Hodgkin's Lymphoma and Brian's Chronicle: A decidedly Curable Cancer:
BRIAN'S
STORY
Brian
was a 20-year old college basketball star who was in excellent condition and
had no history of health problems. He grew up in a tough neighborhood with a
single mother who raised him to be a fine young gentleman. He was awarded a
basketball scholarship to state college, where he became the league leading
scorer during his junior year. When his mother came to visit for a game late in
the season, she noticed that Brian's neck appeared much larger on one side than
the other. The next day, she convinced him to see the college physician who
felt multiple enlarged lymph nodes in his right neck extending from the angle
of the jaw down to just above his collarbone. The doctor ordered a CT scan of
the neck and chest that confirmed multiple abnormal lymph nodes in the right
side of the neck as well as the center of the chest. Brian was referred to a
general surgeon who removed one of the larger lymph nodes in his neck during an
outpatient surgery. This excision biopsy revealed Hodgkin lymphoma.
Following
his diagnosis, Brian was sent for a bone marrow biopsy which was negative. He
also underwent a PET/CT scan that confirmed abnormal activity in the right neck
and chest in multiple lymphomas cancer nodes. Brian was referred to a medical oncologist
who recommended treatment with ABVD chemotherapy. He received 4 cycles which he
tolerated very well, with only moderate fatigue. A restaging PET/CT scan
revealed no residual abnormal activity. He was seen by a radiation oncologist
who recommended low dose involved field radiation therapy (IFRT) after
chemotherapy which was delivered over 3 weeks. Other than a mild sore throat,
Brian tolerated RT quite well. He was seen every 3-6 months by his medical and
radiation oncologist for alternating follow-up visits. Brian has been free of
disease for 7 years.
BASICS
Hodgkin's
lymphoma (HL) is much less common than non-Hodgkin lymphoma (NHL), though it
may be diagnosed in children as well as the elderly. If the disease is
localized, then the 5-year survival is over 90%. Even patients with more
advanced HL have a 5-year survival rate of 75-80%
RISKS
& CAUSES
People
who have history of a first degree relative diagnosed with HL are at a
significantly increased risk of developing the disease themselves. In addition,
patients who have Epstein-Barr virus (EBV) infection early in life appear to
have an increased risk of HL later in life. There appears to be a correlation
with patients of low socioeconomic status also.
SIGNS
& SYMPTOMS
The
vast majority of patients with HL will come to the doctor with the complaint of
an enlarged lymph node or multiple lymph nodes that won't go away. Most
frequently the node will arise in the neck, but may also be felt in other
common lymph node areas including the maxilla (under the arm) and the groin.
Doctors should also ask questions regarding unexplained weight loss, fevers, or
drenching night sweats, the "B symptoms" that are classic for
lymphoma. Although they are only present in a subset of patients, B symptoms
tend to predict for more advanced disease. Rarely, patients may present with
diffuse itching or flushing of the skin when drinking alcohol as an initial
sign of HL.
DIAGNOSIS
Like
NHL, the preferred method of biopsy for HL is complete surgical removal of an
enlarged lymph node (excision biopsy) whenever feasible and safe. There are
multiple subtypes of HL including: nodular sclerosing, mixed cellularity,
lymphocyte rich and lymphocyte depleted. Yet another subtype, called nodular
lymphocyte predominant HL, appears to be biologically different from the
others, but also has an extremely high cure rate.
STAGING
Standard
laboratory evaluation should include complete blood count, serum chemistries
including kidney and liver function, blood levels of lactate dehydrogenase
(LDH) and erythrocyte sedimentation rate (ESR), the latter two of which have
been shown to predict for more advanced disease when elevated. As with NHL, the
Ann Arbor staging system is used. This system is based on the number and sites
of involvement in lymph nodes and other organs, as well as the presence or
absence of B symptoms. Imaging should include CT of involved areas including
neck, chest, abdomen and pelvis. Wherever available, PET/CT is extremely useful
for staging, radiation therapy (RT) planning, and assessing response to
treatment. Bone marrow biopsies are indicated for patients with advanced
disease including those who present with B symptoms. Other factors that may
negatively impact outcome include male sex, age older than 45, low serum
hemoglobin, high white blood cell count, low albumin, and stage IV disease.
TREATMENT
Like
NHL, HL is treated with a combination of chemotherapy drugs. In the case of HL,
four drugs are utilized most commonly in the United States: Adriamycin,
bleomycin, vinblastine, and dacarbazine. The acronym for the combination is
ABVD. Treatment of tens of thousands of patients over the past few decades with
ABVD has shown consistently excellent results.
Patients
with early stage HL generally receive 2-6 cycles of ABVD. Restaging imaging is
obtained after 2-4 cycles to guide further treatment. PET restaging is
predictive of outcome. The combination of ABVD followed by involved field
radiation therapy (IFRT) yields an excellent chance for cure.
Common
acute side effects of ABVD include fatigue, nausea (usually well-controlled
with medication), mild anorexia, decreased blood counts, and hair loss.
Uncommon but serious late side effects following treatment include heart damage
from Adriamycin, lung damage from bleomycin, and nerve damage from vinblastine.
Chemotherapy does put patients at a slightly increased risk for development of
future cancers, most commonly leukemia or NHL.
Since
low doses and fairly small treatment areas are currently utilized for IFRT,
side affects are much less than decades ago when the doses were higher and
treatment areas were larger. Common acute side effects of IFRT include mild
fatigue, possible partial alopecia (hair loss), and sore throat or difficulty
swallowing, depending on the area treated. There is a risk of pneumonitis
(inflammation of the lung) characterized by low-grade fever, dry cough, and
shortness of breath with exertion, that occurs classically 1-3 months after RT.
The 5-10% of patients who develop lung inflammation usually has resolution of
their symptoms within 3-4 weeks after start of steroids. Long-term, despite the
low doses and small RT fields, there remains a small risk of developing a
radiation-induced cancer years after treatment. Patients should be encouraged
to quit smoking prior to RT. Teenage girls and young women who require RT to
the chest should begin annual mammogram (and usually breast MRI) screening
within 7-10 years following treatment or at age 40, whichever comes first.
No comments:
Post a Comment