APFID

Home > Case of the Week

Title : 50 year-old female w/ Relapsed fever, myalgia and arthralgia
Date : January 16, 2011
Contributed by

Eun Jeong Joo, MD
Samsung Medical Center, Seoul, Korea

Patient History
Age/Sex 50 year-old female
Chief complaint Relapsed fever, myalgia and arthralgia which occurred 6 months ago
Present illness

The patient presented with relapsed fever, myalgia and arthralgia which occurred 6 months ago. She was hospitalized with fever, arthralgia in a local hospital at that time, but didn’t get any diagnosis. At three months prior visit to Samsung Medical Center, she readmitted to another hospital for relapsed fever and arthralgia. Chest CT revealed that interstitinal pnemonitis and extensive mediastinal and supraclavicular lymphadenopathy. With impression of sarcoidosis and lymphoma, she was referred to pulmonary department of SMC for further evaluation.

Past medical history

She was a carrier of HBV

Physical examination

Cervical lymph nodes were not palpable and skin lesions were not present.

Initial laboratory findings

CBC 2370-11.3-183K (segmented neutrophil 54.4%)
ESR/CRP 33/0.99
LDH 466

Radiologic findings
Hospital course

Mediastinoscopic biopsy was performed
Histopathologic finding showed EBV associated reactive hyperplasia of the lymph node


 
Question - ID Case of the Week ( January 16, 2011 )
What do you think the most likely diagnosis is?
 
Correct Answer

Chronic active EBV infection

 
Review

Chronic active EBV infection defines chronic or recurrent infectious mononueleosis-like symptoms that persist for a long time and by an unusual pattern of anti-EBV antibodies. Chronic active EBV infection was first described in 1978 and its diagnostic guidelines were proposed in 2005 by Okano and Kimura. Three criteria should be fulfilled to diagnose CAEBV infection.

1. Persistent or recurrent infectious monoculosis like sytmes should exist for over 3 months.

2. Unusual pattern of anti-EBV antibodies with high antivurs-capsid-antigen (anti-VCA) and anti-early antigen (anti-EA) or detect of high EBV genome levels in affected tissues or peripheral blood.

3. Chronic illness that cannot be explained by other known disease processes at diagnosis. 

 

The major signs and symptoms of patients with CAEBV were as follows: fever, hepatosplenomegaly, liver dysfunction, thrombocytopenia, anemia, hypersensitivity to mosquito bites, skin rash, hydroa vacciniforme, diarrhea and uveitis. Life-threatening complications included haemophagocytic syndrome 24%, malignant lymphoma 18%, and disseminated intravascular coagulopathy 16%. In this case, she presented with fever, skin rash, bictyopenia and interstitial pneumonia which are related to symptom and signs of CAEBV

 

Cytotoxic T cells and natural killer cells demonstrated the main role in the regulation of viral infections. The defect of these cells has been related to the development of CAEBV and HLH (haemophagocytic lymphohistiocytosis). The natural host cell for EBV is B cell. Under the control of CTL, the number of infected B cell is limited in small population. In patients with defective immunity of CTL or NK cells, the infected B, T and NK cells proliferated. The several factors are suspected to influence the pathogenesis, but it still remained undiscovered.

 

Chronic active EBV infection is the important differential diagnosis for patients who have chronic constituational symptoms and signs. In certain population with CAEBV, the disease progress to lymphoproliferative disorder and show monoclonality. We should remind that CAEBV could present with the diverse spectrum from EBV associated LPD to malignancy and HLH. 
 

References

Okano M. et. al. Proposed guidelines for diagnosing CAEBV, Am J Haemtol. 2005

Kimura H. et al. Pathogenesis of CAEBV, Rev Med Virol. 2006

Kimura H. et al. Prognostic factors for CAEBV, Journal Infect Dis 2003

Kimura H. et al. Pathogenesis of CAEBV: Is this an infectious disease, lymphoproliferative disorder, or immunodeficiency? Rev. Med. Virol 2006

HLH-2004: Diagnosis and Therapeutic guidelines for HLH, Pediatr Blood Cancer 2007

 


޴ٷΰ ΰ