Inter-hospital Conference 20 March 2012 Hematology/Oncology Department of Pediatric Queen Sirikit National Institute of Child Health Hospital

ผู้ป่วยเด็กชายไทย อายุ 8 ปี ภูมิลำเนา จ ผู้ป่วยเด็กชายไทย อายุ 8 ปี ภูมิลำเนา จ.ปทุมธานี หายใจเหนื่อยมากขึ้น 2 วัน ก่อนมา รพ.

Present illness 4 วัน ก่อนมา รพ. สังเกตุว่าเหนื่อยง่าย ไม่มีไข้ ไอแห้งๆ นอนราบได้ 2 วัน ก่อนมา รพ. หายใจเร็วมากขึ้น ดูเหนื่อย บางครั้ง มีเสียงหายใจดังเหมือนนกหวีด ไอแห้งๆ ท้องอืดมากขึ้น ไม่มีไข้ จึงมา รพ.

Past History 2 เดือน ก่อนมา รพ. เหนื่อยง่ายขึ้น เดินแล้วต้องนั่งพัก ไม่ไอ ไม่มีไข้ นั่งเรียนแล้วหมดสติไป พามาตรวจที่ รพ.เด็ก ตรวจร่างกาย subcostal retraction,pulsus paradoxus 20 mmHg, distant heart sound, wheezing both lungs, liver 2 cm. below RCM

CXR Enlargement of cardiac shadow CT ratio = 0.65 No pulmonary infiltration is seen

Past History CBC: Hb 14.1 g/dl, Hct 42.1%, Platelet 477,000/mm3 WBC 10,800/mm3 (N-65, L-21, E-1, Ba-1, Mo-8, ATL-4%) MCV 86.5 fl, MCH 29.5 pg/cell, MCHC 34.1 g/dl, RDW 12% Echocardiogram: massive pericardial effusion

Past History Pericardial tapping: straw color with fibrin, WBC 850 (Mono 100%), RBC 365 Pericardial fluid Protein 2.44 g/dl, Serum Protein 6.1 g/dl Pericardial fluid sugar 84 mg/dl, Blood Sugar 111 mg/dl Pericardial fluid LDH 351 U/L, serum LDH 849 U/L Pericardial fluid ADA 106, serum ADA 19 U/L Pericardial fluid Culture: no growth, PCR for TB: negative Tuberculin Skin Test : negative 0 mm. Sputum for AFB x 3days: negative

Past History Treat as TB pericarditis: IRZS + Dexamethasone F/U Echocardiogram (1 week after treatment): no pericardial effusion Continue IRZS

Physical examination Vital signs: BT 37oC, RR 28/min., PR 130/min, BP 120/70 mmHg, Pulsus paradoxus BW 29 Kg.(P50-75) Ht 123 cm.(P10-25) General Appearance: A Thai boy, good consciousness, not pale, no jaundice, no neck vein engorged Heart: no active precordium, no distant heart sound, normal S1,S2, no murmur

Physical examination Lungs: expiratory wheezing both lungs Abdomen : no distention, active bowel sound, soft, liver 1 cm. below RCM, spleen was not palpable Extremities: no edema

Problem lists Previous treatment for TB pericarditis Progressive dyspnea Cardiac tamponade

investigation A B C D E F G H I J K L

CBC Hb 14 g/dl Hct 40.8% WBC 16,140/ mm3 (N-94%, L-5%%, M-1%) Platelet 358,000/µL MCV 81.3 fl, MCH 28.9 pg, MCHC 35.4 g/dl RDW 13.5%

U/A Sp.gr 1.005 pH 7.0 Urobilinogen : negative Bilirubin : negative Protein negative Epithelial cell 0-1/HPF WBC 1-2/HPF No RBC

Liver Function Test Total protein 6.18 g/dl (5.7-8.0) Albumin 3.8 g/dl (2.9-4.2) Globulin 2.38 g/dl (1.8-3.2) Total bilirubin 0.51 mg/dl (< 1.00) Direct Bilirubin 0.24 mg/dl (<0.10) Indirect bilirubim 0.27 mg/dl (0-0.5) AST / .ALT 57 / 36 U/L (10-30) ALP 95 U/L (170-420)

Blood Chemistry BUN 8.05 mg/dl Cr 0.46 mg/dl Na 135 mmol/L K 4.53 mmol/L Cl 101 mmol/L CO2 21.8 mmol/L Calcium 8.2 mg/dL Magnesium 0.83 mmol/L Phosphorus 6.0 mg/dl LDH 860 U/L Uric acid 10.85 mg/dl

CXR Enlargement of cardiac shadow Progression of BLL infiltration, combined congestion cannot exclude

EKG

EKG Low voltage in lead I, aVR, aVL and V1 HR 120/min RAE, LAE, no chamber hypertrophy Axis 90o - 120o

Bone Marrow Aspiration

Bone Marrow Aspiration

Bone Marrow Aspiration

Bone Marrow Aspiration Clotted specimen M : E : L = 61 : 12 : 18 Histiocyte 3%, not increased hemophagocytic activity Tumor cell 5%

No evidence of bony metastasis Bone Scan No evidence of bony metastasis

CT-Chest

CT-Chest

CT-Chest

CT-Chest Hypodensity infiltrative mass extending from lower neck, superior-anterior mediastinum, subcarina and hili, posterior aspected of the heart down to diaphram , encasing and compressing mediastinal structures Invasion into LA chamber

CT Abdomen

CT Abdomen

CT Abdomen

CT-Abdomen Multiple soft tissue densities in abdomen are DDx unopacified bowel loops , but cannot R/O mesenteric mass/node

Echocardiogram RAE, LAE Pulmonary vein obstruction due to hypertrophy of Pulmonary vein and extracardiac mass. PV PG 20 mmHg Multiple mass in LA chamber, AV groove Hyperechoic pericardium, no pericardial effusion. LVEF 70% Right pleural effusion 18 mm

Pathology Pericadiectomy: Pericardium

Pathology T lymphoblastic lymphoma is diagnosed Suspected Malignant lymphoma Immunohistochemistry study Positively react with CD3, CD5, CD7 and weekly CD4 CD10, Bcl-2, TdT are positive MPO, CD20, CD34, CD8, CD117, PAX-5 and AE1/AE3 are negative T lymphoblastic lymphoma is diagnosed

Progression Start Dexamethasone 0.6mg/kg/day Set OR for Pericardiectomy Patho: T lymphoblastic lymphoma stage IV Treatment: TPOG-ALL-02-05 F/U Echocardiogram 1 mo after treatment No mass in cardiac chamber Good LV function No pericardial effusion

Approach to cardiac mass

Clinical Features Determined by location of tumor rather than its histological type Rapidly progressive heart failure Arrhythmia Chest pain Cardiac tamponade Superior vena cava syndrome Bruce C J, Heart 2011;97:151-160

Differential Diagnosis Primary cardiac neoplasm Secondary cardiac neoplasm Bruce C J, Heart 2011;97:151-160

J Am Soc Echocardiogr, 2000;13: 1080-3

Primary cardiac neoplasm Assessment of the specific location Endocardium : cardiac myxoma Myocardium : myofibroblastic sarcoma, fibroma, Rhabdomyoma Pericardium: teratoma, mesothelioma, hemangioma, Lymphoma ( Right side heart, multifocal) Grebenc M L, et al, RSNA 2000;20: 1073-1103

Cardiac Lymphoma RA

Secondary cardiac neoplasm Most common malignancies that metastasize to the heart are Carcinomas of lung and Breast Lymphoma Leukemia Pericardium is the most commonly affected site Grebenc M L, et al, RSNA 2000;20: 1073-1103

10-year-old boy presented with progressive breathlessness CXR: marked cardiomegaly Echo: large pericardial effusion Compromising function of the heart Bradycardia after insertion of pericardial drain, cardiac arrest and died Patel J, et al, Annual of Oncology 2010: 21; 1041-1045

10-year-old boy presented with progressive breathlessness Patho: small lymphocytes infiltrattion of RV and LV, stained positively for CD45, CD3, CD8 and TdT Dx: T-cell Lymphoblastic Lymphoma Patel J, et al, Annual of Oncology 2010: 21; 1041-1045

10-year-old boy presented with progressive dyspnea and abdominal pain CXR Echo: massive pericardial effusion, LV decompensation Pericardial tapping Pleural tapping Straw-color fiuld P/S protien ratio: 0.39 P/S LDH ratio : 0.8 Culture: nogrowth AFB: negative Schraader E B, et al, SAMJ 1987: 72; 878-881

10-year-old boy presented with progressive dyspnea and abdominal pain Start IRZS+ Prednisolone 2 wk after treatment Clinical improved, D/C Readmitted 25 days after D/C, progressive dyspnea Pleural and pericardial effusion P/S protien ratio: 0.52 P/S LDH ratio : 0.48 ADA : 11.5 U/L Schraader E B, et al, SAMJ 1987: 72; 878-881

10-year-old boy presented with progressive dyspnea and abdominal pain Cytology: Numerous primitive Lymphocytes CT: medistinal mass Pericardial biopsy Tissue infiltration suggestive of lymphoma Schraader E B, et al, SAMJ 1987: 72; 878-881

Conclusion Primary cardiac lymphoma is very rare. Both B-cell and T-cell lymphoma have been reported RA and RV are the most common sites 20% of NHL presented with pleural effusion High ADA level may be present in pleural effusion cause by TB, SLE, Lymphoma and Leukemia Michael G. Alexandrakis, et al, CHEST 2004;125: 1546-1555 Patel J, et al, Cardiovascular Pathology, 2010;19:343-352 Patel J, et al, Annual of Oncology 2010: 21; 1041-1045