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Flashcards in Hem/onc Tx And Tests Deck (93):
0

How do you diagnose a bleeding disorder?

Get PT, PTT, TT, fibrinogen and bleeding time
Conduct a mixing study
Obtain factor assays

1

What is the tx for mild hemophilia?

DdAVP

2

What is the tx for bleeding episodes due to hemophilia?

Transfusion of clotting factors or cryoprecipitate to at least 40% normal concentration

3

How do you diagnose vWF disease?

Labs : prolonged PTT and bleeding time
Ristocetin cofactor assay is decreased

4

How do you treat vWF?

DDAVP
Tx menorrhagia with OCPs

5

What are diagnostic criteria for diagnosing a patient with a hereditary hypercoaguable state?

Must have 2 abnormal values while asx and untreated with 2 abnormal values in 2 other family members

6

How do you treat a DVT or PE?

Give heparin or LMWH
3-6 months of warfarin for first event
6-12 months for the second event
Lifelong anticoag if it happens 3 times

7

What is the best means of preventing a DVT or PE in a patient who can't be anticoagulated?

An IVC filter

8

How so you diagnose DIC?

PT, PTT, d-dimer, fibrin split products are increased
Fibrinogen level and ALL clotting factors are decreased

9

What is the tx for DIC.

RBC transfusion
Platelet transfusion
Shock management

10

What is the tx for HIT.

Stop heparin
Start lepirudin or argatroban

11

What is the tx for TTP?

Plasma replacement
Plasmapheresis
Steroids to decreases micro thrombus formation

12

What is CI in TTP?

Platelet transfusion - can make it worse!

13

How would you diagnose ITP?

Bone marrow aspirate would show increased megakaryocytes

14

When do you tx ITP?

When patients have symptomatic bleeding (usually when platelets are below 20,000)

15

What is the tx of sx ITP?

Corticosteroids
High dose gamma globulin
Splenectomy (consider if cannot be tapered off of corticosteroids after 3-6 months)

16

What are the second line therapies for ITP?

Rituximab
Anti-D immunoglobulin

17

What test distinguishes between an absorption problem and a dietary deficiency of B12?

Schilling test
Give B12 IM and then give radiolabelled B12 - if shows up in the urine the problem is a dietary deficiency

18

What lab values are consistent with hemolytic anemia?

High LDH, elevated indirect bilirubin and decreased haptoglobin levels
High reticule yet count

19

What is used to detect autoimmune hemolytic anemia?

A Coomb's test

20

How do you diagnose aplastic anemia?

CBC and clinical
Bone marrow bx : hypocellularity and fat

21

What is the tx of aplastic anemia?

Blood transfusion
Stem cell transplantation
Cyclosporine A and antithymocyte globulin for immune suppression and to prevent autoimmune destruction of the marrow

22

What is the gold standard for diagnosing sickle cell?

Hemoglobin electrophoresis

23

How else is sickle cell diagnosed?

Peripheral blood smear

24

What is the tx for hereditary spherocytosis?

Splenectomy

25

How do you diagnose hereditary spherocytosis?

CBC shows low MCV and high MHCH And high RDW
Osmotic fragility test= pos
See sphere RBCS and Howell-jolly bodies on peripheral smear

26

How do you diagnose G6PD?

Do enzyme assay right after an episode
Peripheral smear shows : Heinz bodies and bite cells

27

What is the tx for PNH?

Eculizumab

28

What is tx for autoimmune hemolytic anemia?

IVIG
Splenectomy
Steroids

29

What is the tx of SCD?

Hydroxyurea
Chronic folate supplement
Pneumococcal vaccination
And penicillin BID when patient is less than 5 (MCC of death in children is pneumococcal sepsis)

30

What is the tx of veno-occlusive crises in SCD?

Oxygen
IVF
Pain control
Antibiotics if infection was the trigger

31

How do you keep VOC from progressing to acute chest syndrome?

IVF and incentive spirometry
Keep sickling to less than 40% with simple transfusion or exchange transfusion in ICU

32

What is the chronic tx for SCD when the fail hydroxurea therapy?

Chronic transfusions

33

How are thalassemia s diagnosed?

Hemoglobin electrophoresis
DNA studies

34

How do you treat B-thal major?

Chronic transfusion
BM transplant
Need deferoxamine too to prevent iron overload

35

How do you great hemoglobin H disease?

Chronic blood transfusion + iron chelation

36

How do you diagnose PCV?

CBC with ABGs
Levels of EPO
JAK2 mutation test

37

What is the tx of PCV?

Phlebotomy, keep Hct under 45%
ASA (because prone to thrombotic events)
Hydroxyurea or INF (cytoreductive drugs)

38

How do you diagnose porphyria?

Elevated blood, urine and stool porphyrins
Enzyme assays

39

What is the tx of porphyria?

Heme
Glucose (to decreased heme synthesis)

INF alpha if have Hep C, hydroxychloroquine, phlebotomy

40

How do you diagnose leukemia?

Greater than 20-30% Blasts on smear
Bone marrow aspirate

41

How do you treat AML and ALL?

Chemo
Antibiotics
Transfusion
Colony-stimulating factor
Bone marrow transplant

42

What should be started to prevent tumor lysis syndrome?

Allopurinol

43

How do you treat leukostasis syndrome?

Hydroxurea and leukapheresis (to decrease WBC)

44

How do you diagnose CLL?

Flow cytometry demonstrating the presence of CD5, 20 and 21
CBC shows lymphocytosis ( greater than 5,000) and smudge cells

45

When do you treat CLL?

When the patient becomes symptomatic

46

How do you diagnose CML.

CBC shows high WBC (over 100,000)
Philadelphia chromosome

47

What will the labs show with CML?

LAP is low
LDH, uric acid and B12 are elevated

48

How do you treat the chronic phase of CML?

Imatinib
If young, allogeneic stem cell transplant

49

How do you treat the blast phase of CML?

Imatinib
Dasatinib + hematopoietic stem cell transplant
Clinical trial

50

How do you diagnose hairy cell leukemia?

Bone marrow bx or smear shows hairy cells
TRAP stain

51

What is the first line tx for hairy cell leukemia?

Nucleoside analogs ->> cladribine = adenosine analog (toxic to neoplastic cells)

52

What are the other tx options for hairy cell leukemia?

Splenectomy
INF alpha

53

How do you diagnose lymphoma?

Excisional bx of a lymph node AND extra nodal site

54

What should be done in patients with HIV if you suspect lymphoma?

CSF exam

55

How do you diagnose mycoses fungoides?

Clincal
Histology of skin lesion
EM showing sezary cells

56

What is the mainstay of tx of mycoses fungoides?

Phototherapy

57

What can be used for more severe or extensive mycoses fungoides?

Radiotherapy

58

How do you diagnose Hodgkin's lymphoma?

Excisional lymph node biopsy

59

What are the diagnostic criteria for multiple myeloma?

Greater than 10% plasma cells in the bone marrow and/or histo proven plasma cell infiltration
M Protein in the serum or urine
Lytic bone lesions

60

How do you evaluate a patient for multiple myeloma?

Skeletal survey
Bone marrow bx
Serum and urine protein electrophoresis
CBC

61

What is used in the initial tx of multiple myeloma?

Melphalan (alkylating agent)
Prednisone

62

How do you diagnose Waldenstrom's macroglobulinemia?

Bone marrow bx and aspirate
Shows Dutcher bodies (PAS+ IgM depositis around the nucleus)

Serum and urine protein electrophoresis and immunofixation

63

What is the tx for Waldenstrom's macroglobulinemia?

Plasmapheresis
Chemo

64

How do you confirm the dx of amyloidosis?

Bx with Congo red stain

65

What is the tx of amyloidosis?

Experimental chemo
Transplant

66

How do you diagnose neutropenia?

CBC with ANC

67

What do you do if there is associated anemia or TCP with neutropenia?

Bone marrow bx and aspirate

68

What is the best next step if patient with neutropenia has a fever?

Give broad spectrum antibiotics (cefepime)

69

What can be used to shorten the duration of neutropenia?

G-CSF

70

How do you diagnose eosinophilia?

H&P
CBC with diff
CSF analysis

71

What should you think of if have hematuria and eosinophilia?

Schistosomiasis

72

How do you treat HSP

Corticosteroids
Cytotoxic agents

73

What is tx for tumor lysis syndrome?

IVF
Allopurinol
Rasbaricase

74

How do you confirm HIT?

ELISA antibody test

75

How do you work up ITP?

Physical
CBC
Peripheral blood smear

76

How can you diagnose porphyria?

Aminolevunlimic acid and porphobilinogen in urine

77

When should gonadectomy be performed in androgen insensitivity?

After puberty

78

What is the tx for ITP in children with skin manifestations only?

Observation

79

What is the tx for ITP in children with bleeding?

IVIG or corticosteroids

80

What is the tx for ITP in adults with platelet count less than 30,000 without bleeding?

Obs

81

What is the tx for ITP in adults with bleeding or platelet count less than 30,000?

IVIG
Glucocorticoids

82

What is the test for early detection of hemophilia arthropathy?

MRI

83

What is the prophylaxis for hemophilia arthropathy?

Factor XIII or IX

84

What is the gold standard lab test for hereditary spherocytosis?

Eosin-5-maleimide binding test with the Acidified glycerol lysis test

85

How do you diagnose amyloidosis?

Tissue bx

86

What is the treatment for TTP?

Emergent Plasma exchange

87

What is cryoprecipitate used for?

Von willebrand disease
Factor 8 deficiency
Fibrinogen def

88

When is a platelet transfusion given?

Platelets less than 10,000
Bleeding and platelets less than 50,000

89

What is the tx for primary CNS lymphoma?

Brain irradiation

90

What is the tx for neurocysticercosis?

Albendazole

91

How long is fluconazole administered for HIV patients for cryptococcal meningitis?

Until CD4 is greater than 100 for a year

92

How long should antiretroviral therapy be deferred for after tx if cryptococcal meningitis?

2-8 weeks