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Flashcards

Hematology

annerao Oncology Boards Inglês 43 flashcards

Flashcards do conjunto (43)

  1. 1Hemoglobin F Hb Falpha 2
    gamma 2
  2. 2Hemoglobin A2 Hb A2alpha 2
    delta 2
  3. 3Hemoglobin A1 Hb A1alpha 2
    beta 2
  4. 4Hemoglobin H Hb Hbeta 4
  5. 5Barts Hemoglobingamma 4
  6. 6Heinz bodiesprecipitation of alpha chains in Beta thalassemia major, severe anemia & extramedullary hematopoiesis.
  7. 7Beta ThalassemiaHemoglobin A2
    more than 3.5% is diagnostic.
    (Beta. Minor, Intermedia, Major)
  8. 8alpha thalassemia alpha chain gene deletionalpha trait- no sx
    alpha thal minor- mild or No anemia
    Hg H- unstable Hb, jaundice @birth
    Barts- hydrops fetalis
  9. 9Sickle cell Dz 6th codon of beta globin on chr 11 valine for glutamic acidSC > Sb0 > SC > Sb+
  10. 10RBC low, Hb low, Rdw high, low MCHC BM- cells with iron aggragates around nucleus, iron clusters in RBCSideroblatic anemia
    Impaired Heme synthesis
    Ringed sideroblasts
    pappenheimer bodies
  11. 11increased hemolysis, jaundice, Spleen, pallor, reticulocytosis, normocytic anemia, increased MCHC, nuclear remnants in the cells Most cc of hemolytic anemia in northern europe??Spherocytosis
    defective ankyrin & spectrin
    autosomal dominant
    Howell jolly bodies
    Osmotic fragility test
  12. 12Anemia, abnormal thumbs & radii, microcephaly, short stature, CALS, hyperpigmentation, renal defectsFanconi Anemia
  13. 13Hypophosphatemia due to phosphaturia renal glycosuria with normal bld glucose Aminoaciduria, Tubular proteinuria, proximal RTAFanconi Syndrome
    proximal renal tubular dysfunction
  14. 14Macrocytic anemia, low retics, low wbc, low pltlets, Abnormal thumbs, Short stature, renal defects, hypogonadism, gluacoma, MR, cong.heart defect, dysmorphism(micrognathia, flat thenar)Diamond Blackfan Anemia
  15. 15Low platelets, normal RBC & WBC absent radius, normal thumbs, milk protein allergyTAR syndrome
  16. 16low WBC, exocrine pancreatic failure, Short stature, metaphyseal dysostosisSchwachman Diamond Syndrome
    Autosomal Recessive
    Rx: G-CSF improves nuetropenia
    SCT is curative.
  17. 17Oxidative injury causes Hemolysis? only in Boys.G6PD deficiency, X-linked disorder
    Dx: measure G6PD activity
    Rx: Transfusion
  18. 18Hemolysis, Pts stable and asymptomatic with Hb 5-6 (due to high 2, 3 DPG, oxy curve shifts to easily release o2)Pyruvate Kinase deficiency
    auto Recessive.
    Dx: direct enzyme measurement
    Tx: Splenectomy increases Hb and retic count.
  19. 19Early AM hematuria, low RBC, low pltlets, venous thrombosis, aplastic anemia, chest pain, abd.painParoxysmal nocturnal hemoglobinuria
    complement mediated hemolysis by MAC
    Dx: flow cytometry, sucrose lysis, Ham's acidified serum test
    Rx: steroids, NO donors, transfusion, iron
    SCT is curative.
  20. 20Anemia, jaundice, hemolysis, dark urine, spleen, increased retics, smear -microspheorcytesAutoimmune Hemolytic Anemia (AIHA)
    UA- heme, coombs test,
    Rx: steroids, IVIG, plasmapheresis
    transfusion, splenectomy
  21. 21Neutropenia Q monthly or Q wkly ANC< 200, Repeated fevers, aphtous ulcers, LAD, skin infections, pneumonias, perineal ulcersCyclic Neutropenia
    Autosomal Dominant
    Neutrophil elastase EL2 gene mutation
    Dx: CBC twice a week for 6 wks.
    Rx: G-CSF
  22. 22Neupogen (Filgrastim) Neulasta (PEG-filgrastim)G-CSF (colonystimulating factor 3) stimulstes bome marrow to produce granulocytes and stem cells which are eventually released into blood stream.
  23. 23Neutrophils are chronically low. ANC < 200, BM shows cells in promyelocyte stageSevere Congenital Neutropenia
    Kostmann's Neutropenia
    Rx: G-CSF
    SCT is curative
  24. 24Children less than 3 yrs present with mild skin & mucosal membrane infections. also have low wbc and neutropeniaBenign neutropenia of childhood
  25. 25Toddlers less than 3 yrs present with viral infection, sudden onselt anemia, normal MCV, self resolvingTransient Erythroblastopenia of childhood
  26. 26Formula to calculate ANC from CBCD(segs + bands) X WBC
    < 500 severe neutropenia
    500-1000 moderate
    1000-1500 mild
  27. 27Recurrent skin infections, severe periodontitis, gingivitis, pneumonia, Elevated WBC count, delayed umbilical seperation, OmphalitisLeukocyte adhesion defect LAD
    Defect CD18 locus on Chr21
    Dx: Flow cytometry for CD18
    SCT curative
  28. 28Staph/Ecoli infections, invasive fungal infections, CBC shows elevated WBC with left shiftChronic granulomatous disease
    X-linked / AR
    Dx: Dihydrorhodamine oxidation test
    NBT test
    Rx: Abx, bactrim proph, recombinant interferon gamma
    SCT is curative.
  29. 29Oculocutaneous albinism, neutropenia, prolonged bleeding time, frequent infectionsChediak Higashi Syndrome
    Large granules in neutros and eosinos
    EBV infection can lead to death.
  30. 30Recurrent infections, Eczema, skin cold abscess, Boils, allergies, elevated IgEHyper IgE syndrome
    Rx: Bactrim proph
    SCT is curative
  31. 31Aquired TTP is due to autoantibodies to??ADAMSTS 13
  32. 322-5 yr old healthy pt suddenly presents post viral infection with skin & mucosal bleeding, CBC shows isolated low and large platelets, normal RBC and WBCITP
  33. 33Platelet dysfunction, mild thrombocytopenia with large platelets. Prolonged bleeding time with significant mucocutaneous bleedingBernard Soulier Syndrome
    AR
  34. 34Puertorican descent oculocutaneous albinism, moderate severe bleeding.Hermansky Pudlak Syndrome
    AR
  35. 35Huge hemangiomas, with localized DIC and platelet destruction,Kasabach-merritt Syndrome
  36. 36HemophiliaFactor VIII def
    prolong PTT
    normal PT
  37. 37Hemophilia Treamentmild: desmopressin
    mod: recombinant factor VIII
    severe: prothrombin complex concentrate & recombinant VIIA
  38. 38Christmas DiseaseFactor IX def
    Rx: give recombinant factor IX
  39. 39Von Willebrand Dz type 1: less Vwbf type 2: vwbf doesn't work well type 3: no vwbf activity at alltype 1: desmopressin
    type 2: give factor VIII
    type 3: factor VIII + vwbf
  40. 40Normal PT prolonged PTT But no h/o clinical bleedingFactor X11 (12) def.
    hageman factor
  41. 41normal PT normal PTT normal platelets but still bleeding...?? Bleeding from Umb.cord, ICH, delayed wound healingfactor XIII (13) def
    Dx: Eugloblin lysis test
    Rx: Give factor 13 concentrate/ FFP/ cryoprecipitate
  42. 42prolonged PT, prolonged PTT Thrombocytopenia, hemolytic anemia low fibrinogen, elevated D-dimer Bleeding from everywhereDIC
    Moderate thrombocytopenia give Pltlets
    Severe: FFp/Cryoprecipitate
  43. 43Hemorrhagic dz in a newborn prolonged PT more than prolonged PTTVitamin K deficiency
    (dependent factors- 2,7,9,10, C & S)

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Boards Oncology FlashCards sobre Hematology, criado por annerao em 02-07-2013.
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annerao
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