박 O 호, M/24 병록번호 : Admission: C.C. fever o/s)2 주 전 P.I. 특이병력 없는 자로 내원 2 주 전부터 fever 발생하였고, 6 일 전부터 anorexia, watery diarrhea 발생하여 f/e 위해 외래 통해 입원함. * 2004 년 동일한 증상 있었음. * 거주지 : 과테말라, 군 복무 위해 한국에 와서 1 달 전 제대함. * 최근 여행력 : none

Past Medical History DM/HTN/Tb/Hepatitis(-/-/-/-) Op. History(-) Personal History Smoking/alcohol (-/+) : 소주 0.5 병 / 주 1 회 Family History(-) Past History

Generalfever (+)chill (-)weight change (-) fatigue(-)sweating (-) edema(-) Skinrash (-) itching (-)pigmentation (-) Head/ENTheadache (+)sore throat (-)nasal obstruction(-) otalgia(-)ear discharge(-)hearing disturbance(-) periocular swelling(-) Repiratorycough (-)sputum (-)dyspnea (-) hemoptysis (-) pleuritic pain (-) Review of system - 1 열날 때

Cardiovascularchest pain (-)palpitation (-)orphopnea (-) Gastrointestinalanorexia (+) nausea (-)vomiting (-) diarrhea (+)constipation (-)abdominal distension (-) melena (-)hematochezia (-)hematemesis (-) Urogenitalflnak pain (-)dysuria (-) urgency (-) frequency (-) Neuromuscularnumbness (-)dizziness (-)sleep disturbance(-) weakness (-)sensory loss (-)arthralgia (-) Review of system - 2 watery 6 일전부터

Ht : cmBW : 67.9 kgBMI : 21.7 kg/m2 Initial V/S120/80 mmHg - 88/min - 20/min – 38.7°C – 99% GeneralAlert consciousness Acutely-ill looking appearance Head/ENTIsocoric pupil c pupil light reflex (++/++) Neck vein engorgement (-/-) Neck lymph node enlargement (-/-) clear scelrapinkish conjunctiva ChestSymmetric chest expansion regular heart beat without murmur Clear breathing sound without rale Physical examination - 1

AbdomenSoft and flat abdomen No shifting dullness Normoactive bowel sound Abdominal tenderness/rebound tenderness (-/-) Back/ExtCostovertebral angle tenderness (-/-) Pretibial pitting edema (-/-) Motor/Sensory Physical examination - 2 V V VV 100

Initial Lab Findings - 1  CBC/DC 1,780/ ㎕ (44%) – 13.0 g/dL – 170,000 / ㎕ PT INR 1.23aPTT 48.2 sec  Chemistry TB0.49 mg/dLAST/ALT126/140 U/L ALP/rGT55/53 IU/LProt/Alb6.7/3.6 g/dL BUN/Cr9/1.0 mg/dLNa/K/Cl126/4.4/90 mmol/L Ca/P/Mg7.5/3.1/2.0 mg/dL LD/CK 2,365/92 U/L CRP2.09 mg/dLESR 없음.  PB smear Normocytic normochromic RBCs with anisopoikilocytosis Decreased WBC count, atypical lymphocytes are occasionally seen.

 Urinalysis Occult Blood -Protein -Glucose – RBC 0~1 /HPFWBC 2~4 /HPF  Urine chemistry Urine protein 0/Creatinine 8 Initial Lab Findings - 2

Initial Chest X-ray ( )

Initial EKG

Initial problem list #1. Fever(2 주 ) #2. watery diarrhea #3. elevated CRP (2.09) #4. elevated AST/ALT (126/140) #5. elevated LDH (2,365) #6. hyponatremia (Na: 126) #7. leukopenia (WBC: 1,780(44%))

Initial Assessment & plan #1. Fever(2 주 ) #2. watery diarrhea #3. elevated CRP (2.09) #4. elevated AST/ALT (126/140) #5. elevated LDH (2,365) #6. hyponatremia (Na: 126) #7. leukopenia (WBC: 1,780(44%)) Hepatitis(r/o viral, r/o toxic) Myelodysplastic syndrome Hepatitis viral marker, herbal medicine Hx PB smear, (BM exam) Ferritin level, rash f/u, abdomen CT Autoimmune serologic test(ANA, RF,ANCA) 가을철 발열성 질환 검사 EBV, CMV serologic test Urine chemistry, osmolarity(serum, urine) Diagnostic Plan Broad-spectrum antibiotics Treatment Plan Infectious colitis EBV, CMV infection Leptospira or Rickettsia Still’s disease

Progress note – HD #3 S) Febrile sense(+), diarrhea - improving O) WBC: 1,430(49%) -> 1,080(65%) Procalcitonin: CRP: > 1.33 Malaria (smear/Ag): (-/-) Stool WBC: not found Stool Helminth/Protozoa: (-/-) CDTA: (-) HBV,HIV,HCV,HAV,CMV: all(-) Ferritin: >1,000 ng/ml(15~332)

Progress note – HD #3 ( ) A)#1. r/o Hepatitis -> hepatitis 가능성 ↓. Cryptogenic 가능성 #2. r/o MDS -> 4 년 전 골수 검사 이상 없음. #3. r/o infectious colitis -> CT, stool exam (-). 가능성 ↓ #4. r/o EBV, CMV infection -> 검사 진행 중 #5. r/o Leptospira or Rickettsia -> 검사 진행 중. 가능성 ↓ #6. r/o Still’s disease -> P)#1. ceruloplasmin, anti- mitocholdrial Ab #2. BM exam #4,5. lab f/u #6. autoimmune serologic marker f/u Major ≥ 2 개 총 ≥ 5 개

Progress note – HD #5 ( ) S) Febrile sense(+), rash(-) O) 면역 검사 IgG(694~1618) mg/dL 1590 IgA(68~378) mg/dL187 IgM(60~263) mg/dL175 IgE(~100) IU/mL430 C3(88~201) mg/dL107 C4(16~47) mg/dL45.8 RA factor(~20) IU/mL 10.8 C-ANCAnegative P-ANCAnegative ANANegative 위탁 검사 Ceruloplasmin (16.2~35.6)mg/dL 26 Anti-mitocholdria Abnegative EBV EA-DR IgGNegative EBV EA-DR IgMNegative EBV EBNA IgGPositive EBV VCA IgMNegative CMV culturenegative Anti-CMV IgGNegative Anti-CMV IgMNegative

Progress note – HD #5 ( ) A)#1. r/o Hepatitis -> hepatitis 가능성 ↓. #2. r/o MDS #3. r/o infectious colitis -> 가능성 ↓ #4. r/o EBV, CMV infection -> negative #5. r/o Leptospira or Rickettsia -> 검사 진행 중. 가능성 ↓ #6. r/o Still’s disease -> P)#2. BM exam #6.

Progress note – HD #5 ( )

Progress note – HD #5 ( )

A)#1. r/o Hepatitis -> hepatitis 가능성 ↓. #2. r/o MDS #3. r/o infectious colitis -> 가능성 ↓ #4. r/o EBV, CMV infection -> negative #5. r/o Leptospira or Rickettsia -> 검사 진행 중. 가능성 ↓ #6. r/o Still’s disease with hematologic d/o P)#2. BM exam #6. NSAIDs, Steroid PO naproxen 500 mg bid IV MPD 500mg pulse

Progress note – HD #8 ( ) S)Febrile sense(-) Confused state(+) O)BM exam( ) 1420 IV MPD 500mg (1/4~1/6)

Progress note – HD #8 ( ) A)#1. r/o Hepatitis -> hepatitis 가능성 ↓. #2. r/o MDS #3. r/o infectious colitis -> 가능성 ↓ #4. r/o EBV, CMV infection -> negative #5. r/o Leptospira or Rickettsia -> 검사 진행 중. 가능성 ↓ #6. r/o Still’s disease with hematologic d/o #7. r/o steroid induced psychosis, r/o zolpidem induced delirium P)#2. BM exam #6. NSAIDs, Steroid PO naproxen 500 mg bid IV MPD 500mg pulse(1/4~1/6) -> IV MPD 60mg(1/7~) #7. anti-anxiety(lorazepam), anti-psychotics(quetiapine)

Progress note – HD #12 ( ) O)BM exam( 보고 )

Progress note – HD #12 ( ) O)BM exam( 보고 )

Progress note – HD #12 ( )

Progress note – HD #12 ( )

Progress note – HD #12 ( )

Clinical course 중단 )pip/tazo (1/1~1/5) D5 IV MPD 500mg (1/4~1/6) IV MPD 60mg (1/7~1/9) 50 mg 40 mg 30 mg PO PDL 30 mg Cyclosporine 100mg qd (1/11 ~ 1/14) Cyclosporine 150mg(1/15 ~ ) 아침 / 저녁 100/50mg PO PDL 25 mg BM exam BM 결과보고

Final diagnosis #1. Adult-Onset Still’s disease #2. Hemophagocytic lymphohistiocytosis #3. Steroid induced psychosis