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

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

Chief complaint Progressive dyspnea and Chest tightness for 2 weeks

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

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

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(-)

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

CBC WBC H 10.8 K/cmm 3.4-9.1 | RBC H 5.96 M/cmm 4.24-5.56 Hb L 12.8 g/dl 13.5-17 | Hct 39.8 % 39.1-48.9 MCV L 66.8 fl 82.6-97.4 | MCH L 21.5 pg 28.5-34 MCHC L 32.2 g/dl 33.8-35.6 | RDW H 17.2 % 11.6-13.6 Pl 256 K/cmm 138.1-353.4 | Blast . % Pro . % | Myelo . % Meta . % | Band 3 % Seg H 72 % 43-64 | Eos . % 0-6 Baso . % 0-1 | Mono 9 % 3-9 Lymph L 16 % 27-47 | Aty-lym . % NRBC . % | Remarks .

Biochemistry CREA 1.0 mg/dL 0.7-1. | BIL-T 0.3 mg/dL 0.2-1.4 BIL-D 0.0 mg/dL 0-0.3 | ALK-P H 183 U/L 30-110 AST 26 U/L 0-39 | BUN 14 mg/dL 7-21 LD H 293 U/L 100-200 | CA 9.5 mg/dL 8.6-10.1 P H 4.6 mg/dL 2.5-4.5 | NA 142 mmol/L 135-148 K 3.8 mmol/L 3.5-5 | CL 105 mmol/L 98-107 ALT 9 U/L 0-54 | ALBUMIN H 5.4 g/dL 3.5-5

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

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

CT

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

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

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

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

10/18 1653 CxR After biopsy Air hunger and cyanosis found Intubation Decreased breathing sound of left lung suspected collapse Gas: PH: 7.206 PO2:24.9 PCO2: 114.6 BE:18.1 cHCO3:44.4 sO2:34.3%

10/18 1751 PH: 7.562 PO2: 147.9 PCO2: 26.8 BE: 0 cHCo3: 23.6 sO2: 99.6%

10/19 0345 ET tube dislocation Since that Both lung open Pushed the ET tube deeper PH: 7.411 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

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

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

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

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.

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

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

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

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 1090-1098, November 2007

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

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

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

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. 5 479-481

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

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.

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 1579-1586 5

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