J Korean Med Sci.  2009 Oct;24(5):970-974. 10.3346/jkms.2009.24.5.970.

Kaposi Sarcoma Herpes Virus-associated Hemophagocytic Syndrome Complicated by Multicentric Castleman Disease and Kaposi Sarcoma in a HIV-negative Immunocompetent Patient: An Autopsy Case

• Kim B
• Jeon YK
• Kim CW

• KMID: 1782026
• DOI: http://doi.org/10.3346/jkms.2009.24.5.970

Abstract

Kaposi sarcoma herpes virus (KSHV), also known as human herpesvirus-8, plays an important role in the pathogenesis of Kaposi sarcoma (KS), multicentric Castleman disease (MCD) of the plasma cell type, and primary effusion lymphoma. KSHV is rarely associated with the hemophagocytic syndrome (HPS), but when it does occur, it most occurs in immunocompromised patients. We report herein an unusual case of KSHV-associated HPS in an immunocompetent patient. A previously healthy 62-yr-old male was referred for evaluation of leukocytopenia and multiple lymphadenopathies. After a lymph node biopsy, he was diagnosed with MCD of the plasma cell type. KSHV DNA was detected in the lymph node tissue by polymerase chain reaction. Following a short-term response of the leukocytopenia to prednisolone, mental change, left side weakness, fever, thrombocytopenia, hemolytic anemia, and renal failure developed. Despite intravenous immunoglobulin therapy and plasmapheresis, he expired. The lymph nodes were infiltrated by hemophagocytic histiocytes in the sinuses. Pulmonary nodules and gastric erosions were shown to be KS. KSHV DNA was detected in the stomach, lung, and liver. This is the first case of multiple KSHV associated diseases including MCD and KS with KSHV-associated hemophagocytic syndrome in an HIV-negative, non-transplant, immunocompetent patient.

Keyword

Herpesvirus 8, Human; Lymphohistiocytosis, Hemophagocytic; Giant Lymph Node Hyperplasia; Sarcoma, Kaposi

MeSH Terms

Autopsy
Giant Lymph Node Hyperplasia/complications/*diagnosis/pathology
HIV Seronegativity
Herpesviridae Infections/*diagnosis
Herpesvirus 8, Human/*isolation & purification
Humans
Immunocompetence
Lymphohistiocytosis, Hemophagocytic/*diagnosis/etiology
Magnetic Resonance Imaging
Male
Middle Aged
Polymerase Chain Reaction
Sarcoma, Kaposi/complications/*diagnosis

Figure

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  Fig. 1 Histologic features of initial lymph node biopsy specimen. (A) At low magnification, lymphoid follicular hyperplasia was observed with preserved nodal architecture (H&E, ×12.5). (B) Plasma cells were markedly increased in the interfollicular area (H&E, ×200).

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  Fig. 2 Pathologic features of the lymph nodes and stomach at autopsy. (A) In lymph node sinuses, many macrophages engulfing red blood cells (hemophagocytic histiocytes) were observed (H&E, ×1,000). (B) A few LANA-1-positive lymphocytes were identified (LANA-1 immunostain, ×400). (C) Within the gastric mucosa, a few erosions were found in the body. (D) Microscopically, the lesion was composed of spindle-shaped endothelial cells with nuclear atypism and mitoses. Many extravasated RBCs were also observed (H&E, ×400).

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  Fig. 3 PCR analysis for KSHV. KSHV DNA was detected in the liver (lane 5), spleen (lane 6), stomach (lane 7), lung (lane 8), and lymph nodes (lanes 11 and 12). (Lanes 1 and 2; positive control, lanes 3 and 4; negative control, lane 6; bone marrow, and lane 9; the contralateral lung).

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