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hem/onc > leukocyte disorders pt 2 > Flashcards

leukocyte disorders pt 2 Flashcards

1
Q

what organs are part of the lymph system

A
  1. lymph nodes and lymph vessels
  2. thymus
  3. tonsils/adenoids
  4. spleen
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2
Q

what are the extranodal lymph tissue sites

A
  1. skin
  2. GI tract, liver
  3. bone marrow
  4. testicles
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3
Q

lymph node masses increase and become easier to palpate in what population?

A

between birth - 12y/o

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4
Q

what is the normal LN sizes in children

A
  1. anterior cervical ≤ 2 cm
  2. axillary ≤ 1 cm
  3. inguinal nodes ≤ 1.5 cm
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5
Q

normal LN sizes of adults

A

Normal Findings in Adults
< 1 cm at any location

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6
Q

what types of exposures to infectious etiology would lead to enlarged LNs

A
  1. cat scratch
  2. undercooked meat
  3. tick bite
  4. travel to endemic area
  5. high risk behavior
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7
Q

local adenopathy often indicates a

A

local etiologic pathology

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8
Q

hard nodes = ?
firm, rubbery nodes = ?
softer nodes = ?

A
  1. fibrotic cancers
  2. lymphomas, chronic leukemia
  3. acute leukemia, inflammation
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9
Q

tender lymphadenopathy is indicative of?

A

acute rapid enlargement = indicative of inflammatory process

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10
Q

nontender lymphadenopathy is indicative of ?

A

malignancy

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11
Q

normal LNs should have what type of fixation?

A

mobile, freely moveable

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12
Q

a fixed LN to the skin, underlying or adjacent tissues is indicative of ?

A

malignancy or inflammation of surrounding tissues

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13
Q

what does a “matted” LN mean?

A

nodes become fixed to each other

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14
Q

management/tx for lymphadenopathy alone in children

A
  1. short course of broad-spectrum antibiotic
    - High CA-MRSA prevalence - clindamycin
    - Low CA-MRSA prevalence - cephalexin or amoxicillin-clavulanate
    - Exposure to cat/kittens (cover B. henselae) - add azithromycin
  2. follow up 2-4 wks
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15
Q

management for lymphadenopathy in adults or those with constitutional symptoms

A
  1. work up to r/o malignancy
  2. referral for lymph node biopsy
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16
Q

A malignant overgrowth of the lymphocyte or its precursor within the lymphatic tissue

A

non-hodgkin lymphoma
MC site - lymph nodes

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17
Q

pathophys of non-hodgkin lymphoma

A
  • a monoclonal proliferation of lymphocytic cells
  • All 3 lymphocytic cells can be affected; MC = B cells
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18
Q

causes of non-hodgkin lymphoma

A
  1. Chromosomal translocations
  2. Infections - chronic antigenic stimulation and cytokine dysregulation = lymphocyte stimulation & proliferation
    - EBV (Mono)
    - Hepatitis B/C
    - H. pylori
    - Kaposi sarcoma-associated herpesvirus
  3. Environmental factors
    - chemicals, chemotherapy, radiation exposure
  4. Immunodeficiency
    - AIDS
    - iatrogenic immunosuppression
    - congenital immunodeficiency disorders
  5. Chronic inflammation
    - autoimmune disorders
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19
Q

what is the MC type of lymphoma?

A

non-hodgkin

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20
Q

epidemiology of non-hodgkin

A

50-60y/o
white
male

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21
Q

2 types of clinical presentation of non-hodgkin

A
  1. Indolent - painless, slow growing (worst one)
  2. Aggressive - painless, rapid growth, spread
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22
Q

what are the “B-symptoms” of aggressive non-hodgkins

A
  1. Unexplained weight loss
  2. Unexplained fever
  3. Drenching night sweats
    seen in advanced stage
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23
Q

clinical presentation of indolent non-hodgkins

A
  1. painless and slow growing lymphadenopathy
    - isolated or generalized
    - peripheral or central
    - spontaneous regression may be noted in history
  2. HSM
  3. cytopenias
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24
Q

clinical presentations of aggressive non-hodgkins

A
  1. Fast growing painless lymphadenopathy
    - compresses on surrounding structures - lungs, SVC, bowel, ureters
  2. B-symptoms
  3. HSM, abd/testicular mass
  4. disseminated disease
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25
Q

work up for non-hodgkins

A
  1. CBC - normal until BM infiltrated
  2. peripheral smear - normal
  3. CMP - depends on organ affected
    - ↑ BUN/Cr with hydronephrosis
    - ↑ LFT with hepatic involvement
    - Alkaline phosphatase (ALP) - ↑ liver/bone involvement
  4. LDH - increased if advanced
  5. viral serology
  6. imaging
    - CXR - mediastinal nodes/mass
    - CT w/ contrast - evaluate extent of lymph node involvement
  7. excisional LN bx - diagnostic
    - (+) monoclonal lymphocytes
    - peripheral > central site
  8. BL bone marrow bx
  9. PET scan
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26
Q

what is the diagnostic work up for nonhodgkins

A

Excisional lymph node bx
(+) presence of monoclonal lymphocytes

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27
Q

when is Excisional lymph node bx indicated

A

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

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28
Q

what site to use for LN excision for nonhodgkins

A

peripheral preferred over central
if peripheral node is absent - CT-guided core needle biopsy or laparoscopic biopsy

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29
Q

what staging system is used for nonhodgkins and hodgkins? describe the stages

A

Ann arbor staging system
1. stage 1 = single LN area
2. stage 2 = 2+ LN in same side of diaphragm
3. stage 3 = LN on both sides of diaphragm
4. stage 4 = disseminated or multiple extranodal organs involved

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30
Q

Ann Arbor staging system requires what other work ups

A

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

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31
Q

“A”/”B” symptomatology of the Ann Arbor staging system means?

A

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

32
Q

what are the additional Ann Arbor staging that indicate which system it involves?

A

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

33
Q

tx for indolent nonhodgkins

A
  1. often disseminated at time of diagnosis - incurable
  2. treatment has not shown to increase overall survival
  3. treatment is only recommended if symptomatic
    - single or multidrug chemotherapy
34
Q

tx for aggressive nonhodgkins

A
  1. chemotherapy +/- local radiation therapy
  2. allogeneic stem cell transplant
35
Q

what is the avg survival rate after indolent NHL diagnosis?

A

10-15 yrs

36
Q

what is the prognosis for aggressive NHL

A

depends how many factors they have - decreases by 25%
- 0-1 factor - 75% 5 year survival rate
- 2-3 factors – 50% 5 year survival rate
- 4 -5 factors – 25% 5 year survival rate

37
Q

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

A

Hodgkin Lymphoma

38
Q

etiology of Hodgkin Lymphoma

A
  1. viral
    - EBV
    - HIV
  2. genetic (slight)
39
Q

epidemiology of HL

A
  1. age - 2 peaks
    - young adults - 20’s
    - middle age - 50+ y/o
    no race/ethnic correlation
40
Q

if a pt presents with a painless “mass” on their neck and experiences pain after alcohol consumption, what is the probable diagnosis?

A

hodgkin
MC in neck

41
Q

presentation of hodgkins

A
  1. painless “mass” lymph node
  2. B-symptoms
  3. generalized pruritus
  4. +/- hepatosplenomegaly
    - abdominal fullness, early satiety
  5. +/- mediastinal mass
    - chest pain, cough, SOB
    - SVC syndrome
42
Q

work up for HL

A
  1. Labs
    - CBC: normal or nonspecific findings
    - LDH:↑ with advanced “bulk” disease
    - Alkaline phosphatase (ALP): ↑ liver/bone disease
    - Viral Serology: HIV, HCV, HBV
  2. Lymph node excisional bx
    - (+) Reed-Sternberg cells
  3. Imaging for staging purposes
    - CXR, CT, PET
43
Q

what confirms the diagnosis of HL

A

Reed-Sternberg cells from LN excision biopsy

44
Q

“bulk” with HL means?

A

lymph node >10 cm
or
mediastinal mass >⅓ thoracic diameter

45
Q

tx for HL

A
  1. Stage I-II (with no bulky masses)
    - multi-drug chemotherapy +/- field radiation therapy
  2. Stage III-IV or bulky stage II
    - multi-drug chemotherapy
  3. Relapse after initial treatment
    - high dose chemotherapy and autologous stem cell transplant
46
Q

what are the poor prognostic risk factors for HL

A
  1. Low serum albumin < 4 g/dL
  2. Low hemoglobin < 10.5 g/dL
  3. Male sex
  4. > 45 y/o
  5. Stage IV disease
  6. High WBC > 15,000/mm3
  7. Low ALC count <600/mm 3, <8% of total WBC count, or both
47
Q

an acquired disorder resulting in overproduction of all 3 hematopoietic cell lines

A

Polycythemia Vera

48
Q

a mutation on the JAK2 gene leading to excessive cell growth and division causes what disorder?

A

Polycythemia Vera

49
Q

etiology of Polycythemia Vera

A

ionizing radiation and toxins suggested as risk factors
unknown otherwise

50
Q

epidemiology of polycythemia vera

A

peak incidence seen between 50-70 y/o
no sex or ethnic predilection

51
Q

clinical presentation of polycythemia vera

A
  1. Fatigue, HA, dizziness, vertigo, tinnitus, visual disturbances, chest pain, intermittent claudication
    - from increased blood viscosity
  2. generalized pruritus (basophilia)
    - worse after warm shower/bath
  3. bleeding - epistaxis, bleeding gums, ecchymosis, GI
    - engorged vessels and platelet dysfunction
  4. Venous thrombosis/thromboembolism/stroke
  5. abd pain
  6. Early satiety (HSM)
  7. Engorged conjunctival and retinal vessels
  8. Plethora
52
Q

labs for polycythemia vera

A
  1. CBC
    - elevated RBC (normocytic/normochromic), WBC, Plt
    - HCT: > 54% male and 51% female (hallmark)
  2. Peripheral smear (normal)
  3. CMP
  4. Erythropoietin lvl = low (occasionally normal)
  5. Genetic testing: (+) JAK2 mutation
53
Q

what confirms polycythemia vera

A

low erythropoietin levels + presence of JAK2 mutation

54
Q

tx for polycythemia vera

A
  1. therapeutic phlebotomy
    - 1 unit of whole blood (500 ml) removed weekly until hct < 45 %
    - Do not treat resulting iron deficiency - will reverse phlebotomy effects
  2. ASA 81 mg daily - unless CI by active blood loss
  3. lifestyle modifcations
    - Smoking
    - CV risk factors
    - hypoxic conditions
  4. Cytoreductive therapy - hydroxyurea: suppresses bone marrow cellular production by interfering with DNA repair
  5. Ruxolitinib (Jakafi) - (JAK1/JAK2 inhibitor) or pemigatinib (Pemazyre) - (FGFR inhibitor¹)
    indicated if failure to phlebotomy and HU and any of the following:
    - Markedly symptomatic splenomegaly
    - Severe, protracted pruritus
    - Post-PV myelofibrosis
55
Q

what is used if failure to phlebotomy and HU for PV?

A

Ruxolitinib (Jakafi)
also if any of the following happen:
1. Markedly symptomatic splenomegaly
2. Severe, protracted pruritus
3. Post-PV myelofibrosis

56
Q

SE and CI of hydroxyurea

A
  1. SE: neutropenia, anemia, oral ulcers, mild GI upset, rash, nail changes hyperpigmentation
  2. CI: Severe bone marrow suppression, pregnancy, attempted conception, breast feeding
57
Q

POC for low-risk pts with polycythemia vera

A
  1. Therapeutic phlebotomy
  2. 81 mg ASA
  3. life-style modifications
  4. Cytoreductive therapy only if:
    - uncontrolled PV-associated symptoms
    - progressive increase of leukocyte and/or platelet counts
    - symptomatic or progressive splenomegaly
    - poor tolerance of phlebotomy
58
Q

POC for high-risk pts with polycythemia vera

A

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

59
Q

what is the MC of death with polycythemia vera?

A

thrombosis

60
Q

complications with polycythemia vera

A
  1. thrombosis
  2. conversion to myelofibrosis, CML or rarely AML
61
Q

a disorder of increased proliferation of the megakaryocytes (precursor for platelet)

A

Essential Thrombocytosis (ET)
from genetic mutation - MC is JAK2

62
Q

clinical presentation of essential thrombocytosis (ET)

A
  1. Microvascular occlusion - pain in fingers/toes
    - relieved with ASA
  2. Thrombosis - mesenteric, hepatic or portal vessels, peripheral DVT
  3. HA
  4. Transient dizziness, unsteadiness, vertigo, syncope - transient ischemic attacks (TIA - “mini-stroke”)
  5. Bleeding (less common)
63
Q

labs for essential thrombocytosis (ET)

A
  1. CBC
    - elevated platelet count may be >2,000,000 /mcL
    - mild leukocytosis
  2. Peripheral blood smear
    - large platelets
  3. CMP
  4. Bone marrow aspiration/biopsy
    - increased megakaryocytes
  5. Genetic testing
    - (+) JAK2, CALR or MPL mutation
64
Q

risk factors for thrombosis in essential thrombocytosis (ET)

A
  1. > 60 y/o
  2. hx of thrombosis
  3. Plt > 1,500,000/µL
  4. obesity
  5. CV risk factors : smoking, HTN, hyperlipidemia
  6. hypercoagulable state
  7. JAK2 mutation
65
Q

Risk staging for Essential Thrombocytosis (ET)

A
  1. High-risk disease
    - hx of thrombosis at any age and/or age >60 with a JAK2 mutation
  2. Intermediate-risk disease
    - Age >60, no JAK2 mutation detected, and no hx of thrombosis
  3. Low-risk disease
    - Age ≤60 with JAK2 mutation and no hx of thrombosis
  4. Very-low-risk disease
    - Age ≤60, no JAK2 mutation detected, and no hx of thrombosis
66
Q

Management for very low - low risk ET

A
  1. observation, ASA 81 mg daily
  2. avoid NSAIDS - use increases risk of bleeding
67
Q

management for Intermediate - High risk with ET

A

hydroxyurea with a target plt count of 100,000 to 400,000/µL

68
Q

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

A

Secondary Erythrocytosis

69
Q

etiology of Secondary Erythrocytosis

A
  1. Tissue hypoxia
  2. Decreased renal perfusion
  3. Inappropriate EPO stimulation
  4. Testosterone administration
70
Q

presentation of Secondary Erythrocytosis

A
  1. arterial oxygen - low in hypoxemic conditions
  2. skin
    - ruddy complexion - “plethora”
    - hypoxic patients will present with acrocyanosis
  3. neurologic - related to increased blood viscosity
    - headaches, lethargy, and confusion
  4. clubbing of fingers - long term hypoxia
  5. NO splenomegaly
71
Q

what differentiates Secondary Erythrocytosis from PV?

A

NO splenomegaly in secondary

72
Q

labs with secondary erythrocytosis

A
  1. CBC - increased RBC, Hgb, Hct, +/- slight increase in plt
  2. (-) JAK2 gene
  3. Increased EPO level
  4. Elevated carboxyhemoglobin levels in CO poisoning or heavy smokers
73
Q

an elevated platelet count that develops secondary to another disorder

A

Reactive Thrombocytosis

74
Q

pathophys of Reactive Thrombocytosis

A
  1. increased megakaryocyte
    - increased inflammatory cytokines
    - stimulation of RBC progenitors stimulates
    • Anemia: hemorrhage, iron deficiency, hemolysis
  2. accelerated platelet release
  3. reduced platelet sequestration/turnover
    - asplenia

(depends on the etiology)

75
Q

labs for Reactive Thrombocytosis

A
  1. CBC
    - elevated plt, (+/-) low RBC, H&H
  2. Inflammatory condition evaluation?
    - Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Antinuclear antibody (ANA), rheumatoid factor (RF)
  3. Malignancy?
    - Cytogenetic analysis
  4. Hematologic disorder?
    - Iron studies
    - Peripheral blood smear
    - Reticulocyte count

(based upon your DDx)

hem/onc (13 decks)

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