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Inter-hospital Conference 20 March 2012

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.ปทุมธานี หายใจเหนื่อยมากขึ้น 2 วัน ก่อนมา รพ. Present illness.

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Inter-hospital Conference 20 March 2012

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  1. Inter-hospital Conference20 March 2012 Hematology/Oncology Department of Pediatric Queen Sirikit National Institute of Child Health Hospital

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

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

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

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

  6. 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

  7. 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

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

  9. 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

  10. 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

  11. Problem lists • Previous treatment for TB pericarditis • Progressive dyspnea • Cardiac tamponade

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

  13. 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%

  14. 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

  15. 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)

  16. 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

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

  18. EKG

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

  20. Bone Marrow Aspiration

  21. Bone Marrow Aspiration

  22. Bone Marrow Aspiration

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

  24. Bone Scan No evidence of bony metastasis

  25. CT-Chest

  26. CT-Chest

  27. CT-Chest

  28. 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

  29. CT Abdomen

  30. CT Abdomen

  31. CT Abdomen

  32. CT-Abdomen • Multiple soft tissue densities in abdomen are DDxunopacified bowel loops , but cannot R/O mesenteric mass/node

  33. 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

  34. Pathology • Pericadiectomy: Pericardium

  35. 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

  36. 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

  37. Approach to cardiac mass

  38. 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

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

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

  41. 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

  42. Cardiac Lymphoma RA

  43. 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

  44. 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

  45. 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

  46. 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

  47. 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

  48. 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

  49. 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

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