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Step 2 CK > Hem Onc > Flashcards

Hem Onc Flashcards

1
Q

Iron deficiency/thalassemia/anemia chronic disease iron, tibc, ferritin?

A

Iron deficiency-decreased iron, ferritin, increased TIBC, decrease transferrin
Thalassemia-increased iron, ferritin, decreased TIBC, increased transferrin
ACD-decreased iron, decreased TIBC, normal/increased ferritin, normal/decreased TIBC

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2
Q

Triad of symptoms in lead poisoning?

A

GI (abdominal pain, constipation), Neuro (peripheral neruopathy, cognitive impairment), Anemia (microcytic)

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3
Q

Tx. and complications of hereditary spherocytosis

A

Tx. folic acid, blood transfusions, splenectomy (definitive). Complications are pigment gallstones (extravascular hemolysis), aplastic crisis from B19 infection

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4
Q

Lab findings G6PD (LDH, haptoglobin, Hb, indirect bilirubin)

A

Increased LDH, decreased haptoglobin, decreased Hb, increased indirect bilirubin

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5
Q

Lab findings PNH?

A

Pancytopenia (destruction of all cells due to deficit of CD55/59), Elevated LDH and low haptoglobin, unconjugated hyperbilirubinemia, hemoglobinuria

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6
Q

Tx. PNH?

A
  • Prednisone
  • Iron/folate supplementation
  • Eculizumab (monoconal Ab inhibiting complement activation)
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7
Q

HUS findings? (4)

A

Microangiopathic hemolytic anemia (w/thrombocytopenia), fever, skin and mucosal bleeding, renal insufficiency (increased creatinine, hematuria, proteinuria, casts)

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8
Q

VTE testing for patients with more concerning findings of malignancy or recurrent or multiple site (eg cerebral, hepatic vein)

A

CT scan abdomen/pelvis/chest

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9
Q

Giant cell tumor (location, x-ray finding, pathogenesis)

A

Epiphysis, soap-bubble, osteolytic lesions from large osteoclast giant cells

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10
Q

Lymphadenopathy (benign vs malignant) characteristics

A

benign-2 cm, firm, solitary

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11
Q

Androgen abuse reproductive, CV, psychiatric and hematologic consequences

A

Reproductive (gynecomastia, small testes, azospermia), CV(LVH, increaed LDL and low HDL), Aggression and mood problems, hematologic (polycythemia and possible hypercoagulability)

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12
Q

MGUS vs Multiple Myeloma

A

MGUS (no CRAB, monoclonal protein 3 g/dL, >10% plasma, B2 microglobulin increased)

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13
Q

ITP lab finding/peripheral smear?

A

Lab finding: isolated thrombocytopenia, megakaryocytes on peripheral smear

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14
Q

ITP treatment children

A

Skin manifestations only: observe; bleeding: IVIG/glucocorticoids

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15
Q

ITP treatment adults

A

> 30K without bleeding: Observe;

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16
Q

Intravascular vs Extravascular hemolysis pathogenesis

A

Intravascular-RBC structural damage leading to RBC structural damage in intravascular space
Extravascular-RBC destroyed by phagocytes and RAA

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17
Q

Tx. AIHA (warm agglutinin vs. cold)

A

Warm-corticosteroids, IVIG, splenectomy for refractory
Cold-avoid cold, Rituximab +/- fludarabine or cyclophosphamide, cyclosporine, or other immunosuppresive stop production of antibody. STEROIDS DO NOT WORK COLD AGGLUTININ

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18
Q

Risk of sepsis for how long after splenectomy and recommendations to help try and decrease risk?

A

30 yrs or longer, triple vaccine 3-5 wks before splenectomy and oral penicillin for 5 years after

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19
Q

Features, timeframe, and cause of transfusion-related acute lung injury?

A

Dyspnea and pulmonary edema, within 6 hours, caused by donor anti-leukocyte antibodies

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20
Q

What is primary hypotension reaction and where is it seen?

A

Hypotension often seen in patients taking ACE inhibitors. Occurs within minutes of transfusion and caused by bradykinin in blood products as well as normal bradykinin buildup in patients

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21
Q

Triad of osler weber rendu?

A

Widespread telangiectasias, recurrent eistaxis, widespread AV malformations (mucus membranes, skin, GI tract)

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22
Q

Tx. of cancer related anorexia/cachexia syndrome?

A

Progesterone analogs (megestrol acetate and medroxyprogesterone acetate) and corticosteroids

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23
Q

Which 3 patient populations need irradiation to RBC before transfusion?

A

1) BMT recipients
2) Acquired or congenital cellular immunodeficiency
3) Blood components donated by 1st/2nd degree relatives

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24
Q

Which 4 patient populations need leukoreduction before RBC transfusion?

A

1) Chronically transfused patients (eg hemochromatosis)
2) CMV seroneative at risk patients (eg aids, transpant patients)
3) Potential transplant recipients
4) previous febrile nonhemolytic transfusion rxn

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25
Q

Which 3 populations need washing before RBC transfusion?

A

1) IgA deficieny
2) Complement dependent autoimmune hemolytic anemia
3) continued allergic rxn despite antihistaine

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26
Q

Anaphylaxis symptoms, timeframe, cause?

A

Urticaria, hypotension, dyspnea, angioedema, seconds to minutes, recipient IgA antibodies

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27
Q

Febrile acute hemolytic symptoms, timeframe, cause?

A

Fever, chill, flank pain, hemoglobinuria, renal failure and DIC.

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28
Q

Febrile nonhemolytic symptoms, timeframe, cause?

A

Fever and chill, 1-6 hours, cytokines accumulation during blood storage

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29
Q

TRALI (transfusion associated acute lung injury) symptoms, timefram, cause

A

Dyspnea, non-cardiogenic pulm edema, within 6 hours, donor anti-leukocyte antibodies

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30
Q

Allergic rxn blood transfusion symptoms, timeframe, cause?

A

Urticaria, flushing, angioedema, pruritis, 2-3 hours of transfusion, recipient IgE antibodies and mast cell activation

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31
Q

Delayed hemolytic symptoms, timeframe, cause?

A

Mild fever and hemolytic anemia, 2-10 days after, anamnestic antibody response

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32
Q

Gold standard confirmatory test HIT?

A

Serotonin release assay

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33
Q

Treatment HIT?

A

Remove all heparin. Supplement with fondaparinux or DTI (eg agratroban)

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34
Q

Factor Xa vs Warfarin (MOA, therapeutic effect, acute dvt tx, need for monitoring, antidote for hemorrhage)

A

Xa: DTI, 2-4 hrs, single dose, no monitoring, no antidote)
Warfarin: vit k antagonist, 5-7 days, bridge with heparin for 5 days, PT/INR, ffp and vit K as antidote)

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35
Q

Initial drug for hypercalcemia of malignancy depending on calcium level?

A

Asymptomatic or 14): Acute Normal saline plus calcitonin and long term with bisphosphonates

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36
Q

Anemia cause best initial test?

A

CBC

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37
Q

Anemia cause next best test?

A

Peripheral blood smear

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38
Q

Echinocytes or burr cells on smear etiology?

A

Uremia

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39
Q

Where is folate deficiency commonly seen?

A

Alcoholics and pregnant women

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40
Q

MCC B12 deficiency

A

Pernicious anemia

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41
Q

Confirmation of B12 deficiency

A

Serum B12

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42
Q

Tx of Bthal major or minor?

A

B thal minor-generally no treatment. B thal major-transfusions, iron chelation therapy to prevent secondary hemochromatosis.

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43
Q

4 commonly tested causes of autoimmune hemolytic anemia?

A
  • Lupus erythematous (lupus like syndromes, such as procainamide, hydralazine, and isoniazid)
  • Drugs (methyldopa, but penicillins, cephalosporins, sulfa drugs, and quinidine have also been implicated)
  • Leukemia or lymphoma
  • Infection (mycoplasma, EBV, syphilis)
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44
Q

When should screening be performed for children with lead poisoning?

A

6 months in children with risk factors

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45
Q

Tx. lead poisoning

A

avoid exposure (best strategy) as well as lead chelation if necessary. Use succimer in children and dimercaprol in adults; in severe cases, use dimercaprol plus EDTA for children/adults

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46
Q

Tx for sideroblastic anemia

A

supportive/pyridoxine

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47
Q

What is myelophthisic anemia? Waht clues on smear/biopsy suggest its presence?

A
  • Space occupying lesion in bone marrow. common causes are malignant invasion that destroys bone marrow and myelodysplasia or myelofibrosis
  • Smear with anisocytosis (diff size), poikilocytosis (diff shape), nucleated RBC, giant and/or bizarre looking platelets and teardrop shaped RBC
  • Biopsy either “dry tap” or malignant cells
48
Q

MCC blood transfusion rxn

A

Lab error. Use O neg blood to avoid a rxn when you can’t wait for blood typing or when blood bank does not have patient’s blood type

49
Q

Side effect large transfusions?

A

Bleeding diathesis from dilutional thrombocytopenia and citrate as calcium chelator

50
Q

Best initial therapy/ Long term therapy for waldenstrom macroglobulinemia?

A

Best initial-plasmapheresis

Long term-rituximab or prednisone or cyclophosphamide

51
Q

Tx. DIC?

A

Treat underlying condition followed by FFP

52
Q

TTP pathogenesis?

A

Deficiency of ADAMTS 12 (vWF metalloprotease)–>degradation of vWF multimers–> increase large vWF multimers–>increase platelet adhesion–>increase platelet aggregation and thrombosis

53
Q

TTP symptoms?

A

Fever, thrombocytopenia, hemolytic anemia, neurologic sequale

54
Q

TTP treatment?

A

Plasmapheresis, steroids. NO PLATELET b/c WORSE DISEASE

55
Q

3 major drugs that cause folic acid deficiency and subsequent anemia that need supplementation?

A

Phenytoin, Methotrexate (vs 5-FU not reversible with folic acid), Trimethoprim

56
Q

Expected symptoms with certain hematocrit

>30-35, 25-30, 20-25,

A

None, Dyspnea (worse on exertion) and fatigue, Lightheadedness and angina, syncope and chest pain

57
Q

Reticulocyte count in microcytic anemia? What is exception?

A

Low reticulocyte count. Alpha thal with 3 gene deleted (HbH) has reticulocytosis

58
Q

Reticulocyte count in macrocytic anemia?

A

Low

59
Q

When is reticulocyte count high?

A

Blood loss and hemolysis

60
Q

Indicate to transfuse (PRBC) for anemia?

A

Symptomatic, elder patient with heart disease and low hematocrit (

61
Q

Indication for FFP?

A

DIC, warfarin reversal, cirrhosis

62
Q

MCC sideroblastic anemia?

A

Alcohol–>bone marrow suppression

63
Q

Best initital test microcytic?

A

Iron studies

64
Q

Only form of microcytic anemia where circulating iron is elevated?

A

Sideroblastic

65
Q

Most accurate test sideroblastic?

A

Prussian blue

66
Q

Most accurate test iron deficiency?

A

Bone marrow biopsy for sustained iron

67
Q

Most accurate test thalassemia and findings?

A

Electrophoresis. Findings: Alpha normal until 3 gene deleted with HbH and 4 gene deleted in Hb bart (incompatible with life). B-minor (HbA2>3.5%), B-major (increase HbF+A2 on electrophoresis)

68
Q

Microcytic Anemia with ESRD routinely responds to what?

A

Erythropoietin replacement. Must be careful and have adequate iron stores because risk of EPO induced surge in RBC production

69
Q

Where is B12 absorbed?

A

terminal ileum

70
Q

Increase homocysteine and MVA in what deficiency compared to increased homocysteine in what deficiency?

A

Both-B12, One-Folate

71
Q

Complication of B12 or folate replacement?

A

Hypokalemia

72
Q

Best initial/most accurate test sickle cell?

A

Peripheral smear/Hb electrophoresis

73
Q

Tx. sickle cell

A

Hydration/oxygen, fever or elevated WBC use abx (ceftriaxone, levofloxacin or moxifloxacin), folic acid (excess cell turnover and reticulocytosis) replacement, hydroxyurea

74
Q

When is exchange transfusion indicated for severe vasoocclusive crisis?

A

-Acute chest syndrome, priapism stroke, visual disturbance from retinal infarction

75
Q

What is aplastic crisis in sickle cell? Best initial/most accurate test and best therapy?

A

Parvovirus B19 infection that freezes bone marrow production decreasing reticulocyte count.
Best initial-CBC, most accurate-PCR for DNA. IVIG for therapy

76
Q

Most accurate test AIHA warm vs. cold?

A

Coombs test (warm), Cold agglutinin titer (cold)

77
Q

Best initial/most accurate G6PD?

A

Best initial-PBC (bite cell and heinz body w/ methylene blue), Most accurate-g6pd 1-2 months after onset of hemolysis

78
Q

HUS vs. TTP associations for cause?

A

HUS-E coli O157:H7

TTP-ticlodipine, clopidogrel, cyclosporine, AIDS, SLE

79
Q

Presentation and etiology aplastic anemia?

A

Anemia, infection, thrombocytopenia (pancytopenia) of unclear etiology

80
Q

Definitive tx aplastic anemia?

A

50: Antithymocyte globulin (ATG) and cyclosporine/tacrolimus

81
Q

Why pruritis in PC vera?

A

Histamine release from increased basophils

82
Q

Most accurate test PC vera?

A

JAK2 mutation (V617F)

83
Q

Tx. PC vera?

A

Phlebotomy and aspirin prevent thrombosis, hydroxyurea helps lower cell count, allopurinol or rasburicase protects against uric acid rise, antihistamines

84
Q

What drug inhibits JAK2?

A

Ruxolitinib

85
Q

Essential thrombocytosis vs reactive thrombocytosis?

A

Essential greater than 1 million, Reactive 500-700k

86
Q

Essential thrombocytosis tx.?

A

60 w/ thromboses or >1.5 million: Hydroxyurea or anagrelide (when red cell suppression from hydroxyurea)

87
Q

Tx for myelofibrosis?

A

55: Thalidomide/lenalidomide (increase bone marrow production)

88
Q

Best initial/most accurate acute leukemia?

A

-PBC showing blasts best initial, flow cytometry most accurate

89
Q

Most common presentation AML (M3 type)?

A

DIC

90
Q

Best initial/most accurate acute leukemia?

A

Best initial-PBC, most accurate-flow cytometry

91
Q

Translocation AML and ALL?

A

AML-(15;17), ALL (12;21) is good prognosis or (9;22) is bad prognosis

92
Q

Tx. AML/ALL?

A

1) Chemo
2) Poor prognosis (via cytogenetics)–BMT; Good prognosis–>More chemo
3) Add ATRA (AML) or intrathecal methotrexate (prevent CNS/testes metastases) for ALL

93
Q

Best initial/Most accurate test CML

A

1) LAP, 2) BCRABL on PCR or FISH peripheral smear.

94
Q

Tx. CML?

A

Tyrosine kinase inhibitors (imatinib) best initial and BMT curative

95
Q

WHO CML phases?

A

Chronic -20$ blasts with extramedullary hematopoiesis

96
Q

Characteristics of myelodysplastic syndrome?

A

Hypercellular bone marrow with megaloblastic hematopoiesis, pancytopenia. Risk of transformation to AML but most die from infection/bleeding before. Blasts

97
Q

Characteristic cell of myelodysplastic syndrome>

A

Pelger Huet (bilobed nucleus)

98
Q

Tx. myelodysplastic syndrome

A

1) Transfusion 2) Erythropoietin 3) Azacitidine or decitabine 4) Lenalidomide (for those with 5q deletion) 5) BMT

99
Q

CLL presentation?

A

Increased WBC, increased lymphocytes, smudge cells in ELDERLY.

100
Q

What is richter phenomenon?

A

Occurs in CLL where conversion to high-grade lymphoma happens in 5% of patients

101
Q

Tx based on staging CLL?

A

Stage 0 (elevated WBC), Stage I (lymphadenopathy), and stage II (hepatosplenomegaly) there is no Treatment! Stage III (anemia) and stage IV (thrombocytopenia) are treated with fludarabine, cyclophosphamide, AND rituximab. If fludarabine fails, Alemtuzumab (anti-CD-52)

102
Q

Tx. hairy cell?

A

Cladirabine or pentostatin

103
Q

Best initial/most accurate hairy cell?

A

Best initial-Smear w/ hairy cells

Most accurate-immunotyping by flow cytometry (CD11c) or TRAP +

104
Q

Best initial test non-hodgkin lymphoma

A

Excisional biopsy

105
Q

Tx. local vs advanced disease non-hodgkin lymphoma>

A

Local (stage 1a and IIa)-radiation and small dose/course chemo
Advanced (stage III/IV and B symptoms)-combination chemo with CHOP (Cyclophosphamide, adriamycin, vincriostine (oncovin), prednisone) and rituximab, antibody against CD20

106
Q

Tx. hodgkin disease?

A

Stage Ia and IIA: local radiation with small dose chemo

Stage III/IV or any B symptoms: ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)

107
Q

Vincristine side effect?

A

Neuropathy

108
Q

Cisplatin side effect?

A

Nephro/ototoxicity

109
Q

Most common presentation/cause of death in multiple myeloma?

A

Presentation-pathologic bone fracture

Cause of death-Infection followed by renal failure

110
Q

Best initial most accurate multiple myeloma?

A

Best initial-x-ray of bone. Most accurate: Plasma cell>10% on bone marrow biopsy in addition to SPEP (spike) and bence jones protein

111
Q

Myeloma treatment?

A

Best initial-combo dexamethasone with lenalidomide, bortezomib, or both
Most effective-BMT

112
Q

von willebrand disease tx?

A

desmopression and use factor VIII or vWF concentrate with no response

113
Q

Factor XI deficiency tx?

A

No bleeding-no therapy

Bleeding-FFP

114
Q

Factor XII deficiency tx?

A

No bleeding so no therapy needed

115
Q

Tx DIC?

A

FFP, cryopercipitate if FFP NOT control bleeding

116
Q

What is only thrombophilia important to test for with first clot?

A

Antiphospholipid syndrome

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