Contributions
Abstract: PB1759
Type: Publication Only
Background
Aims
A case, who was diagnosed with EBV-LPD and developed PRCA during follow-up, is presented.
Methods
A 75-year-old woman with pain in upper and lower extremities applied to our center in February 2016. Her past medical history was unremarkable except for rheumatoid arthritis. On physical examination bilateral cervical, submandibular, axillary lymphadenopathies (LAP) and splenomegaly were detected. Laboratory tests revealed normochromic normocytic anemia, elevated serum lactate dehydrogenase and acute phase reactants. Positron emission tomography (PET) showed supra- and infradiaphragmatic malignant lymph nodes and splenic involvement. An excisional biopsy of cervical LAP was performed. Pathological examination showed CD20 (+) and CD30 (+) large B cells in the interfollicular area. EBV early RNA signals were ckecked by in-situ hybridization and viral transcripts were detected. Diagnosis of EBV-LPD was made. During diagnostic work-up deepening of anemia with reticulocytopenia, increased transfusion requirement and inadequate response to transfusion necessitated a bone marrow aspiration and biopsy. Pathological examination of the bone marrow was compatible with PRCA. Parvovirus IgM and DNA was negative; IgG was found to be positive. Because of the lack of response to steroids, Rituximab was given (375 mg/m2, weekly). Anemia and patient’s clinical condition improved after 8 weeks of treatment.
Results
In the pathogenesis of LPD polyclonal lymphoid response to an antigenic trigger is thought to be followed by development of monoclonal neoplastic diseases. In our case, this trigger was thought to be EBV as it is known as one of the main causative agents for LPD in the literature. Clinical complaints and physical examination findings are common among all patients and frequently not leading to a definitive diagnosis in most of them as it is the case in our patient. Compared to the strong association of secondary PRCA with parvovirus B19 its association with EBV is rare. PRCA can develop before the diagnosis, during the course and after the remission of LPD. In our case we observed PRCA in the follow-up period of EBV-LPD.
Conclusion
On the basis of EBV-LPD being more common in transplant setting our case was thought to be unique due to the absence of transplantation or immunosuppression history. This case report points out to the possibility of oexistence of two rare diseases, EBV-LPD and PRCA.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): EBV, Pure red cell aplasia, Lymphoproliferative disorder
Abstract: PB1759
Type: Publication Only
Background
Aims
A case, who was diagnosed with EBV-LPD and developed PRCA during follow-up, is presented.
Methods
A 75-year-old woman with pain in upper and lower extremities applied to our center in February 2016. Her past medical history was unremarkable except for rheumatoid arthritis. On physical examination bilateral cervical, submandibular, axillary lymphadenopathies (LAP) and splenomegaly were detected. Laboratory tests revealed normochromic normocytic anemia, elevated serum lactate dehydrogenase and acute phase reactants. Positron emission tomography (PET) showed supra- and infradiaphragmatic malignant lymph nodes and splenic involvement. An excisional biopsy of cervical LAP was performed. Pathological examination showed CD20 (+) and CD30 (+) large B cells in the interfollicular area. EBV early RNA signals were ckecked by in-situ hybridization and viral transcripts were detected. Diagnosis of EBV-LPD was made. During diagnostic work-up deepening of anemia with reticulocytopenia, increased transfusion requirement and inadequate response to transfusion necessitated a bone marrow aspiration and biopsy. Pathological examination of the bone marrow was compatible with PRCA. Parvovirus IgM and DNA was negative; IgG was found to be positive. Because of the lack of response to steroids, Rituximab was given (375 mg/m2, weekly). Anemia and patient’s clinical condition improved after 8 weeks of treatment.
Results
In the pathogenesis of LPD polyclonal lymphoid response to an antigenic trigger is thought to be followed by development of monoclonal neoplastic diseases. In our case, this trigger was thought to be EBV as it is known as one of the main causative agents for LPD in the literature. Clinical complaints and physical examination findings are common among all patients and frequently not leading to a definitive diagnosis in most of them as it is the case in our patient. Compared to the strong association of secondary PRCA with parvovirus B19 its association with EBV is rare. PRCA can develop before the diagnosis, during the course and after the remission of LPD. In our case we observed PRCA in the follow-up period of EBV-LPD.
Conclusion
On the basis of EBV-LPD being more common in transplant setting our case was thought to be unique due to the absence of transplantation or immunosuppression history. This case report points out to the possibility of oexistence of two rare diseases, EBV-LPD and PRCA.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): EBV, Pure red cell aplasia, Lymphoproliferative disorder