Background and Aims

We present 4 clinical cases presenting periodic fevers with autimmune diseases& lymphocytes clonal expansion, in which the Epstein barr virus is considered the principal triggering factor.

Methods

Case1:57year-old male patient, hospitalized in our unit for periodic fever and severe asthenia.

Past history:Severe handicapping rheumatoid arthritis(RA)with recent lupus.

Case2:78year-old female patient, hospitalized for severe asthenia, dysphonia+pharyngeal pain+periodic fever.

Case3:85year-old male patient, good general condition, presented with periodic fever for years and episodic severe pancytopenia.

Case4:70-year-old malepatient, admitted for recurrent episodes of asthenia+weight loss+ febrile leuko-neutropenia.

Past history:Hypertension, type 2 diabetes, RA treated with METHOTREXATE stopped for 2years because of neutropenia.

Results

Case1:We noted severe anemia+leucopenia+hyperferritinemia. PCR Epstein barr virus(EBV)positive.

Anti-nuclear antibodies(Ab), anti-DNA& anti CCP Ab were positive.

T-cell large granular lymphocytic leukemia(T-LGL)was confirmed.

Good outcome under Corticosteroides+METHOTREXATE.

Case2:Severe inflammatory anemia, hyperferritinemia(43256 Ug/l)+glycosylated ferritin<10%.

Monoclonal immunoglobulin IgG lambda. serum PCR-EBV:3.3log.

Bone marrow aspirate:granular hyperplasia+hemophagocytosis.

Bone marrow biopsy:reactional plasma cell hyperplasia.

PETscan:lymphadenopathies+hypermetabolic splenomegaly.

We conclude at adult STILL disease due to EBV complicated by haemophagocytic lymphohistiocytosis syndrome(HLH).

Favorable outcome under corticotherapy.

Case3:Infectious, autoimmune, autoinflammatory, hematological analysis were negative except positive PCR-EBV(3.8log).

Bone marrow biopsy:negative.

Spontaneous favorable outcome at each episode with no specific treatment.

Case4: Anti-CCP Ab highly positive. PCR-EBVpositive(6 log)+IL6. IgG4 elevated(2.14g/l).

PETscan:Intense diffuse osteo-medullary hypermetabolism.

Bone marrow biopsy:REACTIONAL T-LYMPHOCYTOSIS+HLH.

Abnormal CD8+&CD4+T-lymphocyte.T-cell receptor gene rearrangement concluding to T-LGL leukemia.

Good response to GANCICLOVIR then steroides+METHOTREXATE.

Conclusions

EBV is implicated in various hematological&autoimmune conditions.

We think that there is specific EBV genomic integration site also in acute T-cell LGL leukemia & not just in NK-LGL leukemia