Case Studies in Leukemia and Lymphoma Flashcards

1
Q

What are myelodysplastic syndromes (MDS)?

A

age related (~70 y/o) acquired genetic damage to hematopoietic cells which leads to ineffective erythropoiesis

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2
Q

How does MDS typically present?

A

anemia with macrocytosis

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3
Q

if the peripheral blood smear shows large cells with large nuclei and visible nucleoli, what are you looking at?

A

blasts

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4
Q

what study helps differentiate myeloid blasts from lymphoid blasts?

A

flow cytometry

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5
Q

if lab results show leukocytosis, anemia, and thrombocytopenia, what should you be thinking until proven otherwise?

A

bone marrow problem

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6
Q

how do you calculate an absolute neutrophil count?

A

WBC x (neutrophil % + Band %)

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7
Q

What is a severe neutropenia defined as?

A

an ANC < 500

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8
Q

If a patient has a neutropenic fever, how do you treat them?

A

need to get cultures but then start with empiric antibiotics

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9
Q

How do you treat AML?

A

admission for treatment: induction therapy (treat underlying disease)

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10
Q

What are empiric antibiotics?

A

Empiric coverage is targeted at gram negative organisms first

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11
Q

the first line of empiric antibiotics is used to provide coverage against what?

A

pseudomonas coverage

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12
Q

what are examples of first line empiric antibiotics?

A

cefepime, piperacillin-tazobactam, meropenem, and ceftazidine

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13
Q

Addition of MRSA coverage is often added if there is concern for what?

A

line infection, PNA, or soft tissue infection

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14
Q

what is an example of an antibiotic that offers MRSA coverage?

A

vancomycin

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15
Q

if fever persists after 48 hours after onset of antibiotic therapy, what is typically added?

A

fungal coverage

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16
Q

what are 2 examples of antibiotics that offer fungal coverage?

A

amphotericin B, voriconazole

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17
Q

what confirms the diagnosis of AML?

A

anything greater than 20% blasts in bone marrow

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18
Q

induction chemotherapy for AML is done where?

A

in the hospital- 10-14 day induction course based on AML subtype and genetic profile

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19
Q

What stain do you do to look for fungi?

A

silver stain

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20
Q

How can candida be identified? and how do you treat candida?

A

can be identified with silver stain; treat with voriconazole/ amphotericin B

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21
Q

what is consolidation chemo?

A

when you intensify chemo to make sure no cancer cells are left in the body

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22
Q

what is maintenance chemo?

A

for some cancers, longer term therapy to maintain remission and prevent relapse

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23
Q

What is acute lymphoblastic leukemia?

A

malignancy of T or B lymphoblast

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24
Q

how do you make the diagnosis of ALL?

A

if there is greater than 20% lymphoblasts on bone marrow biopsy

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25
Q

What coagulation studies resemble DIC?

A

increase PT/PTT, increased D-dimer, and decreased fibrinogen

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26
Q

if a peripheral smear shows circulating blasts with auer rods, what do you suspect?

A

acute promyelocytic leukemia

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27
Q

which translocation is consistent with acute promyelocytic leukemia?

A

t(15;17)

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28
Q

What occurs in acute promyelocytic leukemia?

A

the leukemic cells express tissue factor, which activates factor X; high levels of Annexin II receptors on APL cells convert plasminogen into plasmin

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29
Q

what two things are highly expressed by APL cells?

A

Tissue Factor and Annexin II

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30
Q

What are two treatment options for acute promyelocytic leukemia?

A

Immediate treatment!! ATO (arsenic trioxide) and ATRA (all trans retinoic acid)

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31
Q

What is a LAP score?

A

you are looking for alkaline phosphatase and you are staining the leukocytes with this special stain that will show up positive in cells that are reactive

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32
Q

what are reactive cells?

A

ones that are autoimmune, fighting infection, or just reactive to a stressful stimuli

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33
Q

what does a positive LAP score tell you?

A

leukemoid reaction

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34
Q

what does a low LAP score tell you?

A

neoplasm

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35
Q

how does a leukemoid reaction appear in a centrifuged sample?

A

there is only a mild increase in the buffy coat

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36
Q

how does leukemia appear in a centrifuged sample?

A

the buffy layer is huge

37
Q

What is a common other finding present in the majority of patients with CML?

A

splenomegaly

38
Q

What does the bone marrow look like in CML?

A

hypercellular bone marrow consisting of predominant mature granulocytes

39
Q

what translocation is associated with CML?

A

t(9;22)

40
Q

CML is a part of what broader category?

A

myeloproliferative neoplasms

41
Q

besides the hypercellularity, what is another common finding of CML?

A

pseudo-gaucher cells

42
Q

what are pseudo-gaucher cells?

A

macrophages that are cleaning up cellular debris in situations like CML where there is high rate of cell turnover in the marrow

43
Q

what is the result of the t(9;22) translocation that occurs in CML?

A

the protein resulting from this rearranged gene is a “constitutively active” tyrosine kinase

44
Q

What medication is used to treat CML?

A

Imatinib (Gleevec/Glivec)

45
Q

what is imatinib’s mechanism of action?

A

it is a tyrosine kinase inhibitor- it goes in and blocks where that ATP binds

46
Q

What is the gold standard for testing for CML?

A

BCR-ABL PCR

47
Q

what defines successful treatment in CML cases?

A

hematologic response (white count normalizes) and a molecular response (negative BCR-ABL PCR)

48
Q

why is monitoring CML so important?

A

CML can progress to an aggressive disease

49
Q

what are the different phases of CML?

A

chronic phase–> accelerated phase–> blastic phase

50
Q

in CML, what is the high white count predominated by?

A

mature granulocytes

51
Q

What are the causes of lymphadenopathy you should think about?

A

Malignancy, infection, auto-immunity, miscellaneous, iatrogenic

52
Q

when evaluating the lymph nodes, what are the risk factors for malignancy?

A

age older than 40 years, duration has been longer than a month, supraclavicular location

53
Q

what are 3 things that when evaluating the lymph nodes would make you do a biopsy?

A

location was subclavian (aka supraclavicular), it was fixed “hard as a rock”, and the lymph node was greater than 4 cm

54
Q

what can the fine needle aspiration be used for only?

A

non-hodgkin lymphoma

55
Q

what is the gold standard biopsy?

A

excision/excisional biopsy

56
Q

what is the only way to diagnose hodgkin-lymphoma

A

excision/excisional biopsy

57
Q

what is a prime location for lymphoma?

A

around the appendix- there is lots of lymphoid tissue there

58
Q

when the description of a biopsy is “sheet of cells or appears like a starry night/sky” what should you think of?

A

Burkitt Lymphoma

59
Q

what translocation is associated with burkitt lymphoma?

A

t(8;14)

60
Q

what does the t(8;14) involve?

A

MYC; it increases proliferation

61
Q

what is one of the worst lymphoma translocations?

A

t(8;14)

62
Q

Burkitt lymphoma has a high risk of what?

A

CNS lymphoma

63
Q

how do you treat burkitt lymphoma with CNS involvement?

A

chemotherapy with intrathecal administration

64
Q

if a patient is undergoing chemotherapy and then stops producing urine and their BMP reveals significant kidney injury, what should you suspect?

A

Tumor lysis syndrome

65
Q

what is tumor lysis syndrome (and the specifics)?

A

significant electrolyte imbalance’ hyperkalemia, hyperuricemia, hyperphosphatemia, and hypocalcemia

66
Q

How do you treat TLS?

A

aggressive IV fluids- urine output average of 100 cc/hr; treat prophylactically with allopurinol

67
Q

what is allopurinol?

A

it is a xanthine oxidase inhibitor that prevents uric acid formation

68
Q

how do you treat hyperkalemia?

A

elimination of K: furosemide; or temporizing: glucose/insulin, calcium gluconate, and albuterol

69
Q

how do you treat hyperuricemia?

A

with rasburicase

70
Q

what should you know before treating a patient with rasburicase?

A

if the patient has a G6PD deficiency- because rasburicase in those patients can cause hemolytic anemia

71
Q

how do you treat hypocalcemia?

A

calcium gluconate

72
Q

how do Burkitt lymphoma patients present?

A

with a huge mass generally

73
Q

What is hodgkin lymphoma?

A

it is a neoplastic lymphoid proliferation with scattered B-derived reed-sternberg cells in an abundant backgroun of T lymphocytes, eosinophils, and other inflammatory cells- it is a VERY inflammatory state

74
Q

what do Reed-Sternberg cells look like and what are they associated with?

A

they look like owl eyes; they are associated with hodgkin lymphoma

75
Q

How is HL typically treated?

A

chemotherapy is very effective; typically 21 or 28 day cycle of chemo therapy

76
Q

Hodgkin lymphoma typically present in 3 ways. What are they?

A

lymphadenopathy (but not always), B symptoms, incidentally found on CXR

77
Q

If a peripheral blood smear shows 90% small lymphocytes and then flow cytometry shows monoclonal B cells, what should you diagnose it as?

A

CLL/SLL

78
Q

how does CLL typically present?

A

it is usually asymptomatic- but they show LOTS of lymphocytes on their CBC

79
Q

how do you diagnose CLL?

A

through peripheral blood- no need to biopsy the lymph node

80
Q

if a patient has a history of CLL and then new symptoms present such as anemia and thrombocytopenia, what should you suspect?

A

richter’s transformation

81
Q

what is richter’s transformation?

A

you get a low grade lymphoma like CLL that progresses to a high grade lymphoma, which are deadly

82
Q

what is an example of a high grade lymphoma?

A

DLBCL

83
Q

What are the symptoms of richter’s transformation?

A

increase fatigue and B symptoms

84
Q

what is the treatment for richter’s transformation?

A

chemotherapy and RITUXIMAB

85
Q

what is retuximab?

A

a monoclonal antibody- specifically a CD20 antibody

86
Q

how does rituximab work?

A

it is an antibody against CD-20 cell receptors, and most of your lymphocytes have CD20 markers on them

87
Q

Burkitt lymphoma, Hodgkin lymphoma, and CLL are all diagnosed in different ways- how is burkitt lymphoma diagnosed?

A

mass effect

88
Q

Burkitt lymphoma, Hodgkin lymphoma, and CLL are all diagnosed in different ways-how is hodgkin lymphoma diagnosed?

A

constitutional symptoms (B symptoms)

89
Q

Burkitt lymphoma, Hodgkin lymphoma, and CLL are all diagnosed in different ways- how is CLL diagnosed?

A

peripheral blood abnormalities