are associated with HIV?
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aggressive types - Burkitt, large cell immunoblastic, diffuse large b-cell lymphoma; lymphoma of the CNS is common
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which type of cell is more commonly involved in lymphoma associated with HIV?
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B-cell; NHL > HL; frequently with extranodal cites; EBV associated - MYC rearrangments
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what is a predisposing factor for NHL associacated with HIV
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increasing incidence with lower CD4 counts
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what are the characteristics of NHL in transplant pts?
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often high stage, extranodal sites, aggressive, B-cell> T-cell, EBV involved
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what organ transplants are more associated with NHL?
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heart and lung > heart or liver> kidney
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which transplant associated NHL responds well to treatment?
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polymorphic B-cell hyperplasia may resopnd to decreased immunosuppression
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which transplant associated NHL does not respond well to treatment?
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malignant lymphoma
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when is a fine needle aspirate cytology a good option?
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deep-seated or difficult to reach sites; that are non-lymphoid malignancies; it is non-invasion; can be combined withflow cytometry; NOT good for HL
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what are the benefits of a excisional biopsy?
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allows good histology with plenty of tissue, but it is invasive, must have OR, difficult for deep seated sites, and difficult if pt is really sick
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how should you handle a lymph node biospy?
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lymph node should be intact, given to pathology immediately, fresh- do not put in formalin or other fixatives
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what characterizes HL?
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RS cells (binucleated with chromatin clearing and alrge eosinophilic neucloi that look like owl's eyes) ina background of benign lymphcytes and other cells (eosinophils, plasma cells and histocytes)
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HL vs. NHL: spread
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HL- orderly, contiguous; NHL - disseminated, non-contiguous
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HL vs. NHL: involvment site
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HL - central and axial nodes; NHL - both central and peripheral nodes
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HL vs. NHL: mesenteric nodes and Waldeye'rs ring involvment:
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HL - seldom; NHL - often
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HL vs. NHL: extranodal presentation?
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HL- rare; NHL - not uncommon
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what are the demographics of HL?
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bimodal distribution - 20's (either sex) and >60s; incidence increases with educational level
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what type of HL is most common in pts in their 20s?
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nodular sclerosis
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what type of HL is most common in pts >60?
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mixed cellularity type - more aggressive
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what are the predisposing factors to HL?
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1. genetics, 2. EBV, 3. immunosuprression (May be a cause or may be a consequence)
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what is the cell of origin for nodular lymphocyte predominant subtype of HL?
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B- lymphocytes - may be cause of other HLs
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what are the 4 types of HL in the Rye classification?
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1. lymphocytic predmoniant, 2. nodular sclerosis, 3. mixed cellularity, 4. lyphocytic depletion
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what is different about the WHO classification of HL vs. Rye?
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who separaed nodular lymphocyte predmoniant HL from others which they call classical HL: nodular sclerosis, mixed cellularity, lymphocyte rich and lymphocyte deplete
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what are the characteristics of nodular lymphocyte predmoninant HL?
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Male, young, often localized - cervial, axillary or inguinal nodes; 5% of HLs
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what is the course of nodular lymphocyte predominant HL?
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indolent, long survival; may relapse late; may transform to large cell NHL
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rare RS cells, predominance of background cells in a lymph node that is divided into nodules
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nodular lymphocyte predominant HL
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what is the HL where male = female?
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nodular sclerosis
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what location is commonly involved in nodular sclerosis HL?
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mediastinal, cervial or supraclavicular (above the diaphargm) usually localized and stage II at diagnosis
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bands of collagen dividing lymph node into nodules, with a fibrosis capsule, and lacunar RS cells
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nodular sclerosis
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what is the second most common HL?
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mixed cellularity type - more aggressive
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histology: more RS cells, fewer benign cells, background can have fibrosis
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mixed cellularity type - more aggressive
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histology: rare RS cells, predominantly lymphocytes and other benign cells
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lymphocyte-rich HL
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what is the presentation of lymphocyte rich HL?
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older, men>women, low stage at diagnosis, indolent course, prolonged survival
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what are the characteristics of lymphocyte-deplete HL?
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rare - many that were caleed LDHL are now called NHL; older, males, advanced stage and aggressive course
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what are the 2 variants of lymphocyte-deplete HL?
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1. abundnat RS with paucity of benign cells and 2. diffuse fibrosis with rare RS cells
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what does pathologic staging require?
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staging laparotomy - biospy of multiple lymph node groups, splenectomy and multiple liver biospies; used to decide between radiation alone or with chome
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what are the prognostic factors for HL?
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worse if:
>45 yoa, bulky mediastinal diseaes, systemic symptoms, invovlement of bone marrow, liver, pleura and/or multiple extranodal sites; incrased LDH, beta2-microgolbulin and ESR |
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when is radiation used in HL therapy?
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as adjuvant for bulky disease
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what are the 4 types of CLL?
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B-cell chronic lymphocytic leukemia (CLL), hairy cell, prolymphocytic leukemias
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what is the most common adult leukemia in the US?
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CLL
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what is the demographics of CLL?
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older, men, more common in agricultural and asbestos works, some genetics
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what is the most common cause of chronic lymphocytosis in audlts?
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CLL
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which neoplasms consists of monoclonal lymphyctosis of small, mature appearing B-cell
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CLL
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what are the 2 variants of CLL?
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pre-germinal center: "unmutated IgH - more aggressive; and post-germinal center - mutate IgH - less aggressive
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what is the presentation of CLL?
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frequently asymptomatic - unexpected findings on CBC; lymphadenopahty common, can have hepatomegaly
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how do you diagnose CLL?
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lymphocytosis > 5000/uL, characteristic immunophenotype with small mature appearing lymphocytes and smudge cells
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smudge cells are common in what?
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CLL
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what is the immunphenotype of CLL?
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1. DIM surface monoclonal Ig expressed, b-cell markers and CD5
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CD5 marker on B-cell lymphoma
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CLL
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what predicts aggressive CLL?
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1. lymphocyte doubling time <12 months,
2. diffuse pattern of marrow infiltration, 3. presence of cytogenetic abnormaliteis, 4. bone marrow compromise, 5. unmutated IgH, 6. CD38 expression, 7. ZAP-70 expression |
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what are the 2 staging systems used for CLL?
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Rai and Binet - presence of bone marrow involvment puts you in high risk
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what is the clinical course of CLL?
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intermediate - 1 yr survival, 10% very indolent
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what is the most frequent cause of death in CLL?
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infection - most commonly respiratory tract (S. pneumonia, H. influenzae, S. aureas, pseudomonas, gram (+) enterococccie
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what are the complications of CLL?
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infection, anemia and thrombocytopenia, autoimmune phenomena - Abs agaisnt RBC, +DAT, mass effects, transformation into aggressive disease
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what are infections in CLL due to?
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hypogammaglobinulinemia
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what is Richter's syndrome?
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transformation of CLL to aggressive disease - 3-5% transform to large cell NHL
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what is the DOC for CLL?
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fludarabine - nucleoside analogue, can lead to opportunisitic infections
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what are indications for treatment of CLL?
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lymphocytosis is NOT an indication. Indications: 1. rapid lympohcyte doubling time, 2. bulky lymphadenoapthy, 3. bone marrow compromise, 4. autoimmune phenomena
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what is hairy cell leukemia?
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variant of CLL - uncommon; older adults 50s, men
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what are the characteristics of hairy cell leukemia?
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cytopenias, splenomegaly - often massive, distinictive "hairy" lymphocytes in blood
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TRAP stain
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hairy cell leukemia
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bone marrow: lots of fibrosis with even spaced lymphoid cells surrounded by clear halo = fried egg cells
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hairy cell leukemia
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what is unusual about hairy cell leukemia and the spleen?
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it involves the red pulp - most other involve the white pulp
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splenomegaly - discomfort and early satiety and recurrent pyogenic infections
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hairy cell leukemia
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how do you treat hairy cell leukemia?
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cladribine: nucleoside analogue, single dose causes durable complete resonses in many
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lymphocytosis of T-cells with multilobated or convoluted nuclei
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Adult T-cell leukemia/lymphoma
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what neoplasm is associated with HTLV-1
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Adult T-cell leukemia/lymphoma
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what is the most common lymphoid maligancy in blacks?
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multiple myeloma
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plasma cells in bone marrow, bone lesions, monoclonal Ig or Ig fragments in serume or urine
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multiple myeloma
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what are the predisposing factors of multiple myeloma?
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ionizaing radiation, increased in farms, petroleum, wood, leather and asbsetos industries
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what is the most common cause of death in multiple myeloma?
|
infections - pneumonitis and pyelomehpritis are common
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what is the cause of bone lesions in multiple myeloma?
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osteoclast activiating factors produced by plasma cells - pathological fractures and bone pain is common
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what are the 4 key features of multiple myeloma?
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1. bone lesions, 2. infections, 3. renal insufficiency, 4. hypercalcemia
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what is the second most common cause of death in multiple myeloma?
|
renal insufficiency
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what is the diagnostic triad for multiple myeloma?
|
1. M-spike IgG > IgA (can be light chain only which won't have a spike),
2. monoclonal plasma cells in bone marrow and 3. end organ or tissue impariment: CRAB - calcium abnormalities, renal abnormalites, anemia and bone lesiosn |
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what determines prognosis is multiple myeloma?
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B2-micorglobulin - increases with increasing tumor burden; plasma cell labeling index increases with worse prognosis, anaplastic myeloma worse prognosis
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what is the treatment for multiple myeloma?
|
hematopoeitic stem cells autologous or allogeneic if possible
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monoclonal IgM with lymphoplasmocytic lymphoma
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Waldenstrom's macroglobulinemia
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what is the common feature of amyloidosis?
|
precipitation of protein in beta-pleated sheets
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what is the most common type of amyloidosis?
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AL type - Ig light chain fragments; Lambda > kappa
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what is the second most common type of amyloidosis?
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AA type: SAA protein - seen with chronic inflammatory conditions = RA
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congo red apple-green birefringent
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amlyoidosis
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what organs are most often involved in amyloidosis?
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heart, kidney, skin, liver, lungs, and nerves
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when do you see AL type amyloidosis?
|
multiple myeloma or Waldenstrom's; but can be primary diseae
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clonal proliferation of immature hematopoietic precursors with absent/impaired response to regulatory controls and imparied maturation
|
acute leukemias
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what is suppressed in acute leukemias?
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normal hematopoiesis - granulocytopenia, anemia, thrombocytopenia
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what population is ALL common?
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kids more than adults
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what is the main cause of death in Acute leukemias?
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suppression of normal hematopoiesis - infection and hemorrhage
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what are the metabolic complications of acute leukemias?
|
hyperuricemia, hyperphosphatemia, tumor lysis syndrome with acute renal failure, hyper or hypo-kalemia
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what is hyperviscocity syndrome?
|
medical emergency, occurs with high blast counts, AML >> ALL, CNS and respiratory symptoms; treat with leukapheresis and chemo
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what type of cell is most common with ALL?
|
B cell precursors, the rest are precursor t-cells
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basophilic cytoplasm and clear cytoplasmic vacuoles in mature B-cells
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Burkitt cell leukemia
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what are the prognosis factors for ALL?
|
age - 2-10 yoa best; WBC count - lower the better, phenotype early precursor B-cell - best; hyperdiploid - favorable, t(12;21) - favorable; t(9;22) or t(8;14) = both very unfavorable
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how do you treat ALL?
|
3 phases: remission induction, consolidtaion (intensification) and maintianence - for 2-3 years
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what is needed in treatment of ALL?
|
CNS prophylasxis - to prevent relapse
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what is the most common leukemia in adults?
|
AML - increases with age male > female
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t(8;21)
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AML-M2 - diagnostic without blast count
|
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t(15;17)
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acute promyelocytic AML - diagnostic without blast count
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inv(16)
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AML-M4Eo - diagnostic without blast count
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t(11q23)
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Infant AML - M4, M5
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deletion of 5, 7, 5q or 7a
|
therapy related AML
|
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AML prognosis: t(8;21)
|
favorable
|
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AML prognosis: t(15;17)
|
favorable
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AML prognosis: Inv(16)
|
favorable
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AML prognosis: t(11q23)
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unfavorable
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AML prognosis: deletions of 5, 7, 5q or 7q
|
unfavorable
|
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myeloperoxidase stain
|
AML - diagnostic
|
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|
auer rods
|
AML
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what are the prognosis factors of AML?
|
better if: <40, de novo, low WBC count, DIC absent, LDH normal
|
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what is unique about acute promyelocytic leukemia?
|
AML subtype that affects young pt, associated with DIC, and has a unique therapy: ATRA! T(15;17) PML/RARalpha
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PML/RARalpha
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acute promyelocytic AML
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what is the major cause of death in myelodysplastic syndrome?
|
marrow failure (not leukemic transformation)
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cytopenia, morphologic abnormalities in blood in marrow - with increased apoptosis and ineffective hematopoeisis
|
myelodysplastic syndrome
|
|
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how do myelodysplastic syndromes present?
|
elderly men, nonspeciifc symptoms, recurrent infections or hemorrages
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what neoplasm has decreased cells that are abnormal?
|
myelodysplastic syndrome
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what is treatment-associated myelodysplastic syndrome?
|
occurs after chemo for HL, NHL; alkylating agents and nitrosoureas, usualy 5-10 yrs after therapy
|
|
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ringed sideroblasts
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myelodysplastic syndrome commonly causes these, can be seen with some meds, alcohol abuse and some inhertied conditions
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what is 5- syndrome?
|
type of myelodysplastic syndrome - older pt, women, refracotry macrocytic anemia, normal or increased platelet count, 5q- is SOLE abnormality; prolonged survival
|
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what are the prognosis factors for myelodysplasia?
|
older - worse, more cytopenias - worse, more blasts - worse, 7-, 7q-, 5- >3 abnormalities - worse
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where are the msot common sites for relapse of ALL?
|
CNS, testes, marrow
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chronic proliferation of hematopoietic stem cells with maturation and differentiation preserved leading to increased numbers of mature, relatively normal cells
|
chronic myeloproliferative neoplasms
|
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which chronic myeloproliferative neoplasms have the greatest risk of transfomring to AML?
|
CML and primary myelofibrosis - PV or ET have a low risk
|
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what are the cytogenic abnormaites found in chronic myeloprolifreative neoplasms?
|
TK - controls growth and STAT pathway; and JAK2-617F occurs in all non-CML variants
|
|
|
philadelphia chromosome
|
CML
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t(9;22)
|
CML
|
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|
bcr/abl
|
CML - codes for p210 that is a more active TK; rare cases make p190
|
|
|
what is a predisposing factor for CML?
|
ionizing radiation
|
|
|
histology: granulocytosis, all stages of maturation present, predmonient myelocytes and segs, increased basophils
|
CML
|
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what is associated with increased basophils?
|
CML
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what are the findings of CML?
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splenomegaly - can be marked,
WBC high, thormbocytosis, LAP score low; GOUT can occur |
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what is the course of CML?
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chronic phase - 3or 4 years; blast crisis >20% blasts in marrow or blood; acclerated phase - increased blasts, WBC count - msot develop crisis; burn out - fibrotic phase resembling PM
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what is the DOC for CML?
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Gleevec, hydroxyuria can help with WBC count
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what is Gleevec?
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inhibitor of bcr/abl TK - blocks ATP binding site- initial TOC for chronic phase CML
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what is the curative treatmetn for CML?
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allogenic bone marrow transplant - the earlier the better
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excess production of RBCs
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polycythemia vera
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what is the main risk of polycythemia vera?
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thrombosis - treat with phlebotomy+/- hydroxyurea
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marked increase in platelets
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essential thrombocythemia
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how can you distinguish PV from reactive process?
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in PV EPO is low
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hypercellular marrow with stainable iron
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PV
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pruritis after takinga hot shower
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PV
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"ruddy" skin - red/dark blotches
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PV
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what population does ET affect more?
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women
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giant platelets, bizarre shaped platetes, megakaryocyte nuclear fragments >600K platelet count
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ET
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clustering megakaryocytes in marrow
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ET
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leukoerythroblast reaction in blood, fibrosis in marrow, splenomegaly
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PM
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tear drop RBCs, nulceated RBCs, immature granulocyte precursors and giant platelets
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PM
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bone marrow stains for reticulin
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PM
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what are the major causes of death in PM?
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hemorrhage, infection, heart failure, leukemic transformation
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what kind of LAP score does CML have?
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decreased
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what is actually increased in PV?
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RBC mass (not % that is erythrocytosis)
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what leukemia can have thrombocytosis?
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CML
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