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32 Cards in this Set
- Front
- Back
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What is leukemia?
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Malignant disease of bone marrow stem cells.
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What are the age ranges for the common leukemias?
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Acute lymphoblastic leukemia (ALL): Newborn to 14 years old
Acute myeloblastic leukemia (AML): 15-60 Chronic myelogenous leukemia (CML): 40-60+ Chronic lymphocytic leukemia (CLL): 60+ |
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What is the most common overall type of leukemia?
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Chronic lymphocytic leukemia (CLL)
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What is the pathogenesis of leukemia?
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There is a block in stem cell differentiation -> monoclonal proliferation of neoplastic leukocytes behind the block
Leukemic cells replace most of the bone marrow -> replace normal hematopoietic cells -> enter peripheral blood -> metastasize |
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In which leukemia are the CNS and testicles involved?
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ALL
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What is a major lab difference between chronic and acute leukemias?
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Acute leukemia have blast cells present, usually >20% in bone marrow.
Chronic leukemias have <10% blast cells in bone marrow. |
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Which leukemia has the worst survival rate? Best survival rate?
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Worst - AML: 21% 5-year survival.
Best – CML: 89% 5-year survival. |
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What is polycythemia vera? What is the pathogenesis?
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Inappropriate absolute increase in Hb, Hct, and RBC count.
This is a clonal expansion of the myeloid stem cell, most commonly due to mutation of JAK2 gene on chromosome 9. This leads to increased production of RBCs, granulocytes (neutrophils, eosinophils, basophils), mast cells, and platelets |
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What are the major and minor criteria for diagnosing polycythemia vera? What is required for diagnosis?
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Major:
Increased RBC mass: >36 ml/kg (male), >32 ml/kg (female) Normal SaO2: (>92%) Splenomegaly Minor: Absolute leukocytosis: >12,000 cells/mm^3 Thrombocytosis: >400,000 Increased serum leukocyte alkaline phosphatase: >100 score Increased serum vitamin B12 Diagnosis is three major criteria or first two major plus two minor |
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What is the pathogenesis of CML?
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Neoplastic clonal expansion of the pluripotential stem cell. Stem cell has the capacity to differentiate into a lymphoid or myeloid stem cell.
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What is the translocation that is associated with CML?
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t9;22 translocation of ABL proto-oncogene. Proto-oncogene fuses with the break cluster region (BCR) on chromosome 22 (BCR-ABL fusion gene) -> Philadelphia chromosome
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What is a blast crisis?
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In CML, a blast crisis occurs when there is an increase in numbers of myeloblasts or lymphoblasts.
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In what conditions do you see teardrop cells?
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Tear drop cells are seen in myelofibrosis and myeloid metaplasia.
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What is the translocation associated with AML?
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T(15;17) is acute promyelocytic leukemia
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What are the clinical findings of AML?
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DIC is common, and always in M3
Gum infiltration Auer rods |
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What are the markers of ALL?
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Terminal deoxynucleotidyl transferase (TdT)
CALLA |
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What is the pathogenesis of Chronic lymphocytic leukemia (CLL)? What is the diagnostic feature?
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Neoplastic disorder of virgin B cells (B cells that cannot differentiate into plasma cells).
Numerous “Smudge” cells (fragile leukemic cells) |
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What is the only leukemia without lymphadenopthy? What does this stain with?
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Hairy cell leukemia
Stains TRAP (tartrate-resistant acid phosphatase) |
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What is the pathogenesis of non-Hodgkin’s lymphoma?
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Mutation produces a block at a specific stage in development of B or T cells.
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What are the B-cell Non-Hodgkin’s lymphoma?
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Burkitt’s lymphoma
Diffuse large B-cell lymphoma Extranodal marginal zone lymphoma Mantle cell lymphoma Follicular lymphoma Small lymphocytic lymphoma (SLL) |
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What is the translocation associated with Burkitt’s lymphoma?
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t(8;14) -> c-myc activation
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What is the translocation associated with Follicular lymphoma?
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t(14;18) -> overexpression of BCL2 antiapoptosis gene
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Which lymphoma may be caused by HTLV-1?
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Adult T-cell lymphoma
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What is the presentation of Mycosis fungoides and Sezary syndrome?
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Mycosis fungoides: begins in skin (rash to plaque to nodular masses) -> progresses to lymph nodes, lung, liver, and spleen; groups of neoplastic cells in the epidermis are called Pautrier’s microabscesses.
Sezary syndrome: mycosis fungoides with a leukemic phase |
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What are the Hodgkin’s lymphomas?
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Nodular sclerosing (60%)
Mixed cellularity (30%) Lymphocyte predominant (5%) Lymphocyte depleted (5%) |
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What are Reed-Sternberg cells? What markers are found on them?
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Distinctive tumor giant cells; binucleate or bilobed with the 2 halves as mirror images.
RS cells are CD30+ and CD15+ B-cell origin |
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In which Hodgkin’s lymphoma are the Reed-Sternberg cells seen best?
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Mixed cellularity
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Which Hodgkin’s lymphoma is more common in females? What is a characteristic of this type?
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Nodular sclerosing, with collagen banding and lacunar cells.
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What determines the prognosis of Hodgkin’s lymphoma? Which type generally has the best prognosis? Worst prognosis?
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The lymphocyte-to-Reed Sternberg cells ratio. The higher the ratio the better the prognosis.
Best prognosis: Lymphocyte predominant Worst prognosis: Lymphocyte depleted |
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In what disease will you find “tennis rackets” on EM?
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Langerhans cell histiocytosis (LCH). This is a proliferative disorder of dendritic (Langerhans) cells. Defective cells express S-100 and CD1a. The tennis racket shapes on EM are Birbeck granules.
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What is the most common primary tumor arising within bone in the elderly ( >40 to 50)?
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Multiple myeloma
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What are the symptoms associated with multiple myeloma?
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Bone pain due to “punched out” lytic lesions
Hypercalcemia Renal failure Anemia with rouleax formation Bence Jones proteins: Ig light chains in urine Recurrent infection |
