Leukocyte Disorder- Part II- Exam 2 Flashcards
What are some extranodal lymph tissue sites?
skin
GI tract, liver
bone marrow
testicles
What are normal lymph nodes sizes in children for the anterior cervical, axillary and inguinal nodes? What is it for adults?
anterior cervical ≤ 2 cm
axillary ≤ 1 cm
inguinal nodes ≤ 1.5 cm
normal for adults is 1cm
A patient presents with enlarged lymph nodes, what questions should you ask?
cat scratch, undercooked meat, tick bite, travel to endemic area, high risk behavior
constitutional symptoms
any adverse medication reactions
What size does it need to be in order to be considered large in an adult?
Size (ex:1x1.5 cm)
What does the consistency of a lymph node tell you, hard nodes?
fibrotic cancers
What does the consistency of a lymph node tell you, firm rubbery nodes?
lymphomas, chronic leukemia
What does the consistency of a lymph node tell you, softer nodes?
acute leukemia, inflammation
What does a tender lymph node tell you? no tenderness?
Tenderness = acute rapid enlargement = indicative of inflammatory process
No tenderness = more indicative of malignancy
In terms of fixation a normal node will be _____, a malignant node will be ______
normal= mobile
fixed to skin, or unable to move= malignancy or inflammation of surrounding tissues
_____ when nodes become fixed to each other
“matted”
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?
clindamycin
add azithromycin
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?
cephalexin or amoxicillin-clavulanate
add azithromycin
What should you do for lymphadenopathy alone in adults or those with constitutional symptoms?
work up to r/o malignancy
referral for lymph node biopsy
_____ A malignant overgrowth of the lymphocyte or its precursor within the lymphatic tissue. What is the MC site?
Non-Hodgkin Lymphoma
lymph nodes
What is the pathophys behind Non-Hodgkin Lymphoma? Which cells are MC affected?
a monoclonal proliferation of lymphocytic cells due to chromosomal translocation
MC is B-cell - 85%
T/NK cell - 15%
What are some risks factors for NHL?
EBV (Mono), Hepatitis B/C, Helicobacter pylori, Kaposi sarcoma-associated herpesvirus, chemicals, chemotherapy, radiation exposure, AIDS, iatrogenic immunosuppression, congenital immunodeficiency disorders, autoimmune disorders
_____ is the MC type of lymphoma. What is the average age of onset?
NHL
average age of onset is 50-60
white, male
What are the two types of clinical presentation of NHL?
indolent: slow growing
aggressive: rapid growth and spread
What is the classic presentation of indolent NHL?
painless and slow growing lymphadenopathy, can be isolated or generalized, spontaneous regression is possible
HSM
cytopenias
What is the classic presentation of aggressive NHL?
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)
What does the CBC of a NHL pt look like?
usually remains normal until bone marrow infiltration, which results in pancytopenia
What does the peripheral smear of a NHL pt look like?
confirms CBC with no morphologic changes
Why would you order a viral serology screening on a NHL pt?
Screening for HIV, HCV, HBV
When you order a CXR on a NHL pt, what are you looking for?
mediastinal nodes/mass
if present then need to order a CT with contrast
**What is the definitive way to dx NHL?
Excisional lymph node bx that is (+) presence of monoclonal lymphocytes
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
When is a bone marrow bx used in NHL?
utilized for staging
bilateral bx needed due to patchy involvement
____ is used in NHL to assess the extent of the disease
PET scan
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”
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
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
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
______ a malignancy of the B-lymphocytes within lymph tissue characterized by the presence of Reed-Sternberg cells
hodgkin lymphoma
hodgkin lymphoma is a malignancy of the ____. What special cells show up on bx?
B cell line
Reed-Sternberg cells: they are mulitnucleated
Hodgkin lymphoma peaks in _____ and ______. What gender? What race?
20’s and 50’s
more common in men, caucasian and black equal chance
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
What is a specific but infrequent finding with HL?
pain after ETOH consumption¹
In HL with bulk disease, the LDH will often be ____
elevated
**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
What confirms the dx of HL?
Lymph node excisional biopsy
confirms diagnosis with (+) Reed-Sternberg cells
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%
What is the treatment for relapse after initial treatment for HL?
high dose chemotherapy and autologous stem cell transplant
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
_____ 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
What are 2 suggested risk factors for polycythemia vera?
ionizing radiation and toxins
What gender and race preference does polycythemia vera have? What age range?
no gender or race predilection
50-70 years old
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
Why do polycythemia vera pts have abdominal discomfort?
ulcer formation from increased gastric acidity due to excessive histamine
What is the hallmark CBC lab finding of polycythemia vera?
**HCT: > 54% male and 51% female
elevated RBC (normocytic/normochromic), WBC, Plt
What does the peripheral smear look like on a polycythemia vera pt?
normal
Erythropoietin level on polycythemia vera pt is usually ______
low (occasionally normal)
What is the way you can confirm the dx of Polycythemia Vera?
Genetic testing: (+) JAK2 mutation
What is the management of polycythemia vera?
therapeutic phlebotomy
1 unit of whole blood (500 ml) removed weekly until hct < 45 %
hydroxyurea
1 unit of phlebotomy reduces the hct by _____ (normal-sized adult)
3% points
What would you NOT want to give to a polycythemia vera pt? What do you want to give?
iron supplementation
ASA 81mg daily
_____ suppresses bone marrow cellular production by interfering with DNA repair. What is the MC side effect?
hydroxyurea
severe bone marrow suppression
**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
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)
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
What is the treatment for high risk Polycythemia Vera pts?
Therapeutic phlebotomy, low dose ASA, life-style modifications and HU
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
______ 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
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
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
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
______ determines treatment in essential thrombocytosis?
genetic testing
**What 3 major factors increase your risk of thrombosis in ET?
> 60 y/o
hx of thrombosis
JAK2 mutation
What are the risk stratifications for ET?
What is the management of very low- low risk ET?
observation, ASA 81 mg daily
avoid NSAIDS - use increases risk of bleeding
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
_____ 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
_____ an elevated RBC/Hgb/Hct related to an acquired or congenital disorder
secondary erythrocytosis
What are 4 etiology factors that contribute towards secondary erythrocytosis?
tissue hypoxia
decreased renal perfusion
Inappropriate EPO stimulation
Testosterone administration
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
What are very important lab values that points to secondary erythrocytosis?
Negative JAK2 gene
**Increased EPO level
What will the CBC look like in secondary erythrocytosis?
CBC - increased RBC, Hgb, Hct, +/- slight increase in plt
______ an elevated platelet count that develops secondary to another disorder
Reactive Thrombocytosis
____ results from an increased megakaryocyte proliferation/maturation
results from increased production of inflammatory cytokines
Reactive Thrombocytosis
Reactive Thrombocytosis pathophysiology includes what 3 things?
increased megakaryocyte proliferation/maturation
accelerated platelet release
reduced platelet sequestration/turnover
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
