Inter-hospital Conference 20 March 2012
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Transcript of Inter-hospital Conference 20 March 2012
Inter-hospital Conference20 March 2012
Hematology/Oncology Department of PediatricQueen Sirikit National Institute of Child Health Hospital
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Present illness
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พามิาตุรวจท� รพ.เด็ก ตุรวจร%างกาย subcostal retraction,pulsus paradoxus 20 mmHg, distant heart sound, wheezing both lungs, liver 2 cm. below RCM
Past History
• Enlargement of cardiac shadow
• CT ratio = 0.65 • No pulmonary
infiltration is seen
CXR
• 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
Past History
• 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
Physical examination
• Previous treatment for TB pericarditis
• Progressive dyspnea• Cardiac tamponade
Problem lists
investigation
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 101mmol/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%
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
CTAbdomen
CTAbdomen
CTAbdomen
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– 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
Patel J, et al, Annual of Oncology 2010: 21; 1041-1045
•Patho: small lymphocytes infiltrattion of RV and LV, stained positively for CD45, CD3, CD8 and TdT
•Dx: T-cell Lymphoblastic Lymphoma
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: 1546-1555Patel J, et al, Cardiovascular Pathology, 2010;19:343-352
Patel J, et al, Annual of Oncology 2010: 21; 1041-1045