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Hem/Onc > Leukocyte Disorder- Part II- Exam 2 > Flashcards

Leukocyte Disorder- Part II- Exam 2 Flashcards

1
Q

What are some extranodal lymph tissue sites?

A

skin
GI tract, liver
bone marrow
testicles

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2
Q

What are normal lymph nodes sizes in children for the anterior cervical, axillary and inguinal nodes? What is it for adults?

A

anterior cervical ≤ 2 cm
axillary ≤ 1 cm
inguinal nodes ≤ 1.5 cm

normal for adults is 1cm

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3
Q

A patient presents with enlarged lymph nodes, what questions should you ask?

A

cat scratch, undercooked meat, tick bite, travel to endemic area, high risk behavior

constitutional symptoms

any adverse medication reactions

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4
Q

What size does it need to be in order to be considered large in an adult?

A

Size (ex:1x1.5 cm)

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5
Q

What does the consistency of a lymph node tell you, hard nodes?

A

fibrotic cancers

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6
Q

What does the consistency of a lymph node tell you, firm rubbery nodes?

A

lymphomas, chronic leukemia

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7
Q

What does the consistency of a lymph node tell you, softer nodes?

A

acute leukemia, inflammation

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8
Q

What does a tender lymph node tell you? no tenderness?

A

Tenderness = acute rapid enlargement = indicative of inflammatory process

No tenderness = more indicative of malignancy

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9
Q

In terms of fixation a normal node will be _____, a malignant node will be ______

A

normal= mobile

fixed to skin, or unable to move= malignancy or inflammation of surrounding tissues

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10
Q

_____ when nodes become fixed to each other

A

“matted”

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11
Q

What should you prescribe a child with lymphadenopathy only and with a HIGH MRSA risk? What happens if they also have a cat scratch/bite?

A

clindamycin

add azithromycin

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12
Q

What should you prescribe a child with lymphadenopathy only and with a low MRSA risk? What happens if they also have a cat scratch/bite?

A

cephalexin or amoxicillin-clavulanate

add azithromycin

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13
Q

What should you do for lymphadenopathy alone in adults or those with constitutional symptoms?

A

work up to r/o malignancy
referral for lymph node biopsy

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14
Q

_____ A malignant overgrowth of the lymphocyte or its precursor within the lymphatic tissue. What is the MC site?

A

Non-Hodgkin Lymphoma

lymph nodes

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15
Q

What is the pathophys behind Non-Hodgkin Lymphoma? Which cells are MC affected?

A

a monoclonal proliferation of lymphocytic cells due to chromosomal translocation

MC is B-cell - 85%
T/NK cell - 15%

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16
Q

What are some risks factors for NHL?

A

EBV (Mono), Hepatitis B/C, Helicobacter pylori, Kaposi sarcoma-associated herpesvirus, chemicals, chemotherapy, radiation exposure, AIDS, iatrogenic immunosuppression, congenital immunodeficiency disorders, autoimmune disorders

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17
Q

_____ is the MC type of lymphoma. What is the average age of onset?

A

NHL

average age of onset is 50-60

white, male

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18
Q

What are the two types of clinical presentation of NHL?

A

indolent: slow growing

aggressive: rapid growth and spread

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19
Q

What is the classic presentation of indolent NHL?

A

painless and slow growing lymphadenopathy, can be isolated or generalized, spontaneous regression is possible

HSM

cytopenias

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20
Q

What is the classic presentation of aggressive NHL?

A

Fast growing painless lymphadenopathy that
compresses on surrounding structures: lungs, superior vena cava (syndrome)¹, bowel, ureters

weight loss, fever, drenching night sweats

HSM, abdominal/testicular mass

symptoms that it has spread to other areas (GI, vertebrae, bone marrow, CNS)

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21
Q

What does the CBC of a NHL pt look like?

A

usually remains normal until bone marrow infiltration, which results in pancytopenia

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22
Q

What does the peripheral smear of a NHL pt look like?

A

confirms CBC with no morphologic changes

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23
Q

Why would you order a viral serology screening on a NHL pt?

A

Screening for HIV, HCV, HBV

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24
Q

When you order a CXR on a NHL pt, what are you looking for?

A

mediastinal nodes/mass

if present then need to order a CT with contrast

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25
Q

**What is the definitive way to dx NHL?

A

Excisional lymph node bx that is (+) presence of monoclonal lymphocytes

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26
Q

What is the indication for a lymph node bx?

A

suspicious lymph node > 2.25 cm² or 2 cm in a single diameter

peripheral node is preferred

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27
Q

When is a bone marrow bx used in NHL?

A

utilized for staging
bilateral bx needed due to patchy involvement

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28
Q

____ is used in NHL to assess the extent of the disease

A

PET scan

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29
Q

What is the Ann Arbor staging system? What are requirements? What does A and B mean?

A

determines treatment and prognosis for NHL

PET/CT of the neck, chest, abdomen and pelvis in addition to bilateral bone marrow aspiration/biopsy

A - no systemic symptoms
B - presence of “B-symptoms”

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30
Q

Describe the entire Ann Arbor staging system. What does ESPHM all stand for?

A

Stage I - A single lymph node area or single extranodal site
Stage II - Two or more lymph node areas on the same side of the diaphragm
Stage III - Lymph node areas on both sides of the diaphragm
Stage IV - Disseminated or multiple extranodal organs involved

A - no systemic symptoms
B - presence of “B-symptoms”

E = Extralymphatic site
S = Splenic involvement
P = Pulmonary involvement
H = Hepatic involvement
M = Marrow involvement

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31
Q

What is the treatment for indolent NHL? What is the average survival?

A

often disseminated at time of diagnosis - incurable

treatment has not shown to increase overall survival

treatment is only recommended if symptomatic: single or multidrug chemotherapy

10-15 years after NHL dx

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32
Q

What is the treatment for agressive NHL? What is the prognosis?

A

chemotherapy +/- local radiation therapy
allogeneic stem cell transplant

Based on prognostic factors: Poor prognostic factors:
age > 60
↑ LDH
poor response to standard therapy
Ann Arbor stage III-IV

0-1 factor - 75% 5 year survival rate
2-3 factors – 50% 5 year survival rate
4 -5 factors – 25% 5 year survival rate

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33
Q

______ a malignancy of the B-lymphocytes within lymph tissue characterized by the presence of Reed-Sternberg cells

A

hodgkin lymphoma

34
Q

hodgkin lymphoma is a malignancy of the ____. What special cells show up on bx?

A

B cell line

Reed-Sternberg cells: they are mulitnucleated

35
Q

Hodgkin lymphoma peaks in _____ and ______. What gender? What race?

A

20’s and 50’s

more common in men, caucasian and black equal chance

36
Q

What is the classic presentation of HL?

A

painless mass (lymph node) MC in the neck

B symptoms in 40% of patients

10-15% have pruritus

Can have HSM and mediastinal masses but not always

37
Q

What is a specific but infrequent finding with HL?

A

pain after ETOH consumption¹

38
Q

In HL with bulk disease, the LDH will often be ____

A

elevated

39
Q

**What does “bulk disease” refer to? What is it associated with?

A

“Bulk” refers to a lymph node > 10 cm or mediastinal mass > ⅓ thoracic diameter

Hodgkin Lymphoma

40
Q

What confirms the dx of HL?

A

Lymph node excisional biopsy
confirms diagnosis with (+) Reed-Sternberg cells

41
Q

Define what is stage I-II HL. What is the treatment? What is the prognosis?

A

Same Ann Arbor staging as NHL

Stage I-II (with no bulky masses): multi-drug chemotherapy +/- field radiation therapy
-10 yr survival rate of 90%

Stage III-IV or bulky stage II: multi-drug chemotherapy
-10 yr survival rate of 50-60%

42
Q

What is the treatment for relapse after initial treatment for HL?

A

high dose chemotherapy and autologous stem cell transplant

43
Q

What are poor prognostic risk factors for HL?

A

Low serum albumin < 4 g/dL
Low hemoglobin < 10.5 g/dL
Male sex
> 45 y/o
Stage IV disease
High WBC > 15,000/mm3
Low ALC count < 600/mm 3, less than 8% of the total WBC count, or both

44
Q

_____ an acquired disorder resulting in overproduction of all 3 hematopoietic cell lines. What is it a result from?

A

Polycythemia Vera

results from a mutation on the Janus kinase 2 (JAK2) gene¹ leading to excessive cell growth and division

45
Q

What are 2 suggested risk factors for polycythemia vera?

A

ionizing radiation and toxins

46
Q

What gender and race preference does polycythemia vera have? What age range?

A

no gender or race predilection

50-70 years old

47
Q

Fatigue, HA, dizziness, vertigo, tinnitus, visual disturbances, chest pain, intermittent claudication

Generalized pruritus worse after warm shower/bath, some flushing present

epistaxis, bleeding gums, ecchymosis, GI bleeding

Venous thrombosis/thromboembolism/stroke

Abdominal pain/discomfort, early satiety

Engorged conjunctival and retinal vessels

plethora

What am I?

A

Polycythemia Vera

48
Q

Why do polycythemia vera pts have abdominal discomfort?

A

ulcer formation from increased gastric acidity due to excessive histamine

49
Q

What is the hallmark CBC lab finding of polycythemia vera?

A

**HCT: > 54% male and 51% female

elevated RBC (normocytic/normochromic), WBC, Plt

50
Q

What does the peripheral smear look like on a polycythemia vera pt?

A

normal

51
Q

Erythropoietin level on polycythemia vera pt is usually ______

A

low (occasionally normal)

52
Q

What is the way you can confirm the dx of Polycythemia Vera?

A

Genetic testing: (+) JAK2 mutation

53
Q

What is the management of polycythemia vera?

A

therapeutic phlebotomy

1 unit of whole blood (500 ml) removed weekly until hct < 45 %

hydroxyurea

54
Q

1 unit of phlebotomy reduces the hct by _____ (normal-sized adult)

A

3% points

55
Q

What would you NOT want to give to a polycythemia vera pt? What do you want to give?

A

iron supplementation

ASA 81mg daily

56
Q

_____ suppresses bone marrow cellular production by interfering with DNA repair. What is the MC side effect?

A

hydroxyurea

severe bone marrow suppression

57
Q

**What makes a pt low or high risk when talking about polycythemia vera classifications?

A

Low risk: < 60 y/o and no hx of thromboembolism; all others are high risk

58
Q

What is the management for low risk polycythemia vera pts?

A

Therapeutic phlebotomy, 81 mg ASA and life-style modifications (no smoking, control BP, weight, physical activity, COPD, sleep apnea)

59
Q

When would you consider hydroxyurea in a low risk pt?

A

uncontrolled PV-associated symptoms
progressive increase of leukocyte and/or platelet counts
symptomatic or progressive splenomegaly
poor tolerance of phlebotomy

60
Q

What is the treatment for high risk Polycythemia Vera pts?

A

Therapeutic phlebotomy, low dose ASA, life-style modifications and HU

61
Q

Polycythemia Vera median survival is _____. What is the MC cause of death? What are some complications?

A

15 years

thrombosis

conversion to myelofibrosis, CML or rarely AML

62
Q

______ a disorder of increased proliferation of the megakaryocytes (precursor for platelet). What does it result from? What is the average age of dx?

A

Essential Thrombocytosis (ET)

genetic mutations (JAK2, CALR, MPL)

50-60 yrs

63
Q

pain in fingers/toes relieved with ASA

Thrombosis, HA

Transient dizziness, unsteadiness, vertigo, syncope: results from transient ischemia attacks (TIA)

bleeding is less common: seen with plt >1 million/µL, ASA or NSAID use

What am I?

A

essential thrombocytosis

64
Q

What would you expect the CBC of essential thrombocytosis to look like? peripheral blood smear?

A

elevated platelet count may be >2,000,000 /mcL
mild leukocytosis

large platelets

65
Q

What would the bone marrow bx look like on a pt with Essential Thrombocytosis (ET)? What will genetic testing show?

A

increased megakaryocytes

(+) JAK2, CALR or MPL mutation

66
Q

______ determines treatment in essential thrombocytosis?

A

genetic testing

67
Q

**What 3 major factors increase your risk of thrombosis in ET?

A

> 60 y/o
hx of thrombosis
JAK2 mutation

68
Q

What are the risk stratifications for ET?

A
69
Q

What is the management of very low- low risk ET?

A

observation, ASA 81 mg daily
avoid NSAIDS - use increases risk of bleeding

70
Q

What is the management of intermediate- high risk ET?

A

ASA 81 mg + hydroxyurea with a target plt count of 100,000 to 400,000/µL

71
Q

_____ is the MC cause of morbidity/mortality in ET. What can reduce the risk? What is the average survival?

A

thrombosis

risk is reduced with plt count < 500,000/mcL

greater than 15 years with adequate treatment

72
Q

_____ an elevated RBC/Hgb/Hct related to an acquired or congenital disorder

A

secondary erythrocytosis

73
Q

What are 4 etiology factors that contribute towards secondary erythrocytosis?

A

tissue hypoxia

decreased renal perfusion

Inappropriate EPO stimulation

Testosterone administration

74
Q

arterial oxygen

plethora, ruddy skin

HA, lethargy, confusion

clubbing fingers

Splenomegaly less likely:
helps to differentiate secondary erythrocytosis from PV

What am I?

A

secondary erythrocytosis

75
Q

What are very important lab values that points to secondary erythrocytosis?

A

Negative JAK2 gene

**Increased EPO level

76
Q

What will the CBC look like in secondary erythrocytosis?

A

CBC - increased RBC, Hgb, Hct, +/- slight increase in plt

77
Q

______ an elevated platelet count that develops secondary to another disorder

A

Reactive Thrombocytosis

78
Q

____ results from an increased megakaryocyte proliferation/maturation
results from increased production of inflammatory cytokines

A

Reactive Thrombocytosis

79
Q

Reactive Thrombocytosis pathophysiology includes what 3 things?

A

increased megakaryocyte proliferation/maturation

accelerated platelet release

reduced platelet sequestration/turnover

80
Q

What other labs should you also order for reactive thrombocytosis? What are you looking for?

A

Inflammatory condition evaluation:Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Antinuclear antibody (ANA), rheumatoid factor (RF)

looking for causes of the underlying inflammation/autoimmune disorders

81
Q
A

Hem/Onc (14 decks)

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