Hemotology

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pszurnicki
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189697
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Hemotology
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2013-02-24 16:35:56
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Hemotology
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  1. what microcytic anemia has low RDW/?
    Iron deficiency
  2. Best initial test for microcytic anemia?
    ferritin
  3. Who gets IM/IV iron?
    person that refuses blood transfusion
  4. What can cause anemia and neurological symptoms?
    • alcohol,
    • b12
    • folate deficiencies
  5. How do you become deficient in folate?
    • increased cell turnover is most common
    • decreased leafy intake is 2nd most common
  6. Why do you order methylmalonyl acid level?
    bc b12 levels could be normal but you are truly deficient
  7. What is the smear with sickle cell trait vs disease?
    • trait--normal
    • disease-- sickled cells
  8. the only clinical manifestation of sicle cell trait?
    renal
  9. sickle cell + fever, next best step'?
    antibiotics
  10. large Drop in ht in siclke cell patient?
    parvo b 19 virus
  11. Most accurate test for parvo b19?
    • pcr 
    • second is IgM
  12. best treatment for parvo19 ?
    iv immunoglobulin
  13. treatment for priapism, cns, eye infarctions in sickle cell?
    exchange transfusion
  14. How do you prophylax against sickle cell crisis?
    hydroxyurea
  15. RX for autoimmune anemia, hereditary spherocytosis?
    splenectomy
  16. Test for PNH?
    Hams test
  17. What anemia has heinz bodies?
    G6PD deficiency causing anemia
  18. Bite cells?
    G6PD
  19. What is the most common oxidant stress?
    infection
  20. Which type of Acute leukemia has auer rods and myeloperoxidase?
    CALLA +?
    • AML
    • ALL
  21. Which AML presents with DIC?
    M3
  22. All forms of AML except for m3 are treated with?
    • douxorubcin
    •  ara-c
  23. M3 of AML is treated with?
    retinoic acid
  24. Treatement for ALL?
    • doxorubicin
    • vincristine
    • prednisone
  25. Rx for relapsed acute leukemia?
    BMT
  26. Who should get transplanted after 1st remission?
    bad cytogenetics
  27. ALL relapses where?
    RX?
    • in the spinal chord
    • Intrathecal MTX
  28. When are xenografts used?
    heart valves
  29. 4 oncologic emergenices?
    • hypercalcemia
    • superior vena cava syndrome
    • leukostasis
    • spinal cord compression
  30. What WBC count increases risk for leukostasis?
    100,000
  31. HOw do you treat leukostasis?
    leukopheresis
  32. in What age groups are ALL and AML present?
    • AML--over 60
    • ALL--in kids
  33. Presence of what chromosome in ALL shows poor prognosis?
    9:22
  34. Which leukemia has smudge cells?
    CLL
  35. What is the survival expectancy for acute vs chronic leukemias?
    • acute--6 to 12 weeks from diagnosis
    • chronic-6 to 12 years from diagnosis
  36. MCC side effect of CLL?
    autoimmune hemolytic anemia
  37. Leukemia vs leukemoid?
    • LAP--high---leukemoid rxn
    • low--leukemia
  38. most common cause of death in cml?
    blast crisis
  39. Rx for CML?
    imatinib--bcr-abl chromosome antagonist
  40. What will cure CML?
    BMT
  41. underage what age do u do allogenic vs autologous bone marrow transplant?
    • allogenic- less than 50
    • autologous less than 70
  42. neoplasm associated with down syndrome?
    ALL
  43. neoplasm associated with plummer vinson syndrom--atrophic glossitis, esophageal webs, anemia
    squamous cell carcinoma
  44. neoplasm associated with xeroderma pigmentosum, albinism?
    • melanoma
    • basal cell ca
    • squamous cell ca
  45. neoplasm associated with tuberous sclerosis
    • astrocytoma
    • angiomyolipoma
    • cardiac rhabdomyosarcoma
  46. neoplasm associated with chronic atrophic gastritis, pernicous anemia, post surgical remnants?
    gastric adenocarcinoma
  47. neoplasm associated with actinic keratosis?
    squamous cell
  48. neoplasm associated with barrets esophagus?
    esophageal adenocarcinoma
  49. neoplasm associated with cirrhosis?
    HCC
  50. neoplasm associated with ulcerative colitis?
    colonic adenocarcinoma
  51. neoplasm associated with pagets disease of bone?
    • secondary osteosarcoma
    • fibrosarcoma
  52. neoplasm associated with immunodeficiency?
    malignant lymphomas
  53. neoplasm associated with Aids
    • kaposi sarcoma
    • non hodkings lymphoma
  54. Causes of Microcytic anemia?
    • Thalasemia
    • Iron deficiency
    • Chronic disease
    • sideroblastic anemia--ala synthatase def
  55. + dipstick for blood can be what 3 entities?
    • hemoglobin
    • myoglobin
    • rbc
  56. Nephrotoxic drugs?
    • amphotericin
    • aminoglycoside
    • acyglovir
    • cyclosporine
    • cisplatin
  57. Which form of thalasemia has increased reticulocyte count?
    3 gene deletion or hemoglobin H
  58. Thalassemia beta minor and major both have what hemoglobin?
    fetal
  59. How is thalassemia beta minor diagnosed on electrophoresis?
    hbA2 >3.5 %
  60. 5 conditions that lead to cessation of heme synthesis?
    • ala synthase-- sideroblastic anemia/etoh/inh
    • ala dyhydratase/ferrochelatase- lead
    • porphobilinogen deaminase-- acute intermittent porphyria
    • uroporphyrinogen decarboxylase- cuteanea porphyria tarda
  61. what inhibits ala synthase?
    glucose and heme
  62. liver disease, def of b12 and folate cause macrocytic anemia but what 2 only cause hypersegmented neutrophils?
    b12 or folate deficiency
  63. after suspective a macrocytic anemia and cbc showing large mcv, what is the next best step in management?
    peripheral smear to see the presence of hypersegmented neutrophils
  64. Best initial test in suspecting anemia?
    CBC
  65. Best initial test in suspecting SS?
    Best initial test in suspecting macrocytic anemia?
    peripheral smear
  66. Most accurate test in suspecting ss?
    electrophoresis
  67. Most common presentation for children with SS?
    dactylitis
  68. What is seen in Ehrlichia infections?
    morulae inside neutrophils
  69. SS with fever or high WBC, best next management?
    antibiotics
  70. When is exchange transfusion used in SIckle cell?
    • severe vasoocclusive crisis:
    • acute chest syndrome
    • priapism
    • stroke
    • retinal infarction
  71. BEst initial test in a hct drop in a sickle cell pt?
    reticulocyte count due to possible  parvo b19 infection
  72. What is the only defect in sickle cell trait?
    cant concentrate the urine
  73. most accurate test for hereditary spherocytosis?
    osmotic fragility
  74. most accurate test for PNH?
    hams test
  75. What causes autoimmune hemolysis?
    • CLL
    • lymphoma
    • sle
    • alphamethyl dopa
  76. most accurate diagonistic test for autoimmne hemolytic anemia?
    coombs test
  77. autoimmune hemolytic anemia can be igg or igm?
    what causes each?
    • igg--cll, sle, lymphoma, alphamethyl dopa
    • igm--mycoplasma, infections mono, infections
  78. What 2 causes of anemia give spherocytes?
    • hereditary spherocytosis
    • autoimmune hemolysis when ig pinch of a part of the membrane and the rbc becomes a sphere instead of biconcave shape
  79. best intitial therapy for ITP and autoimmune hemolytic anemia?
    steroids
  80. what rx is for recurrent autoimmune hemolysis and ITP?
    splenectomy
  81. severe, acute hemolysis or thrombocytopenia is best treated with ?
    IV Immunoglobulins
  82. MOA of cyclophosphamide?
    covalent linking of DNA at guanine N-7
  83. Toxicity of cyclophosphamide and ifofsamide?
    hemorrhagic cystitis, prevent with mesna
  84. MOA of azathioprine?
    antimetabolite precursor to 6-MP
  85. What drug increases toxicity of Azathioprine?
    allopurinol
  86. Alternative treatments to steroids in autoimmune disease like hemolysis?
    • cyclophosphamide
    • cyclosporine
    • azathioprine
    • mycophenolate mofetil
  87. Rx for autoimmune hemolytic anemia when splenectomy doesnt help?
    Rituximab
  88. Rx for cold agglutinins vs warm agglutinins?
    • cold:                    Warm:
    • rituximab              steroids
    •                              splenectomy
    •        immunosuppresants
    • cyclophosphamide, cyclosporine, azathioprine
  89. What is similar between cryoglobulins and cold agglutinins?
    Differences?
    • both are igM and dont respond to steroids
    • cryoglobulins are seen with hep c, causes joint pain and glomerulonephritis
  90. What is the best initial test for G6PD deficiency?
    MOst accurate test?
    • heinz bodies and bite cells on smear
    • G6PD level 2 months after a hemolytic event
  91. HUS and TTP are both treated wtih?
    plasmapheresis
  92. What is the most accurate test for PNH?
    decreased levels of cd55/59
  93. What is the best initial therapy for PNH?
    steroids
  94. What inactivates c5 in the complement pathway and improves PNH?
    Eculizumab
  95. Next best step when there is delay to plasmapharesis?
    transfuse ffp
  96. How is a true aplastic anemia due to autoimmune destruction of the bone marrow by Tcells treated?
    what if pt is over 50 or there is no matched donor?
    • by  an allogenic bone marrow transplant
    • anti-thymocyte globulin or cyclosporine/tacrolimus
  97. mnemonic for DDx?
    • acquired
    • vascular
    • inflammatory
    • trauma/toxin
    • autoimmune
    • metabolic/medications
    • Infection
    • Neoplastic
    • congenital
    • Degenerative
    • Endocrine/electrical
  98. Most accurate test for Polycythemia Vera?
    JAK2 mutation
  99. What mutation is seen in chronic myeloproliferative disorders such as polycythemia vera, essential thrombocytosis and myelofibrosis?
    JAK2 mutation
  100. WHat kind of kidney pathology increases epo?
    any--cyst, hydronephrosis, wilms tumor, RCC
  101. The best initial therapy for essential Thrombocytosis is ?
    hydroxyurea
  102. The best initial therapy for Polycythemia vera and Essential Thrombosis is what?
    Hydroxyurea
  103. Painfull red hands in Essential thrombosis are treated with?
    aspirin
  104. The best initial test in AML is?
    peripheral smear
  105. What is the most accurate test in AML?
    flow cytometry
  106. What is the translocation in AML M3?
    between chr 15 and chr 17
  107. MPO and Auer Rods are characteristic of what leukemia?
    AML
  108. What chemotherapy does ALL require?
    intrathecal therapy for CNS
  109. What is the next best step after a high neutrophil count is found in CML?
    Leukocyte alkaline phosphatase level
  110. MOst accurate test in CML?
    bcr-abl pcr
  111. What is the rx for leukostasis?
    plasmapharesis
  112. What kind of characteristic deletion is seen in MDS?
    5q deletion
  113. extreme friendliness, elfin facies with deletion of ch 7 is what syndrome?
    WIlliams
  114. Bilobed nucleus found in MDS is also called?
    Pelger-Huet Cells
  115. conversion of CLL into high grade lymphoma is called?
    Richter Phenomenon
  116. What myeloproliferative disorders most commonly converts into AML?
    CML
  117. Best initial for CML?
    Most effective cure for CML?
    • Imatinib
    • BMT
  118. What is done for CLL stage 0 and 1?
    2,3,4?
    • nothing
    • fludarabine + rituximab
  119. Refractory cases of CLL are treated with?
    Mild Cases?
    severe infections?
    ITP or AIHA?
    • cyclophosphamide
    • Chlorambucil
    • steroids
  120. Most accurate test for Hairy cell leukemia?
    TRAP or CD11c
  121. Best initial Therapy for NHL?
    excisional biopsy
  122. What are the staging procedures for NHL?
    • CT of chest, abdomen, pelvis
    • BOne marrow biopsy
  123. What is meant by the four stages for CLL?
    1,2,3,4
    • 0--high Wbc
    • 1-lymphadenopathy
    • 2- hepatosplenomegaly
    • 3-anemia
    • 4- thrombocytopenia
  124. When is local radiation done in NHL?
    stage 1a and IIa
  125. How is Stage III and IV or any condition with B symptoms treated?
    • Cyclophosphamide
    • Hydroxydoxorubicin
    • Oncovorin- vincristine
    • Prednisone
    • Rituximab
  126. Rx for hairy cell leukemia?
    cladribine
  127. PCP prophylaxis is indicated for which leukemia?
    CLL
  128. What is the causative organism in MALT?
    • H. pylori--treat with 
    • ppI
    • metro
    • tetracycline
    • bismuth
  129. How are hodkin and NHL treated?
    • both in stages Ia and IIa get radiation 
    • III and IV--chemotherapy--- adriamycin
    • bleomycin
    • vincristine
    • dacarbazine
  130. Staging for NHL and HD?
    • stage 1- 1 lymph node group
    • stage 2- 2 or more lymph node groups on one side of diaphragm
    • stage 3- both sides of diaphragm
    • stage 4- widespread disease
  131. How are relapses after radiation therapy treated in NHL and HD?
    after chemotherapy?
    • with chemotherapy
    • extra high doses of chemotherapy
  132. calcemia
    renal failure
    anemia
    bone lesions lytic
    MM
  133. Best initial test in suspecting MM?
    Xray
  134. What is the most accurate test for MM?
    bone marrow biopsy showing more than 10% plasma cells
  135. What does a technetium bone scan pick up?
    osteoblastic activity such as in  a prostate ca
  136. BEST INITIal therapy for MM?
    • dexamethasone 
    • lenalidomide
    • bortezomib
  137. Hypercalcemia leads to what kind of symptoms?
    • stones- renal
    • bones--lytic lesions bones
    • groans-constitpation
    • moans--psychotic
  138. What monoclonal gammopathy produces hyperviscosity?
    waldernstrons gammopathy
  139. Best intital therapy for Waldernstrons gammaglobulienemia?
    plasmapharesis
  140. What are dutcher bodies in waldenstrons gammaglobulinemia?
    pas+ IGM around the nucleus
  141. Management of severe bleeding due to thrombocytopenia?
    IViG
  142. next best step when  splenectomy or steroids arent effective in regulating ITP?
    • rituximab
    • azathioprine
    • cyclosporine
    • mycophenolate
  143. best initial therapy for VWF dz?
    • desmopressin
    • then factor 8
  144. most accurate test for hemophilia 
    specific assay for the missing factor 8 or 9
  145. factor 11 or 12 deficiency has increased bleeding/?
    factor 11
  146. What replaces fibrinogen in DIC?
    cryoprecipitate
  147. cause of hypercoagulabitliy
    • factor V leiden
    • ATIII deficiency
    • Prothrombin mutation
    • protein C and S
  148. Tests to confirm HIT?
    serotonin release assay

    platelet factor 4 antibody elisa
  149. Rx for HIT??
    lepirudin, bivalirudin direct thrombin inhibitor
  150. what is the only cause of thrombosis that elevates PTT?
    antiphospholipid syndrome
  151. BEst initial test for APL is ?
    most accurate test for lupus anticoagulant is ?
    • mixing study
    • russel viper venom test
  152. what anemia can progress to acute leukemia?
    sideroblastic anemia
  153. cause of hydrops fetalis?
    hemoglobin bart-- 4 gamma chains
  154. hemoglobin a2?
    2 delta chains
  155. what causes sickling of sickle cells?
    • hypoxia
    • infection
    • dehydration
    • acidosis
  156. on what antibiotic are sickle cell pts on untill 5 years old?
    penicillin
  157. acanthocyte (spurr or spiky) is seen where?
    abetalipoproteinemia
  158. How do steroids affect wbc count?
    • raises it acutely
    • but diminishes it chronically
  159.  Causes of eosinophilia?
    • drugs
    • neoplasm
    • atopic
    • asthma
    • adrenal insuffiecncy
    • ain
    • autoimmune
    • collagen vascular disease
    • parasites
  160. how u monitor lmwh?
    antifactor Xa
  161. symptoms of polycythemia vera?
    • thrombosis
    • eythromelalgia- burning in the hands and feet
    • pruritis
  162. Rx for pv?
    • aspirin for thrombosis and for the erythromelalgia
    • antimetabolite
  163. most common form of hodking disase?
    nodular scleroris--fibrosis of l. nodes
  164. horshoe kidney, short stature, varioius pigmentation, pancytopenia, absence of thumbs
    fanconi anemia
  165. What is the WAGR complex?
    • wilms tumor
    • aniridia
    • genitourinary malformation
    • retardation--mental-motor
  166. Hb goal in CKD?
    10-12
  167. causes of basophilic stapling?
    • thalassemia
    • alcohol
    • lead
  168. vit b12 def vs folate def?
    no neurological symp;toms in folate deficiency
  169. what anemia goes on to acute leukemia in 10% of patients?
    sideroblastic anemia

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