CASE STUDY #1 NODES and B SYMPTOMS

History and Physical Findings

A 27-year-old female comes in to the clinic complaining of having fevers accompanied by drenching
night sweats almost every day over the past month. She has also noticed she has had a weight loss of 8-
10 pounds over the last month. She states that up until now, she had never been to the doctor for
anything other than routine check-ups and gynecologic exams.
General: Thin female in no acute distress. Height 170 cm (67), weight 46 kg (101 lbs)
Vitals: T 37
o
C (98.6
o
F), P 95, BP 96/66, RR 16
Skin: No abnormality noted
HEENT: The oropharynx benign. Mucous membranes are pink and moist
Neck: Supple
Nodes: A 1 x 2.5 cm non-tender, firm, mobile lymph node is noted in the upper right anterior cervical chain.
No other lymphadenopathy is palpable
Pulm: Unlabored respiration, clear to auscultation and percussion bilaterally
CV: Normal S1, S2, regular, no murmurs
Abd: Soft, non-tender, non-distended, without organomegaly or masses detectable
Extr: Warm, well perfused, no edema
Neuro: Alert and oriented to person, place, and time. No motor or sensory defects noted

Question#1
What is the primary nursing intervention needed to care for this patient at the current time?
a. Inquire if she has a thermometer and instruct her on taking her temperature every 4 hours.
b. Provide education and reassurance related to her current symptoms and then coordinate
labwork and a biopsy with medical direction.
c. Make a referral to the dietician to assist her with her weight loss.
d. Contact palliative care as she likely has a terminal diagnosis.



Laboratory findings
WBC=13.2x10E9/L; Hgb= 90g/L; Plts=408x10E9/L; K+= 4.3mmol/L; Cr=90umol/L; Ca=2.59mmol/L; PO4=
0.7mmol/L; LDH=296U/L; ALK PHOS= 174U/L; ALT=55U/L; ALB=22g/L; Uric acid=361umol/L; CRP=
9.1mg/L; ESR=35mm; TSH=4.0mU/L; HBV= negative; HCV=negative; HIV=negative; DAT=negative
Pathology report: Presence of Reed Steenberg cells staining positive for CD15 and CD30
Question #2
Based on your understanding of the lab values and pathology report what diagnoses do you suspect?
a. Thymoma
b. Follicular Lymphoma
c. Infectious mononucleosis
d. Hodgkins Disease
e. Primary mediastinal B-cell lymphoma

Epidemiology
Hodgkin lymphoma is less common than the group of non-Hodgkin lymphomas, with an incidence of
about 8,500/year in the United States in 2010. There is a peak in individuals 15 to 30 years of age,
followed by another increase in incidence in persons over 55 years old.
A viral cause for Hodgkin disease has been suspected because of the association of Epstein Barr Virus
(EBV) infection in about 30-40% of cases. However, the role of EBV infection remains controversial.
Additional support for a viral cause or for susceptibility to the disease based on an immune mechanism
comes from the observation that the disease is more common in individuals of higher socioeconomic
status, suggesting that less exposure to certain infectious agents early in life may set the stage for the
potential development of disease.
As for outcome, Hodgkin lymphoma and pediatric acute lymphoid leukemia were two of the first
diseases to have their natural history dramatically altered by modern chemotherapy regimens. Today,
even most relatively advanced cases of Hodgkin lymphoma are cured, resulting in rates of overall 5-year
survival of 85% or more. As will be described in more detail below, a large focus of treatment today is on
retaining a high rate of cure while reducing the toxicity of therapy (Marks, 2011)
Classification
The World Health Organization (WHO) describes two major categories of Hodgkin lymphoma: classical
Hodgkin lymphoma, representing about 95% of cases, and nodular lymphocyte predominant Hodgkin
disease, representing the remainder. Classical Hodgkin lymphoma is further divided into four
subcategories: nodular sclerosis, mixed cellularity, lymphocyte-depleted, and lymphocyte-rich.
Classical Hodgkin lymphoma is characterized by the presence of Reed-Sternberg cells in a background of
lymphocytes. Over the past decades research has demonstrated that the Reed- Sternberg cells are the
malignant cells in Hodgkin disease. These cells have a specific cell surface phenotype, and usually stain
positive for CD30 and CD15. The different sub-types of classical Hodgkin lymphoma are distinguished by
the context in which the Reed-Sternberg cells appear (Marks, 2011).

Subtypes of Classical Hodgkin Lymphoma

Nodular sclerosis classical Hodgkin
lymphoma is the most common subtype of
Hodgkin lymphoma in the West. It affects
males and females with equal frequency
and most commonly presents with cervical,
axillary, and mediastinal
lymphadenopathy. The pathology of the
lymph node shows that there are broad
collagen bands and some lymphocytes
surrounding the Reed-Sternberg cells.

Mixed cellularity Hodgkin lymphoma occurs
more commonly in developing countries and
in patients with HIV infection. It is about
equally common in males as in females. It
tends to present at a more advanced stage
with involvement of peripheral lymph nodes
and the spleen. The lymph nodes
architecture is replaced with Reed-Sternberg
cells, which appear in a mixed inflammatory
background of eosinophils, plasma cells and
histiocytes.

Lymphocyte-depleted Hodgkin lymphoma
is also seen more commonly in developing
countries. It is four times as common in
males as in females and often presents
with systemic symptoms (B symptoms)
such as fevers, chills and night sweats). As
with mixed cellularity disease, it often
presents at a more advanced stage, in this
case with retroperitoneal and abdominal
disease. Pathology shows a hypocellular
background with fibrosis and fewer
lymphocytes than other types.
Lymphocyte-depleted Hodgkin lymphoma
has the most aggressive course and
poorest prognosis of the Hodgkin
lymphomas.

Lymphocyte-rich Hodgkin lymphoma is
twice as common in males as in females. It
tends to present with peripheral lymph node
involvement at an early stage. The affected
lymph nodes have few Reed-Sternberg cells
in a lymphocytic infiltrate with some
histiocytes but few eosinophils. Following
treatment, lymphocyte-rich Hodgkin
lymphoma is associated with a low incidence
of relapse and an excellent prognosis.


Nodular Lymphocyte Predominant Hodgkin Lymphoma
The second major category of Hodgkin lymphoma described by WHO is nodular lymphocyte
predominant Hodgkin disease, which represents about 5% of cases. This type is distinguished by the
presence of popcorn cells, called LP cells according to the current WHO classification. The surface
marker phenotype of these cells is distinctly different from that found in classical Hodgkin lymphoma:
they are negative for CD30 and CD15, but positive for CD45 and CD20. It is about three times more
common in males that in females. It tends to present with involvement of a single lymph node rather
than a group of lymph nodes. The popcorn or LP cells occur with a background of small lymphocytes in a
nodular pattern (at least partially). Nodular lymphocyte-predominant Hodgkin disease is associated with
a good response to therapy, but tends to relapse. It also may appear to progress to other types of
lymphoma, such as diffuse large B cell lymphoma (a non-Hodgkin lymphoma) (Marks, 2011).




Question #3
You are working with Dr. S and notice in the dictation it states the patient has stage III disease. However
you know that the patient presented with only one enlarged node and the presence of B symptoms.
You assume:
a. There must be another staging system that has been used to stage this patients disease.
b. The transcriptionist has committed a typing error because the physician is never wrong.
c. The dictation you are viewing is the wrong patient.
d. The dictation is wrong because the patient has stage IB Hodgkin Disease according to the Ann Arbor
Staging system which is typically used to stage this disease.
e. Never assume anything!

Staging
The Ann Arbor system is used for staging Hodgkin disease into four major categories. The utility of this
staging system is that it has implications for both treatment and prognosis. Stages I III may be
associated with a single site of localized extra lymphatic involvement, in which case an E is appended to
that given stage. For this purpose, the spleen is considered to be equivalent to a large lymph node.
Each stage may also be associated with the absence or presence of systemic symptoms (called B
symptoms). An A or B is routinely appended to the assigned stage in order to denote this, as the
presence of systemic symptoms is generally associated with a poorer prognosis for any given stage
(Marks, 2011).

Unfavorable prognostic factors for stage I and II Hodgkin disease include the presence of B symptoms,
more than three nodal sites of disease, or an ESR of 50 mm/hr or more, and mediastinal masses 10 cm
in diameter or more.


Hodgkin Disease Staging
Stage Description
I Involvement of a single lymph node region
II Involvement of two or more lymph node regions on the same side of the diaphragm
III Involvement of lymph node regions on both sides of the diaphragm
IV Multifocal/disseminated involvement of one or more extralymphatic organs
A No systemic symptoms present
B Unexplained fevers >38
o
C, drenching night sweats, or weight loss >10% body weight
For stage III and IV disease, seven adverse prognostic factors have been identified:
Age 45 years or older
Male gender
Stage IV disease
Albumin < 4 g/dL
Hemoglobin < 10.5 g/dL
WBC > 15,000/L
Relative lymphocyte count < 8% of total WBC or absolute number < 600/L
The International Prognostic Score for Hodgkin disease uses these to predict who might benefit from
more intensive therapy; the presence of at least four indicates that this may be the case (Marks, 2011).

Question #4
After confirmation that the patient indeed has stage IB Hodgkin Disease you anticipate you need to
teach her about the chemotherapy she will receive. First line treatment/ chemotherapy for Stage 1B
Hodgkin Disease is:
a. CDDPGem (Cisplatin/Gemcitabine).
b. AC (Adriamycin/Cyclophosphamide).
c. ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine).
d. R-CHOP (Rituximab/Cyclophosphamide/Doxorubicin/Oncovin/Prednisone).

Question #5
The toxicities most associated with ABVD that are necessary for this patient to know is the following:
a. Adriamycin is a vesicant, turns your urine pink-red and increases your risk of cardiotoxicity
b. You will experience nausea and vomiting so it is important to take your scheduled antiemetics as
prescribed.
c. You may experience constipation and peripheral neuropathy related to the Vinblastine.
d. Dacarbazine is not a vesicant but it can be irritating to the veins.
e. Bleomycin can cause pulmonary toxicity so it is important to reports any signs of shortness of
breath, or changes in your breathing.
f. All of the above.

Question #6
Being an astute hematology/bmt nurse you are well aware many tests will be ordered before, during
and after treatment. To ensure safe practice you check to see the physician has ordered the following:
a. Labwork prior to each cycle
b. A baseline ECG, MUGA or ECHO prior to Adriamycin or if the patient becomes symptomatic.
c. Pulmonary function tests per institution standard when administering Bleomycin.
d. Interim PET/CT scans per physician direction.
e. All of the above.

Question #7
Given the age of the patient, no co-morbid conditions, stage of her disease and your own experience
with caring for patients with Hodgkin Disease you anticipate this patient will:
a. Have trouble battling this disease for decades to come.
b. Have a high chance of cure after completion of all her chemotherapy.
c. Likely need an allogeneic bone marrow transplant.
d. None of the above.
Prognosis and Treatment
Along with pediatric acute lymphoid leukemia, Hodgkin disease was one of the first cancers to be
effectively cured by modern therapy. Extended field radiotherapy was found to provide cure for patients
with Stage I and II disease, particularly when there were no associated symptoms. Combination
chemotherapy regimens were developed through clinical research, and eventually, one consisting of
mechlorethamine (a nitrogen mustard), vincristine (Oncovin is a brand name), procarbazine and
prednisone (MOPP) was used in combination with radiotherapy and associated with a high cure rate of
more advanced disease. However, due to its high rate of associated side effects, including secondary
leukemias, other less toxic approaches were developed (Mark, 2011).
Modern therapy for Hodgkin lymphoma involves radiotherapy or chemotherapy for early stage disease
(usually IA or IIA) and chemotherapy for all other stages, either alone or with radiotherapy to the
involved areas. Careful consideration is generally given to the use of radiotherapy in Hodgkin lymphoma
because it is so responsive to chemotherapy and because radiation therapy is associated with long-term
side effects, including therapy-related cancers such as breast cancer in a woman treated to the
mediastinal region.
The most common combination chemotherapy regimen used in the United States includes doxorubicin
(Adriamycin is a brand name), bleomycin, vinblastine and dacarbazine (ABVD). This is associated with an
approximately 80% remission rate, even in advanced disease. However, there are significant toxicities.
Cumulative doses of doxorubicin can be associated with cardiac toxicity, particularly when above a
threshold. Bleomycin is associated with the development of pulmonary toxicity in some patients, and
therefore, close patient monitoring of pulmonary function is required.
Other combination regimens exist and may be appropriate in high-risk patients. When patients relapse,
they are generally treated with salvage chemotherapy regimens in order to achieve a second remission
and then may proceed to autologous hematopoietic stem cell transplant.
In this patients case, a bone marrow aspirate and biopsy was performed as part of the staging
evaluation. However, it should be noted that in current practice, it is often omitted from the evaluation,
particularly in individuals with normal blood counts.
Following documentation of normal baseline cardiac and pulmonary function, this patient received
treatment with two cycles of ABVD chemotherapy. PET-CT restaging scans after this showed no
evidence of uptake in any of the lymph node regions, nor in the mediastinum. A small residual mass
observed on the CT scan in the mediastinum was not felt to represent active disease, but rather to
represent fibrotic scar tissue (this is not uncommonly observed in the treatment of Hodgkin disease with
mediastinal involvement). After this, two more cycles of ABVD chemotherapy were administered,
followed by radiation therapy to the region of the chest involved with the mediastinal mass. After
completion of therapy, she was followed every three months and had repeat scanning performed at
regular intervals for two years. Five years out, she is disease-free. The 5-year survival of the overall
population of patients with Hodgkin lymphoma is now about 85%. Survival is even better than this today
for patients with favorable prognostic features independent of the exact treatment regimen
administered.



References
Marks, P. Fevers, nightsweats, and weight loss. Am Soc Hematol teaching cases 2011.

Advani RH. Optimal therapy of advanced Hodgkin lymphoma. Hematology Am Soc Hematol Educ
Program 2011; 2011:310-6.
Connors JM. Positron emission tomography in the management of Hodgkin lymphoma. Hematology Am
Soc Hematol Educ Program 2011; 2011:317-22.
Hoppe RT, Advani RH, Ai WZ et al. Hodgkin lymphoma. J Natl Compr Canc Netw 2011; 9:1020-1058.
Mani H, Jaffe ES. Hodgkin lymphoma: an update on its biology with newer insights into classification.
Clin Lymphoma Myeloma 2009; 9:206-216.