Plasma cells increased markedly in lymph node in hemophagocytic syndrome: a case report
© Congyang et al; licensee BioMed Central Ltd. 2009
Received: 6 November 2009
Accepted: 27 November 2009
Published: 27 November 2009
Hemophagocytic syndrome is a rare clinicopathological condition characterized by the activation of the mononuclear phagocyte system, resulting in hemophagocytosis in the reticuloendothelial systems. The pathogenesis of HPS remains unclear.
We report the case of a 20-year-old soldier suffering from HPS. Because of long history fever and no reasons being found, his left groin lymph node and left neck lymph node biopsy were done with two weeks interval. We found a marked increase in plasma cells in left neck lymph node during the course of the disease.
Our result provides a new thought for the researchers to understand the mechanisms responsible for the phagocytosis in HPS.
Hemophagocytic syndrome (HPS) is a rare entity characterized by the dysfunction of cytotoxic T cells (CTL) and natural killer (NK) cells, and the activation of the mononuclear phagocyte system [1, 2]. Its clinical symptoms include long-term high fever, cytopenias, hepatosplenomegaly, lymphadenopathy, and coagulopathy. HPS is divided into primary and secondary HPS, both of which are trigged by acute infections . The pathophysiology of HPS is very complex, but most researchers now believe that it involves the dysregulation of CTLs and activation of the mononuclear phagocyte system (MPS). In the HPS patient phagocytosis of blood cells is a very complex process and may be related to the production of specific immunoglobulins [1, 3–5]. In the current case, we found that plasma cells increased markedly in left neck lymph node during the progression of HPS. This observation supports the idea that antibody responses against blood cells may play an important role in the progression of HPS.
Laboratory findings on admission
Blood cell counts
0.80 × 109/L
3.42 × 1012/L
156 × 109/L
HPS is a life-threatening condition characterized by the activation of the MPS. It can be divided into genetic HPS and secondary HPS . Genetic HPS can result from autosomal defects, or can also be associated with immune deficiencies, such as Chediak-Higashi syndrome, X-linked lymphoproliferative syndrome, and Griscelli syndrome. Secondary HPS is associated with exogenous agents (including viruses, bacteria, fungi, parasites, and toxins), endogenous products (including those resulting from tissue damage, metabolic products, free radical stress), rheumatic diseases, and malignant diseases [2, 7, 8]. In the current case, we were unable to detect the triggering agent, despite culturing for bacteria, parasites, and fungi, and testing for antibodies to HAV, HBV, HCV, HIV, treponema pallidum, CP, MP, RSV, and Adv, and the patient's fever failed to resolve despite treatment with broad-spectrum antibiotics and antiviral drugs. We found no association between drug use and HPS. A diagnosis of lymphoma was eliminated because of the tissue structure and immunoarchitecture.
Although progress has been made in understanding the pathophysiology of HPS, the exact mechanisms responsible for the phagocytosis are unknown in HPS. Researchers found out that there have the RBC antibodies in the EBV-HPS and anti-RBC antibodies were involved in the phagocytosis of RBCs by macrophages . Also several other researchers have demonstrated that virus infection can induce antibody responses against RBCs, platelets, lymphocytes, and endothelial cells [5, 9]. As we known the antibodies are produced by the plasma cells. However there has no reports about the exactly changes of plasma cells in HPS patients. In our case the number of plasma cells was markedly increased from 8/HPF in the groin lymph node to 17/HPF in the cervical lymph node during the disease progression. Although we didn't detect changes of special antibodies against blood cells, this observation maybe provide a new idea for researchers to study the pathophysiology of HPS.
This case report showed plasma cells increased markedly in the left neck node during the progression of HPS. The result maybe provide one way to study the pathophysiology of the HPS.
Written informed consent was obtained from the patient' older brother for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this Journal.
cytotoxic T cells
mononuclear phagocyte system
hepatitis A virus
hepatitis B virus
hepatitis C virus
human immunodeficiency virus
respiratory syncytial virus
white blood cell count
red blood cell
international normalized ratio
activated partial thromboplastin time
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