Inter-hospital Conference 20 March 2012

งานนำเสนอเรื่อง: "Inter-hospital Conference 20 March 2012"— ใบสำเนางานนำเสนอ:

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

2 ผู้ป่วยเด็กชายไทย อายุ 8 ปี ภูมิลำเนา จ
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 ตรวจร่างกาย subcostal 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/mm 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 g/dl ( ) Globulin g/dl ( ) Total bilirubin mg/dl (< 1.00) Direct Bilirubin mg/dl (<0.10) Indirect bilirubim mg/dl (0-0.5) AST / .ALT 57 / 36 U/L (10-30) ALP 95 U/L ( )

16 Blood Chemistry BUN 8.05 mg/dl Cr 0.46 mg/dl Na 135 mmol/L
K mmol/L Cl 101 mmol/L CO mmol/L Calcium mg/dL Magnesium 0.83 mmol/L Phosphorus 6.0 mg/dl LDH 860 U/L Uric acid 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 No evidence of bony metastasis
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 DDx unopacified 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 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

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:

39 Differential Diagnosis
Primary cardiac neoplasm Secondary cardiac neoplasm Bruce C J, Heart 2011;97:

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:

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:

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;

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;

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;

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 : U/L Schraader E B, et al, SAMJ 1987: 72;

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;

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: Patel J, et al, Cardiovascular Pathology, 2010;19: Patel J, et al, Annual of Oncology 2010: 21;