1. 허우영·차재명·이정일·주광로·정성원·신현필·
대한소화기내시경학회 월례 집담회
허우영·차재명·이정일·주광로·정성원·신현필·
경희대학교 동서신의학병원 내과학교실

2. CC & PI CC : Exertional dyspnea, dizziness Remote onset: 2 months ago
Recent aggravation: 1 months ago
PI:A 60-year-old man presented to the emergency department with complaints of dyspnea and dizziness. Two months ago, he underwent an upper endoscopy, colonoscopy and cardiac single photon emission computed tomography in another hospital and the results were all within normal limits.

3. PMHx, FHx & SHx Past-medical Hx:
DM/HT/Tuberculosis/Hepatitis (-/-/-/-)
allergy(-), operation(-)
Biopsy of a chest wall mass, a fine needle aspiration
of a cervical lymphadenopathy in another hospital
 non-specific finding.
Family Hx: unremarkable
Social Hx: non-smoker
non-drinker

4. Review of systems
General: fever/chill (-/-), weight loss(+) : 7kg/2 mon
HEENT: headache (-),sore throat (-), tinnitus(-)
Respiratory: dyspnea(+), cough/sputum (-/-),
CV: chest pain (-) orthopnea (-), DOE(+)
GI: anorexia/ nausea/ vomiting (-/-/-)
constipation/diarrhea (-/-)
melena/hematochezia (+/-) : 5 days ago
GU: dysuria/urgency/freqeuncy (-/-/+), nocturia (-)

5. Physical examination (I)
V/S: 117/65 mmHg - 79/min - 20/min ℃
height : 177 cm weight : 87.5 kg
G/A: healthy, well-oriented
HEENT: 2cm ovoid mass on the forehead
and right vestibule of the oral cavity
2.5cm ovoid mass in the submandibular area
Chest : Rt. supraclavicular lymphadenopathy
and bilateral axillary lymphadenopathies
without tenderness
2cm ovoid mass on the chest wall

6. Physical examination (II)
Abdomen : slightly distended abdomen
no palpable organomegaly
normoactive bowel sound
tenderness/rebound tenderness (-/-)
Back and Extremities
deformities (-), pretibial pitting edema (-),
CVA tenderness (-)
Neurology: motor/sensory – intact

7. Laboratory Data CBC: 6,800/mm3 - 6.2 g/dl – 297 x 103/mm3
(Seg 72.2% Lympho 17.7%)
Chemical battery:
protein/albumin 6.0/3.7 g/dl, AST/ALT 11/17 IU/L,
ALP/rGT 94/16 IU/L, TB 0.3 mg/dl
LD 192 IU/L CK 60 IU/L
Ca/P 6.8/2.4 mg/dl, cholesterol 93 mg/dl
glucose 125 mg/dl, BUN/Cr 18/1.1 mg/dl
Na:139 mEq/L, K: 3.9 mEq/L, Cl:107 mEq/L

8. S-iron was 13 ㎕/dl S-ferritin 5.4 ng/ml
Total iron-binding capacity 394 ㎕/dl
CEA 0.6ng/mL CA U/mL
Anti-HIV Ab (-)
Urine analysis: WNL, HBsAg/Ab(-/+)
EKG: normal sinus rhythm, Chest X-ray: normal

9. EGD
A friable ulcerofungating mass was noted over 10 cm segment in the proximal jejunum

10. Abdominal CT
Abdominal CT shows a 9.4 x 9.0 cm enhancing lobulated mass originating from the proximal jejunum.

11. SBS
Small bowel series shows a growing exophytic mass (arrows) with central ulcers in the proximal jejunum.

12. Multiple areas of lymphoma infiltration.
About a 2.3 cm ovoid mass in the right submandibular space.
(B, C) Multiple oval or round masses in the musculoskeletal soft tissue
in the chest wall and pectoralis major muscle.
(D) Right supraclavicular lymphadenopathy.

13. Diffuse plasmablastic infiltrate with abundant cytoplasm, round nuclei, and occasionally central locating nucleoli (H-E stain, x400).
The lymphoid infiltrate was positive for MUM1 (Polymer method, x200).
Lambda light chain reactivity is seen in virtually all tumor cells
(Polymer method, x400).
(D) The stain for Ki-67 demonstrates nuclear staining in approximately 70%
of the neoplastic cells (Polymer method, x400).

14. Plasmablastic lymphoma
Final Diagnosis
Plasmablastic lymphoma

15. Plasmablastic lymphoma(I)
A subtype of diffuse large B-cell lymphoma
Male gender
Associated with immunodeficiency
HIV infection
Most patients primary oral lesions
1/3  extra-oral locations
G-I tract : m/c extra-oral site
Progression
Frequently widespread
The central nervous system, abdominal viscera, musculoskeletal soft tissue

16. Plasmablastic lymphoma(II)
The histological appearance
Monomorphic with a diffuse lymphoid infiltrate and a cohesive growth pattern.
The neoplastic cells resemble plasmablasts with eccentric round or oval nuclei that have fine chromatin.

17. Plasmablastic lymphoma(III)
Immunophenotype
Negative for the typical B-cell antigens
e.g., CD20 and CD45
Positive for plasma cell markers
MUM1, EMA, CD38, and CD138
Immunoglobulin light chains in the cytoplasm
Positive staining for lambda, but not kappa
High rate of mitotic activity by the Ki-67 proliferation index

18. Plasmablastic lymphoma(IV)
DDx.
Plasmacytoma
Mature plasma cells without a high rate of mitotic activity.
Diffuse large B-cell lymphomas
Nearly always express CD20, CD45RA and CD79a
Burkitt’s lymphomas
Express CD20, CD45RA and CD79a, the membrane-bound IgM heavy chain isotype

19. Plasmablastic lymphoma(V)
Prognosis
Very poor with a rapidly progressive clinical course.
An optimal chemotherapy regimen has not been established.