1. Combine Conference (8, Jan. 07’ ) Case report Case report Reporter :NS R2 洪培恩 Reporter :NS R2 洪培恩 R3 吳孟庭 R3 吳孟庭

2. Case 1 方 X, 68 Y/O, male 方 X, 68 Y/O, male ID: 001876582H ID: 001876582H Admission date: 95/12/12 ~ Admission date: 95/12/12 ~ Chief complaint: Progressive weakness of R't side limbs and dizziness with falling down accident for 5 days Chief complaint: Progressive weakness of R't side limbs and dizziness with falling down accident for 5 days

3. History 1. Personal Hx: Smoking 1PPD for 30 years Past Hx: Hypertension for 10+ years Past Hx: Hypertension for 10+ years 2. He was brought to 台大雲林分院 and brain MRI on 95/12/11 revealed L't parietal and frontal tumor lesion, suspected metastatic tumor. 3. Visit our NS OPD, Cons’ alert, MP:R‘t side limbs,Gr 2 4. Admission for further evaluation and treatment

4. Neurologic Examination Consciousness: E4V5M6 Consciousness: E4V5M6 Pupil: R/L: 3.0/3.0 without light reflex Pupil: R/L: 3.0/3.0 without light reflex Babinski sign: bilateral no response Babinski sign: bilateral no response No R-L disorientation, no dysphagia No R-L disorientation, no dysphagia Increased DTR, MP- RUE/RLE:Gr 2/2 Increased DTR, MP- RUE/RLE:Gr 2/2 CN: intact CN: intact Sensory function: intact Sensory function: intact

5. DateNa (mEq/l)K (mEq/l)Cre (mg/dl)Ca(mg/dl) 144 4.1 0.7 8.6 DateWBC (/cumm) Hb (g/dl) Platelet (/cumm) Neu (%) Lym (%) 15000 13.0 187K 81.4 11.5 Tumor markers AFP 1.32 ng/ml CA-199 13.30 Unit/ml CEA 2.78 ng/ml SCC < 0.3 ng/ml Free-PSA 0.57 ng/ml LaboratoryData

6. Image study CxR: Slight prominent bil. hilar regions CxR: Slight prominent bil. hilar regions Sono,Abdomen: non-significant finding Sono,Abdomen: non-significant finding Chest CT(contrast) on 12/14: Chest CT(contrast) on 12/14: Bullous formation in left upper lung, small subpleural infiltration in both lowers Bullous formation in left upper lung, small subpleural infiltration in both lowers Small lymphadenopathy in subaortic region of Small lymphadenopathy in subaortic region of mediastinum mediastinum

7. Operation (mass effect and for diagnosis) 2006/12/18 Left F-T-P craniotomy with removal of tumor under Brainlab navigator 2006/12/18 Left F-T-P craniotomy with removal of tumor under Brainlab navigator OP finding OP finding Gray brown soft tissue mass was found about 3 and 5 cm in size over left frontal and parietal region, respectively Gray brown soft tissue mass was found about 3 and 5 cm in size over left frontal and parietal region, respectively Froxen section: high grade malignancy Froxen section: high grade malignancy

8. Post op course Seizure episode x 1 (Depakene level: 39.9 ug/ml) Seizure episode x 1 (Depakene level: 39.9 ug/ml) Biopsy report: Peripheral T-cell lymphoma, unspecified. Biopsy report: Peripheral T-cell lymphoma, unspecified. Consult CCRT  WBRT(2000cGy/8fr.) start on 12/27 Consult CCRT  WBRT(2000cGy/8fr.) start on 12/27 For complete lymphoma staging  For complete lymphoma staging  CT,abdomen: No abnormal LAPs in the abdomen CT,abdomen: No abnormal LAPs in the abdomen Gallium scan: Increased uptake in the bil. pul. Hili Gallium scan: Increased uptake in the bil. pul. Hili Bone marrow examination: not representative, No lymphoma is seen Bone marrow examination: not representative, No lymphoma is seen

9. Further treatment Rehabilitation Rehabilitation Suggest chemoport placement and systemic chemotherapy Suggest chemoport placement and systemic chemotherapy

10. Final diagnosis Peripheral T-cell lymphoma with CNS metastasis Peripheral T-cell lymphoma with CNS metastasis

11. Peripheral T Cell Lymphoma They represent 7% of all cases of non-Hodgkin's lymphoma. They represent 7% of all cases of non-Hodgkin's lymphoma. T-cell prolymphocytic leukemia  a high white blood cell count, usually characteristic prolymphocytic morphology, and expression of surface CD3 with either CD4 or CD4 and CD8 T-cell prolymphocytic leukemia  a high white blood cell count, usually characteristic prolymphocytic morphology, and expression of surface CD3 with either CD4 or CD4 and CD8 T-cell large granular lymphocytic leukemia  most often CD3, CD8, CD57, and TIA-1 expression. T-cell large granular lymphocytic leukemia  most often CD3, CD8, CD57, and TIA-1 expression. aggressive NK-cell leukemias, and aggressive NK-cell leukemias, and adult T-cell lymphoma/leukemia.. adult T-cell lymphoma/leukemia..

12. Clinical Characteristics Median Age, years : 61 y/o Median Age, years : 61 y/o B Symptoms, % : 50% B Symptoms, % : 50% Bone Marrow Involvement, % : 36% Bone Marrow Involvement, % : 36% Gastrointestinal Tract Involvement, % : 15% Gastrointestinal Tract Involvement, % : 15% % Surviving 5 years : 25% % Surviving 5 years : 25%

13. Diagnosis Mostly CD4+, but a few will be CD8+, both CD4+ and CD8+, or have an NK cell immunophenotype. Mostly CD4+, but a few will be CD8+, both CD4+ and CD8+, or have an NK cell immunophenotype. No characteristic genetic abnormalities have yet been identified, but translocations involving the T cell antigen receptor genes on chromosomes 7 or 14 may be detected. No characteristic genetic abnormalities have yet been identified, but translocations involving the T cell antigen receptor genes on chromosomes 7 or 14 may be detected. human T-cell lymphoma/leukemia viruses I and II (HTLV-I and HTLV-II), Epstein-Barr virus (EBV), and human herpesvirus 8 (HHV- 8) sequences as previously described. human T-cell lymphoma/leukemia viruses I and II (HTLV-I and HTLV-II), Epstein-Barr virus (EBV), and human herpesvirus 8 (HHV- 8) sequences as previously described. The neoplastic nature of a T-cell infiltrate is established by histologic features in combination with failure to determine an inflammatory cause and demonstration of monoclonality of T-cell receptor genes. The neoplastic nature of a T-cell infiltrate is established by histologic features in combination with failure to determine an inflammatory cause and demonstration of monoclonality of T-cell receptor genes.

14. Immunohistochemistry Indirect biotin-avidin method --detect the antigens. Indirect biotin-avidin method --detect the antigens. Monoclonal antibodies used in the tests were for detecting CD20, CD30, and anaplastic lymphoma kinase (ALK) ; CD3, CD4, CD8, CD56, CD57, Ki-67, granzyme B, pancytokeratin, and glial fibrillary acidic protein and TiA-1. Monoclonal antibodies used in the tests were for detecting CD20, CD30, and anaplastic lymphoma kinase (ALK) ; CD3, CD4, CD8, CD56, CD57, Ki-67, granzyme B, pancytokeratin, and glial fibrillary acidic protein and TiA-1.

15. Stage evaluation of patient Physical examination Physical examination Documentation of B symptoms Documentation of B symptoms Laboratory evaluation Laboratory evaluation Complete blood counts Complete blood counts Liver function tests Liver function tests Uric acid Uric acid Chest radiograph Chest radiograph Calcium Calcium Serum protein electrophoresis Serum protein electrophoresis Serum B 2 -microglobulin Serum B 2 -microglobulin CT scan of abdomen, pelvis, and usually chest CT scan of abdomen, pelvis, and usually chest Bone marrow biopsy Bone marrow biopsy Lumbar puncture in lymphoblastic, Burkitt's, and diffuse large B cell lymphoma with positive marrow biopsy Lumbar puncture in lymphoblastic, Burkitt's, and diffuse large B cell lymphoma with positive marrow biopsy Gallium scan (SPECT) or PET scan in large-cell lymphoma Gallium scan (SPECT) or PET scan in large-cell lymphoma

16. Treatment Treatment regimens are the same as those used for diffuse large B cell lymphoma, but patients with peripheral T cell lymphoma have a poorer response to treatment. Treatment regimens are the same as those used for diffuse large B cell lymphoma, but patients with peripheral T cell lymphoma have a poorer response to treatment.

17. Thank you for attention