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Flashcards in Hem Onc Deck (116):
1

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

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

2

Triad of symptoms in lead poisoning?

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

3

Tx. and complications of hereditary spherocytosis

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

4

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

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

5

Lab findings PNH?

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

6

Tx. PNH?

-Prednisone
-Iron/folate supplementation
-Eculizumab (monoconal Ab inhibiting complement activation)

7

HUS findings? (4)

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

8

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

CT scan abdomen/pelvis/chest

9

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

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

10

Lymphadenopathy (benign vs malignant) characteristics

benign-2 cm, firm, solitary

11

Androgen abuse reproductive, CV, psychiatric and hematologic consequences

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

12

MGUS vs Multiple Myeloma

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

13

ITP lab finding/peripheral smear?

Lab finding: isolated thrombocytopenia, megakaryocytes on peripheral smear

14

ITP treatment children

Skin manifestations only: observe; bleeding: IVIG/glucocorticoids

15

ITP treatment adults

>30K without bleeding: Observe;

16

Intravascular vs Extravascular hemolysis pathogenesis

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

17

Tx. AIHA (warm agglutinin vs. cold)

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

18

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

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

19

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

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

20

What is primary hypotension reaction and where is it seen?

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

21

Triad of osler weber rendu?

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

22

Tx. of cancer related anorexia/cachexia syndrome?

Progesterone analogs (megestrol acetate and medroxyprogesterone acetate) and corticosteroids

23

Which 3 patient populations need irradiation to RBC before transfusion?

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

24

Which 4 patient populations need leukoreduction before RBC transfusion?

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

25

Which 3 populations need washing before RBC transfusion?

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

26

Anaphylaxis symptoms, timeframe, cause?

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

27

Febrile acute hemolytic symptoms, timeframe, cause?

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

28

Febrile nonhemolytic symptoms, timeframe, cause?

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

29

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

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

30

Allergic rxn blood transfusion symptoms, timeframe, cause?

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

31

Delayed hemolytic symptoms, timeframe, cause?

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

32

Gold standard confirmatory test HIT?

Serotonin release assay

33

Treatment HIT?

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

34

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

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)

35

Initial drug for hypercalcemia of malignancy depending on calcium level?

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

36

Anemia cause best initial test?

CBC

37

Anemia cause next best test?

Peripheral blood smear

38

Echinocytes or burr cells on smear etiology?

Uremia

39

Where is folate deficiency commonly seen?

Alcoholics and pregnant women

40

MCC B12 deficiency

Pernicious anemia

41

Confirmation of B12 deficiency

Serum B12

42

Tx of Bthal major or minor?

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

43

4 commonly tested causes of autoimmune hemolytic anemia?

-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)

44

When should screening be performed for children with lead poisoning?

6 months in children with risk factors

45

Tx. lead poisoning

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

46

Tx for sideroblastic anemia

supportive/pyridoxine

47

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

-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

MCC blood transfusion rxn

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

Side effect large transfusions?

Bleeding diathesis from dilutional thrombocytopenia and citrate as calcium chelator

50

Best initial therapy/ Long term therapy for waldenstrom macroglobulinemia?

Best initial-plasmapheresis
Long term-rituximab or prednisone or cyclophosphamide

51

Tx. DIC?

Treat underlying condition followed by FFP

52

TTP pathogenesis?

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

53

TTP symptoms?

Fever, thrombocytopenia, hemolytic anemia, neurologic sequale

54

TTP treatment?

Plasmapheresis, steroids. NO PLATELET b/c WORSE DISEASE

55

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

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

56

Expected symptoms with certain hematocrit
>30-35, 25-30, 20-25,

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

57

Reticulocyte count in microcytic anemia? What is exception?

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

58

Reticulocyte count in macrocytic anemia?

Low

59

When is reticulocyte count high?

Blood loss and hemolysis

60

Indicate to transfuse (PRBC) for anemia?

Symptomatic, elder patient with heart disease and low hematocrit (

61

Indication for FFP?

DIC, warfarin reversal, cirrhosis

62

MCC sideroblastic anemia?

Alcohol-->bone marrow suppression

63

Best initital test microcytic?

Iron studies

64

Only form of microcytic anemia where circulating iron is elevated?

Sideroblastic

65

Most accurate test sideroblastic?

Prussian blue

66

Most accurate test iron deficiency?

Bone marrow biopsy for sustained iron

67

Most accurate test thalassemia and findings?

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

Microcytic Anemia with ESRD routinely responds to what?

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

69

Where is B12 absorbed?

terminal ileum

70

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

Both-B12, One-Folate

71

Complication of B12 or folate replacement?

Hypokalemia

72

Best initial/most accurate test sickle cell?

Peripheral smear/Hb electrophoresis

73

Tx. sickle cell

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

74

When is exchange transfusion indicated for severe vasoocclusive crisis?

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

75

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

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

76

Most accurate test AIHA warm vs. cold?

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

77

Best initial/most accurate G6PD?

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

78

HUS vs. TTP associations for cause?

HUS-E coli O157:H7
TTP-ticlodipine, clopidogrel, cyclosporine, AIDS, SLE

79

Presentation and etiology aplastic anemia?

Anemia, infection, thrombocytopenia (pancytopenia) of unclear etiology

80

Definitive tx aplastic anemia?

50: Antithymocyte globulin (ATG) and cyclosporine/tacrolimus

81

Why pruritis in PC vera?

Histamine release from increased basophils

82

Most accurate test PC vera?

JAK2 mutation (V617F)

83

Tx. PC vera?

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

84

What drug inhibits JAK2?

Ruxolitinib

85

Essential thrombocytosis vs reactive thrombocytosis?

Essential greater than 1 million, Reactive 500-700k

86

Essential thrombocytosis tx.?

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

87

Tx for myelofibrosis?

55: Thalidomide/lenalidomide (increase bone marrow production)

88

Best initial/most accurate acute leukemia?

-PBC showing blasts best initial, flow cytometry most accurate

89

Most common presentation AML (M3 type)?

DIC

90

Best initial/most accurate acute leukemia?

Best initial-PBC, most accurate-flow cytometry

91

Translocation AML and ALL?

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

92

Tx. AML/ALL?

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

Best initial/Most accurate test CML

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

94

Tx. CML?

Tyrosine kinase inhibitors (imatinib) best initial and BMT curative

95

WHO CML phases?

Chronic -20$ blasts with extramedullary hematopoiesis

96

Characteristics of myelodysplastic syndrome?

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

97

Characteristic cell of myelodysplastic syndrome>

Pelger Huet (bilobed nucleus)

98

Tx. myelodysplastic syndrome

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

99

CLL presentation?

Increased WBC, increased lymphocytes, smudge cells in ELDERLY.

100

What is richter phenomenon?

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

101

Tx based on staging CLL?

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

Tx. hairy cell?

Cladirabine or pentostatin

103

Best initial/most accurate hairy cell?

Best initial-Smear w/ hairy cells
Most accurate-immunotyping by flow cytometry (CD11c) or TRAP +

104

Best initial test non-hodgkin lymphoma

Excisional biopsy

105

Tx. local vs advanced disease non-hodgkin lymphoma>

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

Tx. hodgkin disease?

Stage Ia and IIA: local radiation with small dose chemo
Stage III/IV or any B symptoms: ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)

107

Vincristine side effect?

Neuropathy

108

Cisplatin side effect?

Nephro/ototoxicity

109

Most common presentation/cause of death in multiple myeloma?

Presentation-pathologic bone fracture
Cause of death-Infection followed by renal failure

110

Best initial most accurate multiple myeloma?

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

Myeloma treatment?

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

112

von willebrand disease tx?

desmopression and use factor VIII or vWF concentrate with no response

113

Factor XI deficiency tx?

No bleeding-no therapy
Bleeding-FFP

114

Factor XII deficiency tx?

No bleeding so no therapy needed

115

Tx DIC?

FFP, cryopercipitate if FFP NOT control bleeding

116

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

Antiphospholipid syndrome