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

Leukocyte Disorder- Part II- Exam 2 Flashcards

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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
**What is the definitive way to dx NHL?
Excisional lymph node bx that is (+) presence of monoclonal lymphocytes
26
What is the indication for a lymph node bx?
suspicious lymph node > 2.25 cm² or 2 cm in a single diameter peripheral node is preferred
27
When is a bone marrow bx used in NHL?
utilized for staging bilateral bx needed due to patchy involvement
28
____ is used in NHL to assess the extent of the disease
PET scan
29
What is the Ann Arbor staging system? What are requirements? What does A and B mean?
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”
30
Describe the entire Ann Arbor staging system. What does ESPHM all stand for?
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
31
What is the treatment for indolent NHL? What is the average survival?
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
32
What is the treatment for agressive NHL? What is the prognosis?
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
33
______ a malignancy of the B-lymphocytes within lymph tissue characterized by the presence of Reed-Sternberg cells
hodgkin lymphoma
34
hodgkin lymphoma is a malignancy of the ____. What special cells show up on bx?
B cell line Reed-Sternberg cells: they are mulitnucleated
35
Hodgkin lymphoma peaks in _____ and ______. What gender? What race?
20's and 50's more common in men, caucasian and black equal chance
36
What is the classic presentation of HL?
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
What is a specific but infrequent finding with HL?
pain after ETOH consumption¹
38
In HL with bulk disease, the LDH will often be ____
elevated
39
**What does "bulk disease" refer to? What is it associated with?
“Bulk” refers to a lymph node > 10 cm or mediastinal mass > ⅓ thoracic diameter Hodgkin Lymphoma
40
What confirms the dx of HL?
Lymph node excisional biopsy confirms diagnosis with (+) Reed-Sternberg cells
41
Define what is stage I-II HL. What is the treatment? What is the prognosis?
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
What is the treatment for relapse after initial treatment for HL?
high dose chemotherapy and autologous stem cell transplant
43
What are poor prognostic risk factors for HL?
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
_____ an acquired disorder resulting in overproduction of all 3 hematopoietic cell lines. What is it a result from?
Polycythemia Vera results from a mutation on the Janus kinase 2 (JAK2) gene¹ leading to excessive cell growth and division
45
What are 2 suggested risk factors for polycythemia vera?
ionizing radiation and toxins
46
What gender and race preference does polycythemia vera have? What age range?
no gender or race predilection 50-70 years old
47
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?
Polycythemia Vera
48
Why do polycythemia vera pts have abdominal discomfort?
ulcer formation from increased gastric acidity due to excessive histamine
49
What is the hallmark CBC lab finding of polycythemia vera?
**HCT: > 54% male and 51% female elevated RBC (normocytic/normochromic), WBC, Plt
50
What does the peripheral smear look like on a polycythemia vera pt?
normal
51
Erythropoietin level on polycythemia vera pt is usually ______
low (occasionally normal)
52
What is the way you can confirm the dx of Polycythemia Vera?
Genetic testing: (+) JAK2 mutation
53
What is the management of polycythemia vera?
therapeutic phlebotomy 1 unit of whole blood (500 ml) removed weekly until hct < 45 % hydroxyurea
54
1 unit of phlebotomy reduces the hct by _____ (normal-sized adult)
3% points
55
What would you NOT want to give to a polycythemia vera pt? What do you want to give?
iron supplementation ASA 81mg daily
56
_____ suppresses bone marrow cellular production by interfering with DNA repair. What is the MC side effect?
hydroxyurea severe bone marrow suppression
57
**What makes a pt low or high risk when talking about polycythemia vera classifications?
Low risk: < 60 y/o and no hx of thromboembolism; all others are high risk
58
What is the management for low risk polycythemia vera pts?
Therapeutic phlebotomy, 81 mg ASA and life-style modifications (no smoking, control BP, weight, physical activity, COPD, sleep apnea)
59
When would you consider hydroxyurea in a low risk pt?
uncontrolled PV-associated symptoms progressive increase of leukocyte and/or platelet counts symptomatic or progressive splenomegaly poor tolerance of phlebotomy
60
What is the treatment for high risk Polycythemia Vera pts?
Therapeutic phlebotomy, low dose ASA, life-style modifications and HU
61
Polycythemia Vera median survival is _____. What is the MC cause of death? What are some complications?
15 years thrombosis conversion to myelofibrosis, CML or rarely AML
62
______ a disorder of increased proliferation of the megakaryocytes (precursor for platelet). What does it result from? What is the average age of dx?
Essential Thrombocytosis (ET) genetic mutations (JAK2, CALR, MPL) 50-60 yrs
63
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?
essential thrombocytosis
64
What would you expect the CBC of essential thrombocytosis to look like? peripheral blood smear?
elevated platelet count may be >2,000,000 /mcL mild leukocytosis large platelets
65
What would the bone marrow bx look like on a pt with Essential Thrombocytosis (ET)? What will genetic testing show?
increased megakaryocytes (+) JAK2, CALR or MPL mutation
66
______ determines treatment in essential thrombocytosis?
genetic testing
67
**What 3 major factors increase your risk of thrombosis in ET?
> 60 y/o hx of thrombosis JAK2 mutation
68
What are the risk stratifications for ET?
69
What is the management of very low- low risk ET?
observation, ASA 81 mg daily avoid NSAIDS - use increases risk of bleeding
70
What is the management of intermediate- high risk ET?
ASA 81 mg + hydroxyurea with a target plt count of 100,000 to 400,000/µL
71
_____ is the MC cause of morbidity/mortality in ET. What can reduce the risk? What is the average survival?
thrombosis risk is reduced with plt count < 500,000/mcL greater than 15 years with adequate treatment
72
_____ an elevated RBC/Hgb/Hct related to an acquired or congenital disorder
secondary erythrocytosis
73
What are 4 etiology factors that contribute towards secondary erythrocytosis?
tissue hypoxia decreased renal perfusion Inappropriate EPO stimulation Testosterone administration
74
arterial oxygen plethora, ruddy skin HA, lethargy, confusion clubbing fingers Splenomegaly less likely: helps to differentiate secondary erythrocytosis from PV What am I?
secondary erythrocytosis
75
What are very important lab values that points to secondary erythrocytosis?
Negative JAK2 gene **Increased EPO level
76
What will the CBC look like in secondary erythrocytosis?
CBC - increased RBC, Hgb, Hct, +/- slight increase in plt
77
______ an elevated platelet count that develops secondary to another disorder
Reactive Thrombocytosis
78
____ results from an increased megakaryocyte proliferation/maturation results from increased production of inflammatory cytokines
Reactive Thrombocytosis
79
Reactive Thrombocytosis pathophysiology includes what 3 things?
increased megakaryocyte proliferation/maturation accelerated platelet release reduced platelet sequestration/turnover
80
What other labs should you also order for reactive thrombocytosis? What are you looking for?
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

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