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Step 2 High Yield > Hem/onc Tx And Tests > Flashcards

Hem/onc Tx And Tests Flashcards

0
Q

What is the tx for mild hemophilia?

A

DdAVP

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1
Q

How do you diagnose a bleeding disorder?

A

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

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2
Q

What is the tx for bleeding episodes due to hemophilia?

A

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

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3
Q

How do you diagnose vWF disease?

A

Labs : prolonged PTT and bleeding time

Ristocetin cofactor assay is decreased

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4
Q

How do you treat vWF?

A

DDAVP

Tx menorrhagia with OCPs

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5
Q

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

A

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

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6
Q

How do you treat a DVT or PE?

A

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

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7
Q

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

A

An IVC filter

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8
Q

How so you diagnose DIC?

A

PT, PTT, d-dimer, fibrin split products are increased

Fibrinogen level and ALL clotting factors are decreased

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9
Q

What is the tx for DIC.

A

RBC transfusion
Platelet transfusion
Shock management

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10
Q

What is the tx for HIT.

A

Stop heparin

Start lepirudin or argatroban

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11
Q

What is the tx for TTP?

A

Plasma replacement
Plasmapheresis
Steroids to decreases micro thrombus formation

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12
Q

What is CI in TTP?

A

Platelet transfusion - can make it worse!

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13
Q

How would you diagnose ITP?

A

Bone marrow aspirate would show increased megakaryocytes

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14
Q

When do you tx ITP?

A

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

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15
Q

What is the tx of sx ITP?

A

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

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16
Q

What are the second line therapies for ITP?

A

Rituximab

Anti-D immunoglobulin

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17
Q

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

A

Schilling test

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

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18
Q

What lab values are consistent with hemolytic anemia?

A

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

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19
Q

What is used to detect autoimmune hemolytic anemia?

A

A Coomb’s test

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20
Q

How do you diagnose aplastic anemia?

A

CBC and clinical

Bone marrow bx : hypocellularity and fat

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21
Q

What is the tx of aplastic anemia?

A

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

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22
Q

What is the gold standard for diagnosing sickle cell?

A

Hemoglobin electrophoresis

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23
Q

How else is sickle cell diagnosed?

A

Peripheral blood smear

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24
Q

What is the tx for hereditary spherocytosis?

A

Splenectomy

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25
Q

How do you diagnose hereditary spherocytosis?

A

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

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26
Q

How do you diagnose G6PD?

A

Do enzyme assay right after an episode

Peripheral smear shows : Heinz bodies and bite cells

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27
Q

What is the tx for PNH?

A

Eculizumab

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28
Q

What is tx for autoimmune hemolytic anemia?

A

IVIG
Splenectomy
Steroids

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29
Q

What is the tx of SCD?

A

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

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30
Q

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

A

Oxygen
IVF
Pain control
Antibiotics if infection was the trigger

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31
Q

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

A

IVF and incentive spirometry

Keep sickling to less than 40% with simple transfusion or exchange transfusion in ICU

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32
Q

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

A

Chronic transfusions

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33
Q

How are thalassemia s diagnosed?

A

Hemoglobin electrophoresis

DNA studies

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34
Q

How do you treat B-thal major?

A

Chronic transfusion
BM transplant
Need deferoxamine too to prevent iron overload

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35
Q

How do you great hemoglobin H disease?

A

Chronic blood transfusion + iron chelation

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36
Q

How do you diagnose PCV?

A

CBC with ABGs
Levels of EPO
JAK2 mutation test

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37
Q

What is the tx of PCV?

A

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

38
Q

How do you diagnose porphyria?

A

Elevated blood, urine and stool porphyrins

Enzyme assays

39
Q

What is the tx of porphyria?

A

Heme
Glucose (to decreased heme synthesis)

INF alpha if have Hep C, hydroxychloroquine, phlebotomy

40
Q

How do you diagnose leukemia?

A

Greater than 20-30% Blasts on smear

Bone marrow aspirate

41
Q

How do you treat AML and ALL?

A 
Chemo 
Antibiotics
Transfusion
Colony-stimulating factor 
Bone marrow transplant
42
Q

What should be started to prevent tumor lysis syndrome?

A

Allopurinol

43
Q

How do you treat leukostasis syndrome?

A

Hydroxurea and leukapheresis (to decrease WBC)

44
Q

How do you diagnose CLL?

A

Flow cytometry demonstrating the presence of CD5, 20 and 21

CBC shows lymphocytosis ( greater than 5,000) and smudge cells

45
Q

When do you treat CLL?

A

When the patient becomes symptomatic

46
Q

How do you diagnose CML.

A

CBC shows high WBC (over 100,000)

Philadelphia chromosome

47
Q

What will the labs show with CML?

A

LAP is low

LDH, uric acid and B12 are elevated

48
Q

How do you treat the chronic phase of CML?

A

Imatinib

If young, allogeneic stem cell transplant

49
Q

How do you treat the blast phase of CML?

A

Imatinib
Dasatinib + hematopoietic stem cell transplant
Clinical trial

50
Q

How do you diagnose hairy cell leukemia?

A

Bone marrow bx or smear shows hairy cells

TRAP stain

51
Q

What is the first line tx for hairy cell leukemia?

A

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

52
Q

What are the other tx options for hairy cell leukemia?

A

Splenectomy

INF alpha

53
Q

How do you diagnose lymphoma?

A

Excisional bx of a lymph node AND extra nodal site

54
Q

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

A

CSF exam

55
Q

How do you diagnose mycoses fungoides?

A

Clincal
Histology of skin lesion
EM showing sezary cells

56
Q

What is the mainstay of tx of mycoses fungoides?

A

Phototherapy

57
Q

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

A

Radiotherapy

58
Q

How do you diagnose Hodgkin’s lymphoma?

A

Excisional lymph node biopsy

59
Q

What are the diagnostic criteria for multiple myeloma?

A

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
Q

How do you evaluate a patient for multiple myeloma?

A

Skeletal survey
Bone marrow bx
Serum and urine protein electrophoresis
CBC

61
Q

What is used in the initial tx of multiple myeloma?

A

Melphalan (alkylating agent)

Prednisone

62
Q

How do you diagnose Waldenstrom’s macroglobulinemia?

A

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

Serum and urine protein electrophoresis and immunofixation

63
Q

What is the tx for Waldenstrom’s macroglobulinemia?

A

Plasmapheresis

Chemo

64
Q

How do you confirm the dx of amyloidosis?

A

Bx with Congo red stain

65
Q

What is the tx of amyloidosis?

A

Experimental chemo

Transplant

66
Q

How do you diagnose neutropenia?

A

CBC with ANC

67
Q

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

A

Bone marrow bx and aspirate

68
Q

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

A

Give broad spectrum antibiotics (cefepime)

69
Q

What can be used to shorten the duration of neutropenia?

A

G-CSF

70
Q

How do you diagnose eosinophilia?

A

H&P
CBC with diff
CSF analysis

71
Q

What should you think of if have hematuria and eosinophilia?

A

Schistosomiasis

72
Q

How do you treat HSP

A

Corticosteroids

Cytotoxic agents

73
Q

What is tx for tumor lysis syndrome?

A

IVF
Allopurinol
Rasbaricase

74
Q

How do you confirm HIT?

A

ELISA antibody test

75
Q

How do you work up ITP?

A

Physical
CBC
Peripheral blood smear

76
Q

How can you diagnose porphyria?

A

Aminolevunlimic acid and porphobilinogen in urine

77
Q

When should gonadectomy be performed in androgen insensitivity?

A

After puberty

78
Q

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

A

Observation

79
Q

What is the tx for ITP in children with bleeding?

A

IVIG or corticosteroids

80
Q

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

A

Obs

81
Q

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

A

IVIG

Glucocorticoids

82
Q

What is the test for early detection of hemophilia arthropathy?

A

MRI

83
Q

What is the prophylaxis for hemophilia arthropathy?

A

Factor XIII or IX

84
Q

What is the gold standard lab test for hereditary spherocytosis?

A

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

85
Q

How do you diagnose amyloidosis?

A

Tissue bx

86
Q

What is the treatment for TTP?

A

Emergent Plasma exchange

87
Q

What is cryoprecipitate used for?

A

Von willebrand disease
Factor 8 deficiency
Fibrinogen def

88
Q

When is a platelet transfusion given?

A

Platelets less than 10,000

Bleeding and platelets less than 50,000

89
Q

What is the tx for primary CNS lymphoma?

A

Brain irradiation

90
Q

What is the tx for neurocysticercosis?

A

Albendazole

91
Q

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

A

Until CD4 is greater than 100 for a year

92
Q

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

A

2-8 weeks

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