Two young adult cases of Epstein-Barr virus associated-hemophagocytic lymphohistiocytosis with monoclonal proliferation of virus-infected cells within a short period after primary infection

Kensaku Idutsu, Yasunobu Abe, Takamitsu Matsushima, Eriko Sada, Rie Ohtsuka, Junichi Kiyasu, Motoaki Shiratsuchi, Kazuhiro Kotoh, Junji Nishimura, Shoichi Ohga, Ryoichi Takayanagi

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe complication of Epstein-Barr virus (EBV) infection. Interactions between EBV-infected T cells and activated macrophages cause several conditions such as pancytopenia, liver dysfunction and coagulopathy. We describe here two young adults with EBV-associated HLH with monoclonal proliferation of EBV-infected T cells within a short period after infectious mononucleosis as a primary infection. One patient was a 16-year-old man who developed severe pancytopenia and liver dysfunction two months after infectious mononucleosis. Bone marrow examination showed hemophagocytosis, and laboratory data demonstrated monoclonal proliferation of EBV-infected T cells. Several treatments such as immunosuppressive therapy, chemotherapy and hematopoietic stem cell transplantation were not effective, and the patient died of progressive disease. The other patient was a 19-year-old woman who developed thrombocytopenia and liver dysfunction two months after infectious mononucleosis. Findings of hemophagocytosis and monoclonal proliferation of EBV-infected T cells were similar to those in the first case. Clinical signs and symptoms were resolved completely by immunosuppressive therapy containing methyl-prednisolone and cyclosporine. Since these two cases each demonstrated a distinct clinical course, an investigation of the prognostic factors and treatment strategies for EBV-HLH is warranted.

Original languageEnglish
Pages (from-to)1541-1547
Number of pages7
Journal[Rinshō ketsueki] The Japanese journal of clinical hematology
Volume49
Issue number11
Publication statusPublished - Jan 1 2008

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Hemophagocytic Lymphohistiocytosis
Human Herpesvirus 4
Young Adult
Viruses
Infectious Mononucleosis
Infection
T-Lymphocytes
Liver Diseases
Pancytopenia
Immunosuppressive Agents
Bone Marrow Examination
Epstein-Barr Virus Infections
Hematopoietic Stem Cell Transplantation
Therapeutics
Prednisolone
Thrombocytopenia
Cyclosporine
Signs and Symptoms
Macrophages
Drug Therapy

All Science Journal Classification (ASJC) codes

  • Medicine(all)

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Two young adult cases of Epstein-Barr virus associated-hemophagocytic lymphohistiocytosis with monoclonal proliferation of virus-infected cells within a short period after primary infection. / Idutsu, Kensaku; Abe, Yasunobu; Matsushima, Takamitsu; Sada, Eriko; Ohtsuka, Rie; Kiyasu, Junichi; Shiratsuchi, Motoaki; Kotoh, Kazuhiro; Nishimura, Junji; Ohga, Shoichi; Takayanagi, Ryoichi.

In: [Rinshō ketsueki] The Japanese journal of clinical hematology, Vol. 49, No. 11, 01.01.2008, p. 1541-1547.

Research output: Contribution to journalArticle

Idutsu, Kensaku ; Abe, Yasunobu ; Matsushima, Takamitsu ; Sada, Eriko ; Ohtsuka, Rie ; Kiyasu, Junichi ; Shiratsuchi, Motoaki ; Kotoh, Kazuhiro ; Nishimura, Junji ; Ohga, Shoichi ; Takayanagi, Ryoichi. / Two young adult cases of Epstein-Barr virus associated-hemophagocytic lymphohistiocytosis with monoclonal proliferation of virus-infected cells within a short period after primary infection. In: [Rinshō ketsueki] The Japanese journal of clinical hematology. 2008 ; Vol. 49, No. 11. pp. 1541-1547.
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AU - Sada, Eriko

AU - Ohtsuka, Rie

AU - Kiyasu, Junichi

AU - Shiratsuchi, Motoaki

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