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45 Cards in this Set
- Front
- Back
What is wrong in leukocyte adhesion disorder?
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Deficient selection of CD11a/CD18
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What's wrong in a phagocytosis defect
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ex. decreased opsonins in Bruton's agammaglobulinemia
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what's wrong in microbicidal defect?
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ex. deficiency of myeloperodixase
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What sort of pathogens will you find in a leukocyte function disorder?
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coagulase negitive staphylococcus, growth failure in children, frequent infections, lack of an inflammatory response, severe gingivitus
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What is typical of a leukamoid reaction?
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absolute leukocyte count great than 50k per microliter, can be from perforating appendicits (neutrophils), whooping cough (lymphocytes), cutaneous larva migrans (eosinophils)
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neutrophilic leukocytosis
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above 7,000 microliter from infection, or sterile inflammation with necrosis (MI), drugs (corticosteroids)
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Common leukemia newborn to 14
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ALL
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common leukemia 15-39 years
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AML
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common leukemia 40-60 years
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CML
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Common leukemia 60+years
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CLL
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Signs of acute leukemia
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arupt fever, bleeeding, fatigue, hepatosplenomegaly, lymphadenopathy, CNS involvement (esp ALL), skin (esp T cell), bone pain and tenderness
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Labs in ALL
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peripheral WBC less than 10k to more than 100K per microL, blasts more than 20%, normoto macrocytic anemia, thrombocytopenia
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Type of bone marrow in ALL
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more than 20% blasts, hypercellular
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Clinical findings in chronic leukemia
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hepatosplenomegaly and generalized lymphadenopathy; insiduous onset
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Lab findings in a chronic leukemia
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peripheral-blasts usually less than 10%, normocytoic to marcocyteic anemia thrombocytopenia in CLL (CML has thrombocytosis in 40% of cases)
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bone marrow in chronic leukemia
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hypercellular with less than 10% blasts
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Types of chronic myeloproliferative disorders
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polycythemia, vera, CML, myeloid metaplasia, Essential thrombocyotpenia
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General characteristics of myeloproliferative disorder
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splenomegaly, reactive marrow fibrosis, can transform to acute leukemia
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Lab signs for polycythemia vera
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increase Hb, Hct, and RBC count, low epo
(RBC count=RBC mass/plasma volume) |
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relative polycythemia
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increased RBC count due to volume deplation, RBC mass should be normal as EPO, often after exercising
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Appropriate abosolute polycythemia
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due to hypoxic cause of EPO release (lung disease, congenital heart disease, Living at high alt) probably will have low O2 sat and normal plasma volume
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Clinical symptoms of p. vera
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ruddy face, pruritus after bathing, peptic ulcer disease (histamine release) thrombotic events (hyperviscous blood), gout, splenomegaly
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Risk factors for CML
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prior ionizing radiation and benzene
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Chromosomal abnormality and tx for CML
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Philadelphia chromosome; 9;22, imantinib (gleevac),
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how long to get a blast crisis for CML
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approx 5 years
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lab findings in CML
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peripheral WBC 5ok-100k/microL, myeloid in all stages, thrombocytosis n 40-50% of cases (thrombocytopenia in the others),
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Is leukocyte alkaline phosphate (LAP) ncreased with CML
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no, decreased. LAP is absent in neoblastic cells
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number of cells that have philidelphia chromosome in CML? Bcr-abl
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95% philidelphia, (philiedlphia can be present in other leukemias as well) and 100% with BCR-ABL fusion
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Clinical findings of myelofibrosis and myeoloid metaplasia
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peripheral WBC 10k microliters to 50k microliters, massive splenomegaly with portal hypertension, splenic infarcts with left sided pleural effusions
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Myelodysplastic syndrome lab findings
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severe pancytopenia, dimorphic RBC pop (both microcytic and macrocytic), ringed siderblasts in marrow (nucleated RBCs with excess iron)
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treatment fo AML?
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Induction: chemotherapy “7 + 3” cytarabine (ara-C; cytosine arabinoside) + daunorubicin (or idarubicin)
consolidation:High dose ara-C (aka Cytarabine) (HidAC) fludarabine (used in minitransplant) |
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chromosomal translocation in APL M3?
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t(15;17), treatment with transretinoic acid or ATRA (all trans retinoic acid)
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Smear finding in AML cells
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Auer rods in M1 (rare)-M4 (common)-splinter shaped to rod shaped in cytosol
auer rods ONLY in AML NOT CML |
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clinical findings in AML
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DIC, gingival infiltration (esp M5),
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Most common subtype of ALL
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early pre-b cell (80%) of cases has CALLA, CD10, Tdt, and 90% complete remission
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Common places for ALL to metastasize
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CNS and testis -B cell types
anterior mediastinal masses-t cell types |
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labs findings in ALL
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peripheral WBCs 10k to 100k per microL with over 20% lymphocytes, normocytic anemia
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what virus is associated with adult T cell leukemia?
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HTLV-1 human t cell leukemia virus
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treatments for ALL
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Daunorubicin, Prednisone, Vincristine, L-asparaginase
consolidation: High dose ara-C (aka Cytarabine) (HidAC) HyperCVAD - Cyclophosphamide, Vincristine, Adriamycin (Doxorubicin), Dexamethasone then later, Methotrexate, Ara C - ALL |
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bone marrow findings in ALL
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lytic bone lesions and marrow replaced by CD4 lymphocytes
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What is the most common leukemia overall?
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CLL
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pathogenesis of CLL?
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neoplastic disorder of virgin B cells
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clinical findings in CLL
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general lymphadenopathy, increased incidence of immune hemolytic anemia (both warm and cold)
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lab findings in CLL
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smudge cells on smear, less than 10% lymphoblasts, neutropenia, hypogammaglobulinemia
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stain for harry cell leukemia
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Positive trap stain
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