Case conference Welcome Dr. Lawrence Tierney
Case: 18 year-old male CC) hamatomesis, Fever and cough
HPI) 1 st admission One month ago, he admitted to our hospital because of hematemesis. He had weight loss (-4kg) for a few months, appetite loss 2weeks ago, and tarry stool the day before 1 st admission. He had no abdominal pain. He didn t take NSAIDS. Emergency endoscope was done at the emergency department. We diagnosed as bleeding from gastric ulcer. Endoscopic treatment for bleeding ulcer was done. His anemia was so severe that he was transfused 4 units of PRBC. Also, PPI was started since that time. H.pylori was negative. The temperature raised to 38 intermittently during his admission. His general condition was a little fatigue but his fever resolved spontaneously, and he discharged 1 week later. He was followed up by the GI doctor in OPD.
HPI) 2 nd admission He had dry cough and 38 fever with chillness three days before the 2 nd admission. The fever raised up to 39.8 at the day of this admission. He came to our emergency department again. He had no chest pain, abdominal pain, diarrhea. He denied arthritis nor rash. He never had pets. He denies traveling abroad. All his families were in good health.
PMH) Pneumonia in three years ago and admitted. In that time, his blood test showed thrombocytopenia (70,000/μL). Social Hx) no EtOH, no smoke There are none of the usually recognized risk factors for HIV infection. Medication) PPI started from the 1st admission ROS) *Since several years before, temperatures as high as 38 have occurred in once every two months, but resolved spontaneously. *He has some experience of hives after having insect bites.
Physical findings vital signs BP 100/60mmHg, HR 98/min, RR20/min, SpO2 96 (room air),bt 39.8 general sick, malaise eye icteria - /anemia - throat redish - /tonsils swelling - oral hygene is good neck no lymphadenopathy HS regular, no murmur, S1 S2 S3(-)S4(-) RS wheeze - /crackle - Abd soft &flat, BS is normal hepatomegaly -,splenomegaly + A left axillary lymphnode is palpable, 2cm in diameter, which is firm,elastic and tender.
Labo 1 WBC 3300/µL Seg 35.o% Lym 62.0% Mono 3.0% Hb 12.2g/dL Ht 37.0% MCV 83 Plt 115000/µL Na 137 meq/l K 3.7 meq/l Cl 103 meq/l Ca 8.1 mg/dl BUN 8mg/dL Cr 0.8mg/dL GOT 46IU/L GPT 28IU/L LDH 600IU/L T Bil 0.6mg/dL BS 92mg/dL CRP 1.39
Chest radiograph Chest radiograph shows bilateral multiple nodules.
Endoscopy (1 st Admission) a large ulcer on the upper body of the stomach
ECG * Tachycardia NSR * Mild right axis deviation
Problem list
Problem list fever dry cough appetite loss malaise weight loss hemorrhagic gastric ulcer (adolescent) left axilla lymphadenopathy splenomegaly pancytopenia GOT LDH bilateral multiple nodules of CXR
Discussion What s kind of diagnostic plan and therapeutic plan? Labolatory, imaging, etc
Occam s razors, Hickam s dictum Occam s razor shall apply to a young under 50years old
VINDICATE Vascular less likely Infection IE, Tb, HIV Neoplasm malignant lymphoma Degenerative less likely Intoxication less likely Congenital less likely Auto-immune granulomatous disease vasculitis Trauma less likely Endocrinopathy less likely Iatrogenic Idiopathic Inheritance less likely Psychogenic less likely
Plan We checked Blood culture Chest & abdominal CT Biopsy A left axillary lymph node BAL gastric tissue
Labo2 HTLV-1 - HIV1/2 - IL-2R 2550 U/mL Ferritin 151 ng/ml ANA <40 MPO-ANCA - PR3-ANCA - C3 94 mg/dl C4 30 mg/dl IgG 1099 mg/dl IgA 404 mg/dl IgM 96 mg/dl
Chest CT CT of the chest shows multiple nodular lesion and consolidation. These lesion are along bronchus, and spread to pulmonary parenchyma without destroying lung components. There are many small mediastinal lymph nodes. lymph nodes
Abdominal CT Gastric wall thickness in the lesser curvature Splenomegaly, mass in the spleen Lymphadenopathies around the SMA and abdominal aorta.
pathology Lymph node Gastric tissue BAL Many atypical cells can be seen
CD 56 CD CD 43
Pathological diagnosis highly aggressive NK/T cell lymphoma
Pathological diagnosis highly aggressive NK/T cell lymphoma EBV assosiated
There are three reasons why we came up to the diagnosis of EBV associated lymphoma. 1. It was the early onset at the age of 18. 2. There were repeated history of high fever. 3. The disease was spread to many organs.
Serodiagnosis of Ebstein-Barr virus VCA-IgG (<0.5) VCA-IgM (<0.5) EA-IgG (<0.5) EBNA-IgG (<0.5) Not infected - - - - Acute infected + ± + - Post infected + ± ± + Chronic infected ++ ± +++ ± This case ++(10.9) -(0.2) +(1.5) +(1.5)
Ebstein-Barr virus infection chronic EBV infection CAEBV *Ebstein-Barr virus associated lymphadenopathy *It is resulted from EBV infected T/NK LPD *Fetal in 5-15 years if treated symptomatically due to lymphoma, organ failure, HPS, bleeding, etc.
Final diagnosis experience of hives after having insect bites and thronbocytopenia were signs of CAEBV
After chemothrapy, he received cord blood stem cell transplantation.
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