1. T CELL LYMPHOMA 報告INTERN 許士盟 指導老師 VS 陳建旭
Intern seminar
T CELL LYMPHOMA
報告INTERN 許士盟
指導老師 VS 陳建旭

2. Identification Name: 黃XX Age: 13 years old boy
Admission date: 96/10/17

3. Chief complaint
Progressive dyspnea and Chest tightness for 2 weeks

4. Present illness Past history of asthma, mitral valve prolaps 1m ago
SOB, cough, Orthopnea was noted and progressed in recent 1 month
10/17
To our ER due to severe cough and orthopnea at night
→ Wheezing was noted, treated as asthma
→ Cardiac echo showed mild pericardial effusion
→ Admitted to our general ward

5. Past history Birth History: G1P1, GA: full term, BBW: not known
Apgar score: not known　　　
Feeding: on full diet
Vaccine: all on schedule
Family history: No allergic disease
No history of malignancy
No drug allergy, no food allergy

6. Physical examination Consciousness: clear Appearance: ill looking
Vital signs: T/P/R: 37.2/114/21 BP: 114/61
Conjunctiva: not pale Sclera: not icteric
Neck: soft, no palpable lymph nodes
JVD(-)
Chest: symmetric expansion,
Mild end-expiratory wheezing
Heart: RHB; murmur(-)

7. Physical examination Abdomen: soft and flat.
Tenderness(-) No rebounding pain
Normoactive bowel sound
Liver: impalpable Spleen: impalpable
Extremities : Freely movable, no pitting edema
No palpable lymph nodes

8. CBC
WBC H 10.8 K/cmm | RBC H 5.96 M/cmm Hb L 12.8 g/dl | Hct 39.8 % MCV L 66.8 fl | MCH L 21.5 pg MCHC L 32.2 g/dl | RDW H 17.2 % Pl 256 K/cmm | Blast . % Pro . % | Myelo . % Meta . % | Band 3 % Seg H 72 % | Eos . % 0-6 Baso . % 0-1 | Mono 9 % 3-9 Lymph L 16 % | Aty-lym . % NRBC . % | Remarks .

9. Biochemistry
CREA 1.0 mg/dL | BIL-T 0.3 mg/dL BIL-D 0.0 mg/dL | ALK-P H 183 U/L AST 26 U/L 0-39 | BUN 14 mg/dL 7-21 LD H 293 U/L | CA 9.5 mg/dL P H 4.6 mg/dL | NA 142 mmol/L K 3.8 mmol/L | CL 105 mmol/L ALT 9 U/L 0-54 | ALBUMIN H 5.4 g/dL 3.5-5

10. CXR
96/10/17
96/10/17
96/6/14

11. Tentative diagnosis Mediastinal mass Suspect Thalassemia Plan
Suspected lymphoma
Suspected teratoma
Suspected thymoma
Suspect Thalassemia
Plan
 Arrange CT scan
 Arrange bone marrow biposy

12. CT

13. PICU since 10/17 Arranged CT-guide biopsy
Transferal to PICU on 10/17 night due to orthopnea and dyspnea progressed

14. On 10/18
Change to Echo-guided biopsy under local anesthesia, sitting position
intolerance of lying down in CT room
Frozen report
Small blue round cells suspected Lymphoma or Neuroblastoma
Steroid was given since 10/18
Arranged
whole body bone survey
Bone marrow aspiration and biopsy

15. Pathologic Diagnosis Precursor T-lymphoblastic lymphoma/leukemia.
Mediastinal mass PATHOLOGIC DIAGNOSIS:
Precursor T-lymphoblastic lymphoma/leukemia.
Bone marrow PATHOLOGICAL DIAGNOSIS:
Precursor T-lymphoblastic lymphoma involvement
Bone scan: no evidence of bone metastasis

16. Final diagnosis
T lymphoblastic lymphoma with bone marrow involvement, stage 4

17. 10/18 1653 CxR After biopsy Air hunger and cyanosis found Intubation
Decreased breathing sound of left lung suspected collapse
Gas: PH: PO2:24.9
PCO2: BE:18.1
cHCO3:44.4 sO2:34.3%

18. 10/
PH: 7.562
PO2: 147.9
PCO2: 26.8
BE: 0
cHCo3: 23.6
sO2: 99.6%

19. 10/19 0345 ET tube dislocation Since that Both lung open
Pushed the ET tube deeper
PH: PO2:269.1
PCO2: 40.6 BE:0.5 cHCO3: 25.2
sO2:99.9%
Since that
CO2 retention frequency↓
Tidal volume↑ to 500
No need of frequent ambu-bagging

20. Following course 10/23 Extubation 10/24
Transferred to general ward, continued chemotherapy
No orthopnea, dyspnea, tachypnea
11/10
MBD

21. Summary
13 y/o boy, SOB, cough, Orthopnea was noted and progressed in recent 1 month
Chest x-ray and CT showed anterior mediastinal mass
Biopsy showed T lymphoblastic lymphoma with bone marrow involvement stage 4
Cyanosis and dyspnea after biopsy->intubation
Adjust ET tube depth to adequate location->general condition improve->MBD on 11/10

22. Discussion Critical presentation of mediastinal neoplasms
Investigate mediatinal neoplasms
Management of mediastinal neoplasms

23. Critical presentation of mediastinal neoplasms
Compression of vital structures of superior mediastinum
Superior mediastinal syndrome: S/S refer to compression or obstruction of trachea
Superior vena cava syndrome: S/S refer to compression or obstruction of the superior vena cava
In children, malignant lymphomas are the second most common cause of SVCS
1st: after surgery for congenital heart diseases.

24. Superior vena cava syndrome
S/S:
coughing, hoarseness
dyspnea, orthopnea
chest pain
less common:
anxiety
confusion
lethargy
headache
distorted vision
syncope

25. Investigation
Sedation, anaesthesia or even the supine position for imaging may precipitate rapid decompensation and a sudden cardio-respiratory arrest
Established by the least invasive means
Erect PA and lateral chest X-rays: anterior/middle mediastinal mass
CBC/DC: leukemia.
BUN/Cr, K, P, urate and LDH↑: malignant tumor

26. Investigation with local anaesthetic:
bone marrow biopsy
pleural or pericardial aspiration or
lymph node biopsy may be done
safely with local anaesthetic in a co-operative child

27. Risk Factors of respiratory failure during anesthesia
Orthopnea,
upper body edema,
great vessel compression,
main stem bronchus compression
Clinical and diagnostic imaging findings
predict anesthetic complications in children presenting with malignant mediastinal masses
DORALINA L. ANGHELESCU MD, etc, Pediatric Anesthesia
Volume 17 Issue 11 Page , November 2007

28. Recommended anaesthetic techniques
Spontaneous respiration
Continuous positive airway pressure
Keeping the head of the bed elevated
partial or even full right lateral decubitus position
Without the use of muscle relaxants before intubation
Use of a laryngeal mask airway
The use of a helium–oxygen mixture
Anaesthetic management for the child with a mediastinal mass
Gregory B. Hammer MD, Pediatric Anesthesia
Volume 14 Issue 1 Page 95-97, January 2004

29. Empirical treatment General anaesthetic is not considered safe
Diagnostic material cannot be obtained under local anaesthetic
Chemotherapy+steroids is often rapidly effective and would be the treatment of first choice in children
Impair accurate histological diagnosis and result in suboptimal treatment

30. Back to our patient SVC syndrome, CT showed trachea compression
→High risk for general anesthesia
We choose local anesthesia for biopsy
Still respiratory failure
Intubation
If intubation failure, how can we do?
ECMO
Tracheal stent

31. ECMO 10-yr-old male patient:
dyspnea, orthopnea, and low-grade fever for 3weeks
chest x-ray: a large mediastinal mass
Increasing Large bilateral pleural effusions and pericardial effusion noted by CT
A child with anterior mediastinal mass supported with venoarterial
extracorporeal membrane oxygenation
Talya K. E. Frey, BS, CCP,etc; Pediatr Crit Care Med 2006 Vol. 7, No

32. Decreased preload leading to poor cardiac output
Respiratory insufficiency due to pleural effusions
CPR for 4 mins, Intubation
Consulted CVS On VA-ECMO
Biopsy: T lymphoblastic lymphoma
IV chemotherapy: high-dose steroids, cyclophosphamide, and vincristine

33. He was extubated 4days later
weaned off ECMO after 6 days
Tumor appeared radiographically improved,
Clinical status improved.
Discharged to home on day 28 of hospital stay.

34. Tracheal stent 17 children (10 boys and 7 girls)
Aged: 2 months to 16 years
14 y/o girl, mediastinal mass compressing carina
Removed day 1
24 months well
The long-term outcome remains uncertain
Tracheobronchial obstruction in children: experience with endoscopic airway stenting
Pankaj Kumar FRCS, Andrew P. Bush FRCP,etc The Annals of Thoracic Surgery, Volume 75, Issue 5, May 2003, Pages

35. Summary
Patient with SVC syndrome has high risk of respiratory failure during general anesthesia
Minimal invasive investigation
Empiric therapy should give if pathologic diagnosis can not obtain immediately
If respiratory failure cannot correct by intubation
Consider ECMO or tracheal stent