A case of Epstein-Barr virus associated post-transplant lymphoproliferative disorder with CNS involvement: pathological findings at both biopsy and autopsy.
Post-transplant lymphoproliferative disorder (PTLD) with CNS involvement is an uncommon and fatal side effect of immunosuppressants. A 55-year-old man presented with non-fluent aphasia, fever, neck stiffness and disturbance of consciousness. Twenty-one years previously, the patient had undergone kidney transplantation for chronic renal failure. Brain MRI revealed multiple lesions in the bilateral cerebrum, right cerebellum and medulla oblongata. The brain biopsy showed EBV-positive lymphocytes infiltrating into the subarachnoid and Virchow-Robins space. The diagnosis of PTLD was made and the patient received a reduction in immunosuppressants. However, the patient died of massive bleeding from a rectal ulcer 3 months after the onset. An autopsy conducted 1 month after the biopsy revealed a diffuse large B-cell lymphoma at the biopsy site and extracranial PTLD lesions. Moreover, a human cytomegalovirus infection involving the rectum, pancreas, trachea and bladder was confirmed. Comparisons with past cases clarified the characteristics of this case, in particular, the clinicopathological involvement of leptomeninges. In addition, there have so far been only a limited number of such reports demonstrating detailed pathological findings, including both biopsy and autopsy findings. We herein describe the relationship between clinical and pathological findings and demonstrate the way CNS PTLD lesion progresses.
Departments of Neurology and Pathology, Hamamatsu University School of Medicine, Hamamatsu, Japan. ms1015@hamamed.ac.jp
Makiko
M
Suzuki
Isao
I
Kosugi
Tatsuhiro
T
Terada
Kentarou
K
Shirakawa
Hitoshi
H
Suzuki
Satoshi
S
Kono
Hiroaki
H
Miyajima
D001921:Brain / Q000473:pathology* / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000209:etiology / Q000473:pathology*; D004854:Herpesvirus 4, Human; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation / Q000009:adverse effects / Q000473:pathology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000209:etiology / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged
D007166:Immunosuppressive Agents