Abstract
Abstract 4384
Epstein–Barr virus-associated T lymphoproliferative disease (EBV+ T-LPD, non-immunocompromised hosts) is rare on adults. Normally it is only found on children. This disease, according to recent pathological categorization, can be categorized into following subtypes: (i) category A1, polymorphic LPD without clonal proliferation of EBV-infected cells; (ii) category A2, polymorphic LPD with clonality; (iii) category A3, monomorphic LPD (T-cell or NK cell lymphoma/leukemia) with clonality; and (iv) category B, monomorphic LPD (T-cell lymphoma) with clonality and fulminant clinical course; Clinical symptoms of systemic EBV+ T-LPD include fever, liver, spleen and lymph nodes enlargement, jaundice of bile stasis, liver failure, pancytopenia. Based on degree of pathological changes, there are three groups of EBV+ T-LPD: I) polyclonal II) oligoclonal III) monoclonal (neoplasm). EBER, CD2 and are all positive. Usually drugs for treatment of EBV+ T-LPD include Glucocorticoid, CiclosporinA, and Etoposide.
An adult patient of systemic EBV+ T-LPD (non-immunocompromised hosts) (neoplasm) had been diagnosis and treatment recently by us. The patient is a 46-years-old male, fever, abdominal pain, hypocytosis and lymph nodes enlargement, 2 weeks of jaundice, vomiting and nausea. Physical examination: T: 37–39°C; marasmus, sclera and jaundice, upper left clavicle lymph node enlargement, apparent abdominal distention, upper abdominal pain, severe spleen enlargement. Blood Routine WBC 1.88×109/L; HB 87g/L; PLT 1.7×109/L. SGPT 163 U/L, SGOT 179μmol/L, STB 309μmol/L, CB 178μmol/L, STP43.8 g/L, ALB27.1 g/L, LDH905U /L. KPTT, PT, TT prolonged by a little. No abnormal cells were found in Bone Marrow examination. CT: 1. multiple enlarged lymph nodes were found in upper left clavicle area, inside mediastinum, and at right hilus of lung (diameter up to 1.4cm, with some nodes fusing together). 2. Pleural effusion in both thoraxes. 3. Severe spleen enlargement with several low-concentration nodes found inside. 4. Multiple enlarged lymph nodes found inside abdominal cavity and behind peritoneum. Pathological examination (upper left clavicle lymph node): lymph node normal structure disappears; many heteromorphic lymph-like cells were found, clear nucleus abnormality; Immunophenotype: CD3 +(mostly positive); CD5+(Partially positive); CD7+ (minor positive); CD4,CD8: a few cells positive;. EBER are positive. Pathological diagnosis: Epstein–Barr virus-associated T lymphoproliferative disease, EBV+ T-LPD, (III group, neoplasm stage)
adult patient was treated by many drugs including liver-protection, antibiotics, G –CSF and others. Because many symptoms such as abdominal pain, jaundice and high fever aggravated, plus severe liver and respiratory failure, the patient was infused DXM 10mg/d1-7. After treatment of DXM, many symptoms of the patient mitigated. Then the patient was treated by ECVP (Etoposide 0.1/D1 ~ D4, CTX 0.6/d1, VCR 2mg/D1, D8, Methylprednisolone 160mg/D1 ~ D7,) plus G-CSF (150 ~ 400mg/D). The minimum of WBC was 0.07 ×109/L. During treatment the patient suffered from pneumonia, therefore antibiotics were used. After treating, the temperature of patient returned and stayed normal; abdominal pain, jaundice, and vomiting symptoms alleviated, and eventually disappeared; spleen size was reduced. Among all enlarged superficial lymph nodes and enlarged lymph nodes inside the mediastinum, inside the abdominal cavity, and behind peritoneum, some reduced sizes and some eventually returned to normal sizes. Pleural effusion disappeared. Liver function: SGPT, SGOT and bilirubin index returned to normal. Coagulation function returned to normal. The patient has now finished treatment in hospital and is currently home, waiting for next cycle of treatment. This case of adult patient of systemic EBV+ T-LPD(non-immunocompromised hosts) (neoplasm) indicate that the treated method of ECVP plus G-csf is safe and effective in adult patient of systemic EBV+ T-LPD (neoplasm); no significant toxicity was found during treatment; the early diagnosis and early treatment of adult EBV+ T-LPD is very important. Further study and clinical research on more effective method of treatment on adult EBV+ T-LPD is still desired.
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
