Case Presentation

報告者 : 黃順祥指導者 : 謝宏仁醫師

Basic Data

姓 名 ： 謝承翰病歷號 ： 34316795

年　齡　： 025

性　別　： M

Chief Complaint

Cough with sputum for 5 months

Present IllnessGouty arthritis noted for about 1 year with diet control Cough with sputum since 94/12 Mild dyspnea on exertion in recent 4 monthsBody weight loss 13 kg within recent 1.5 years 新竹馬偕

chest CT scan:multiple enlarged lymph nodes over anterior mediastinum, and a 4X4 cm sized irregular tumor with cavitation over RUL.

Bronchoscopy performed on 95/4/13 showed incomplete obstruction due to external compression over RML and RLL orifice

Lab DataSMAC: UA 11.7

LDH 417 ALK-P 188 G-GT 15 I.P. 5.1

WBC 30800 /CUMM HGB 12.3 g/dl HCT 37.1 % MCV 80.6 fl PLT 665000 /CUMM BAND 0.0 SEG 88.2LYMP 3.8 MONO 2.6 EOS 5.1 BASO 0.3

Lab Datatumor marker:AFP 2.98

CEA 1.91

CA-125 44.46

SCC 4.10

CA-199 2.49

AChRAb 0.51 (0.5)

Beta-HCG 0.05(<10)

IG G: 1950IG A: 354 IG M: 166 Negative:HBsAg-EIA, HBsAb-EIA, H

CV-EIA ANTI-HIV TEST-EIA(NHI) EB VCA IGG, IGM, IGA

Lab Data

First-pass EF and wall motion: 1. LVEF: 62 % (normal range: > 50%). RVEF: 45 % (normal range: > 40%). 2. Wall motion: Normal. 3. Poor venous return is noted in the morning on Apr. 28, 2006, but not so obvious in the study perfomed in the afternoon. SVC symdrome is impressed.

SPIROMETRY PRED ACTUAL %PREDFVC 4.74 1.90 40 FEV1 4.03 1.60 40 FEV1/FVC 86 84

The pulmonary function test shows severely restrictive ventilatory impairment

PRED ACTUAL %PREDDLCO 35.8 18.4 51

The DLCO indicates moderate reduction of gas exchange

ImageCXR

A 8.5cm soft tissue mass noted in RML with consolidation of right lung, in favor of tumor growth with obstruction pneumonitis.

Abdominal CT

1. Small lymph nodes noted at bilateral inguinal regions

2. Splenomegaly

3. Increased soft tissue density at paraaortic region of retroperitoneum

Nuclear MedicineWBBS:

No definite evidence of distant bone metastasis

tumor survey: 1. There is a huge

and irregular mass of heterogeneous

uptake in the right lung, which is compatible with tumor

involvement in this region.

PathologyLung, "anterior mediastinal tumor," CT-guided biopsy---Classical Hodgkin lymphoma Cytology (mediastinal)---Positive for malignant cells, lymphoma is favoredSurgical pathology, bone marrow---hypercellularityCytogenetics-bone marrow ---pending report

Hospital Course4/21: CT guided biopsy: classical Hodgkin lymphoma

4/27: BM biopsy : hypercellularity

4/27: port-A implantation

04-28 : C/T with ABVD

05/03 : discharged

Adriamycin inj 46 MG IVA cardiac toxicity

Bleocin inj 18.5 MG IVA pulmonary fibrosis

Vinblastine inj 11 MG IVA

Dacarbazine inj 693 MG IVA

Hodgkin’s Disease

EtiologyMalignancy of lymphoid tissue , presence of Reed-Sternberg (RS) cell

characteristic way—contiguous spread from lymph node to lymph node, central distribution (mediastinum, neck, etc)

Role of Epstein-Barr virus is suspected

EpidemiologyBimodal distribution of peak incidence: young adults (ages 15-35) (this patient:25) and adults >age 50More prevalent in males, especially young-onset HDRisk in monozygotic twins increases nearly 100-fold

Signs/symptomsMajority are asymptomaticLocalized lymphadenopathy

Central structures: mediastinum, neck/supraclavicularFirm, freely movable, nontender (this patient)may be detected on routine CXR

constitutional symptoms (B symptoms) (this patient)fever >38℃night sweatsweight loss > 10% in 6 months

nonspecific symptoms: rash, cough, chest pain, SOB, bone pain, GI discomfort, fatigue, malaise, pruritismay have disulfuram-like reaction (flushing with alcohol ingestion)

Diagnosisgold standard: lymph node biopsy demonstrating RS cells (large cell with bilobed/multilobed nucleus with prominent nucleoli) in appropriate cellular backgroundflow cytometry: CD15+ and CD30+ on RS cellstaging evaluation after pathologic diagnosis

physical exam with emphasis on lymph nodes, spleen sizepresence of B symptomsLabs: CBC, LFTs, renal function tests, uric acid, ESRChest X-rayCT of thorax, abdomen, and pelvis to document lymphadenopathyBilateral bone marrow biopsy/aspiration to assess for marrow involvementLymphangiograms have generally been replaced by Gallium scans, MRI, and PET scans

Treatment Depends on staging

Stage I: 1 lymphatic structure involvedStage II: >1 lymphatic structure involved, on same side of diaphragmStage III: lymphatic involvement on both sides of diaphragmStage IV: disseminated/marrow involvementB = presence of B symptoms; E = Extralymphatic spreadLocalized disease (stages I-II): radiation therapy

Advanced disease (stages IIB-IV): chemotherapyStandard therapy is ABVD(adriamycin, bleomycin, vincristine, dacarbazine)Salvage therapy for recurrence: MOPP (mechlorethamine, vincristine, procarbazine, prednisone)

Consider autologus bone marrow transplant

Prognosis/clinical courseFour types of HD

Nodular sclerosis(classical): most common; more common in females usually at anterior mediastinum Mixed cellularity: 2nd most common prognosisLymphocyte predominant: uncommon; best prognosisLymphocyte depleted: least common

HD is a curable disease (85% curable with treatment)Post-treatment complications: patients may develop myelodysplasia or myelotenous leukemia 10-15 years following treatment