Today was a fun day in Cardiology, and I was only in from 7a-5p! We actually weren't swamped with consults, which makes for a more manageable day. I spent the afternoon in the cardiac lab. I watched 2-D Echos, Pacemaker implantation, EP-studies (but no ablations today), and finished the afternoon with a cardiac catheterization. This patient presented with shortness of breath and chest pressure, and in the cath lab we found the Right Coronary Artery (RCA) to be 98-99% occluded. So we put in a stent, which opened up the vessel, and her heart was pumping with perfect perfusion once again. It was pretty amazing, to see the dye inject and narrow in the artery, place the balloon and stent, and the flow is as good as new. Exciting stuff! I love to see procedures, I love being able to literally see the patient heal in from of you. So cool! I think I'm coming round to the idea of cardiology...it's at least tolerable now (haha) ;) Pretty amazing, to cure someone just.like.that!
Before:
After:
Another case report completed. Finished with my oncology reports, now I just need to complete a few cardiology case reports - not quite as interesting to me, but it must be done!
Patient identifiers, dates, and other data have been modified to protect patient privacy and confidentiality.
TR is a 62 year old Caucasian female who presents to the
oncology clinic for a 1-month follow up for recurrent follicular lymphoma. On March 12, 2004, TR underwent surgical resection of an extensive retroperitoneal mass,
which revealed pathology of high grade lymphoma, which was most consistent with
Burkitt’s lymphoma. In September 2011, recurrent lymphoma was detected upon
laparoscopic mesenteric lymph node biopsy which showed follicular lymphoma. The
patient denies noticeable waxing and waning of lymphadenopathy, but admits to
abdominal pain particularly in the left lower quadrant. The current management plan for TR is as
follows: R-CHOP x 6 cycles (cycles 2-6 decreased by 20% due to
fatigue) followed by: (600mg) Rituxan 375mg/m 2 weekly x 4 every 6 months;
Management for Recurrence: R-CHOP x 4 cycles, CVP with Rituxan x 2 cycles, (600mg) Rituxan
375mg/2 weekly x 4 every 6 months; Recurrence: Cycles (400mg) Rituxan 250mg/m2, Zavlin
Injection and Rituxan 250mg/m2.
Assessment: Follicular
Lymphoma
Discussion: Follicular lymphoma (FL) is the second most
common lymphoma in the US with an incidence of approximately 2.18 cases per
100,000 persons per year. FL most frequently presents in middle-age and
elderly, with an average age at diagnosis of 60 years. It is slightly more
common in females (1:1.7), and less common in Asians and blacks. The
pathogenesis of FL is not clearly understood, but it is thought to arise from
germinal center B-cells, both centrocytes and centroblasts. Around 85 percent
of patients with FL have t(14;18), which results in the overexpression of B
cell leukemia/lymphoma 2 (BCL-2), an oncogene which blocks apoptosis, leading
to prolonged cell survival. Multiple genetic events are required for the
development of FL since the t(14;18) translocation can be identified in normal
individuals and patients with diffuse large B-cell lymphoma.
Adults with FL typically present with painless peripheral
adenopathy in cervical, axillary, inguinal, and femoral regions, and hilar and
mediastinal nodes are often involved. Waxing and waning lymph node enlargement
is often present long before diagnosis. Some patients, like TR, present with a
large abdominal mass with or without evidence of GI or urinary tract
obstruction. Others may have disease localized to the small intestine, most
commonly the second portion of the duodenum, which is often found incidentally.
CNS involvement is uncommon, but cord compression may develop if it were to
occur. Staging studies usually show widely disseminated disease despite
exhibiting no symptoms with the exception of lymphadenopathy, with overt
involvement of the spleen, liver, and bone marrow in many cases, but
involvement of organs other than lymph or bone marrow is uncommon.
Interestingly, the infamous “B symptoms” of fevers, night sweats, and
unintentional weight loss is only present in about 20 percent of patients with
FL. Continually, there are no characteristic lab abnormalities in FL, and
despite the large tumor burden, serum lactate dehydrogenase is only increased
about 20 percent in patients with FL.
The pathology of FL involves histological examination of the
enlarged lymph nodes, along with immunophenotypic and molecular genetic studies
to support the diagnosis. On histology, enumeration of centroblasts is used to
determine the grade, which influences the treatment modalities. Also of note
histological is the nodular growth pattern, which recapitulates the normal
germinal centers of secondary lymphoid follicles. The interfollicular areas of
FLs, although compressed, also resemble normal lymph nodes in that large
numbers of normal T-cells are often found there, mixed in with variable numbers
of neoplastic cells. Grading of FL is based upon the proportion of centroblasts
(large cells) per high powered field. Grade I is 0-5, Grade II is 6-15, and
Grade III is more than 15 per high powered field. Determining the immunophenotype
also helps to confirm the diagnosis of FL, and most express IgM (40%
demonstrate IgG, rare cases express IgA).
A differential diagnosis for a patient presenting with
lymphadenopathy include reactive follicular hyperplasia, cutaneous follicle
center cell lymphoma, T cell rich large B cell lymphoma, mantel cell lymphoma,
and nodal marginal zone lymphoma in addition to follicular lymphoma.
The diagnosis of FL is based on evaluation of a lymph node
biopsy, and bone marrow examination is done as an important component of
staging (but is not a reliable means of primary diagnosis). Classically, FL on
histology has a distinctly nodular growth pattern and is made up of a mixture
of centrocytes and centroblasts with infrequently seen mitotic or apoptotic
cells. Characteristically, the tumor cells express CD20, CD10, (and >85% of
the time) BCL-6 and are negative for CD5 and CD 23, which can be detected by
FISH or PCR.
The treatment for recurrent FL, as in the case of this
patient, includes the monoclonal antibody, Rituxan, plus chemotherapy. Another
possible adjuvant treatment option is high-dose therapy with transplantation of
autologous stem cells, which has recently been shown to improve survival and
tumor control. However, adverse effects are much higher in patients who undergo
autologous stem cell transplantation.
The prognosis for FL is dependent upon the extent of disease
present at diagnosis. Some patients present with waxing and waning disease for
five or more years without therapy. Other patients with more disseminated
disease and rapid tumor growth require treatment to prevent pain, lymphatic
obstruction, or organ obstruction. There are two ways to measure the prognostic
index for FL, including the Follicular Lymphoma International Prognostic Index
(FLIPI) and tumor grade (I, II, or III). FLIPI uses patient age greater than
60, elevated serum lactate dehydrogenase, performance status, stage, hemoglobin
less than 12 g/dL, and number of extranodal disease (greater than 4) sites to
predict overall survival rates. Recently, a new prognostic index was created,
called FLIPI2. Additionally, there is evidence suggesting that the quality and
quantity of the reactive cell response to FL tumor cells influences biological
behavior and outcome. Several studies have indicated that an immune response
rich in T cells indicates a better prognosis, whereas an immune response
dominated by macrophages indicates a worse prognosis.
References:
Schaaf M, Reiser M, Borchmann
P, Engert A, Skoetz N. High-dose therapy with autologous stem cell
transplantation versus chemotherapy or immuno-chemotherapy for follicular
lymphoma in adults. Cochrane Database of Systematic Reviews 2012, Issue 1. Art.
No.: CD007678. DOI: 10.1002/14651858.CD007678.pub2.
