Transcript Clinical Pathological Conference

Clinical Pathological
Conference
Shrujal Baxi, M.D.
Chief Resident
Department of Medicine
November 9, 2007
Chief Complaint
An 83 year-old man presents with
three days of intermittent chest pain
History of Present Illness
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Six months prior to admission when he noted
decreased exercise tolerance and was found to have
a normocytic anemia thought to be Myelodysplastic
syndrome, but no work up done at that time
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About five months prior to admission, pt noted a
nonproductive, chronic cough that was worse in
evenings and relieved with prn albuterol therapy
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One month prior to admission, the patient again
started experiencing increasing shortness of breath.
5-10lb weight loss over last few months, night
sweats, subjective fevers
History of Present Illness
On day of admission, pt presented with three days
of intermittent chest pain that was substernal
and radiated to his left arm and shoulder. It was
sharp and stabbing in nature and worse with
inspiration. The episodes would last hours and
were variably relieved with sublingual
nitroglycerin.
Past Medical History:
Hypertension ≥ 20 years
Diabetes ≥ 10 years
Hypercholesterolemia ≥ 10 years
Past Surgical History:
Appendectomy
Medications: (outpatient)
Glyburide
Ramipril
Atenolol
Erythropoietin and iron
albuterol prn
Allergies: none
Family History:
Brother died at 55 of MI. No family history of
malignancy, inflammatory conditions
Social History:
Born in the United States, patient fought in East
Asia during World War II. He has no recent
travel.
50 pack year tobacco history, quit 35 years ago. No
alcohol use. No illicit drug use. Pt lives with
wife in upstate New York. Pt worked in
construction prior to retiring at the age of 69.
ROS: otherwise noncontributory
Physical Exam
General: Well developed male with evidence of
respiratory distress who appears younger than
stated age
Vital Signs: BP 105/68 HR 120, regular, RR 20, Temp
98.2, SpO2 92% room air
HEENT: Oropharynx clear and dry
Lymph Nodes: No cervical, axillary or inguinal
lymphadenopathy
Neck: Supple, jugular venous distention difficult to
assess
Physical Exam
Pulmonary: Decreased breath sounds at bases, 1/3
up bilaterally. Dull to percussion
Heart: Decreased heart sounds, tachycardic,
regular rhythm, pulsus paradoxus of 22
Abdominal: Soft, nontender, nondistended, normal
bowel sounds, with liver span of 14cm and dullness
in Traube’s space
Extremities: No peripheral edema, 2+ peripheral
pulses
Skin: No rashes, no purpura, no petechia
Admission Labs
Laboratory
On Admission
Reference Range
Hemoglobin (g/dl)
10.1
13-18
Hematocrit (%)
29.5
40-52
White Cell Count (per mm3)
7,200
4,500-11,000
Neutrophils
53
42-75%
Lymphocytes
22
20-50%
Monocytes
7
2-12%
Eosinophils
18
0-7%
Mean Corpuscular Volume
83.2
80-95
Platelet Count (per mm3)
195,000
150-450,000
MVP
7.3
7.5-10.5
Partial-thromboplastin time, activated (sec)
33.6
23.3-35.6
Prothrombin time (sec)
18.2
10.0-13.8
INR
1.5
.9-1.2
Lactate Dehydrogenase/LDH
348
110-225
Differential Count (%)
Admission Labs
Laboratory
On Admission
Reference Range
Sodium (mmol/liter)
141
135-145
Potassium (mmol/liter)
4.1
3.5-5.0
Chloride (mmol/liter)
104
100-110
Carbon dioxide (mmol/liter)
28
24-32
Urea nitrogen (mg/dl)
21
6-22
Creatinine (mg/dl)
.7
.4-1.2
Glucose
95
65-115
Calcium (mg/dl)
8.5
8.5-10.5
Magnesium (mmol/liter)
0.8
0.7-1.0
Phosphorus (mmol/liter)
2.9
2.6-4.5
Aspartate aminotransferase (U/liter)
25
10-42
Alanine aminotransferase (U/liter)
18
10-42
Total Bilirubin (g/dl)
2.6
0.1-1.2
Alk Phos
109
42-121
Total Protein (g/dl)
6.1
6.4-8.2
Albumin (g/dl)
4.2
3.8-5.1
EKG
Upon Admission
A prompt cardiac evaluation revealed a moderate to
large pericardial effusion with right atrial collapse
with a question of a right atrial mass. Pt was
admitted to CCU for further evaluation. A
diagnostic procedure was performed…
T1
T2
STIR
PATHOLOGY
Dr. Hui Tsou
Clinical Assistant Professor
Department of Pathology
Final Diagnosis
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Diffuse Large B-Cell Lymphoma
(DLBCL) with primary cardiac
involvement
- CD45+, CD20+
- CD3-, CD15-, CD30-, CD10-
Primary Cardiac Tumors
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Prevalence-.002-.025% at autopsy
75% benign in nature
Systemic embolization is presenting
symptom in 25-50% of cases
Metastatic tumors 10-40X more
likely than primary tumor
Primary Cardiac Tumors
Benign (75% of all cases)
 Myxoma
 Rhabdomyoma
 Fibroma
 Teratoma
Malignant (25% of all cases)
 Sarcoma (majority)
• Angiosarcoma
• Rhabdomyosarcoma
 Lymphoma
 Histiocytoma
Primary Cardiac Lymphoma (PCL)
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Defined as presence of Non-Hodgkin’s
Lymphoma confined to the heart or
pericardium
PCL represents <2.0% of 1° cardiac
tumors and 0.5% of extranodal
lymphomas
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More common in immunocompromised
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Increased incidence due to AIDS and
improved imaging techniques
Lymphoma
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Now the 5th most common cancer
diagnosed in both men and women
Represent 4% of all cancers
Approximately 63,000 cases diagnosed
annually
Age at diagnosis is 60 with more than
50% over the age of 65
5 year survival is 63% and 10 year
survival is 49%
Pathophysiology
Pathophysiology
Assignment of Human B-Cell Lymphomas to Their Normal B-Cell Counterparts
Kuppers R et al. N Engl J Med 1999;341:1520-1529
Pathophysiology
PCL
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Common presentations of this uncommon
diagnosis are based on location of tumor
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Right-sided heart failure
Precordial chest pain
Pericardial effusion
Superior vena cava syndrome
Arrhythmia
CHF
Constitutional Symptoms
Pathogenesis of Disease
Tumor Mass from
replicating atypical
lymphoma
cells
Environmental
Factors
Mutation to
Oncogene
of Lymphoid Cell
Release of
Cytokines
(TNF, IL-6)
Tissue invasion of right atrium
and septal wall
Pericardial Effusion
Atrial Fibrillation
Night
Sweats
Weight
Loss
Anemia of
Chronic Disease
Pleural effusions
cough
dyspnea
chest pain
fatigue
Diagnostic Studies
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Labs:  LDH,  IL-2,  ESR
ECG: AV block, RBBB, Inverted T waves,
Low voltage
CXR: Pleural Effusion and/or Cardiomegaly
Echocardiography:
• Hypoechoic masses in the R atrium with pericardial
effusion
• TTE: difficulty visualizing pulmonary vessels, SVC, R
atrium
Diagnostic Studies
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CT
• Appears hypodense or isodense relative to adjacent
myocardium
• + Contrast: heterogenous enhancement
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MRI
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T1 images: Hypointense and Dark
T2 images: Hyperintense and Bright
+ Gadolinium: Heterogenous enhancement
Useful in making diagnosis and assessing response to RX
Nuclear medicine techniques
• Gallium 67
• Technetium-99m hexakis-2-methoxyisobutyl isonitrile
• Thallium-201
Diagnostic Studies
Tissue is the Issue…
 Pericardial fluid
• Diagnostic in 67 % of cases
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Tissue biopsy
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Mediastinoscopy
Thoracoscopic biopsy
TEE guided biopsy
Endomyocardial transvenous biopsy
Exploratory thoracotomy
Treatment
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Treatment for DLBCL is the chemotherapy
regimen of R-CHOP
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R=Rituximab
C=Cyclophosphamide
H=Adriamycin
O=Vincristine
P=Prednisone
Alternative regimens include:
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COP
CHOP
Bone Marrow Transplant
Follow-Up
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Upon admission, pt had pleural and
pericardial drains placed
While work-up continuing, patient
developed rapid afib controlled with lowdose b-blocker
Due to concern of significant atrial wall
involvement of disease, first 2 cycles of RCHOP given in CCU setting with
continuous cardiac monitoring
Patient is currently disease free after
receiving a complete course of R-CHOP
Thank you…
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Dr.
Dr.
Dr.
Dr.
Dr.
Dr.
Dr.
Srichai-Parsia
Kahn
Hui Tsou
Blaser
Grieco
Ballard
Mark Fisch