Conference paper

DIFFICULT TO TREAT – A PATIENT WITH RHEUMATOID ARTHRITIS AND T-CELL LARGE GRANULAR LYMPHOCYTE LEUKEMIA (T-LGLL)

Krešimir Rukavina orcid.org/0000-0003-1856-7382 ; Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University Hospital Centre Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia
Goran Šukara ; Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University Hospital Centre Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia
Branimir Anić ; Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University Hospital Centre Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia

Abstract

Introduction: According to ultrasound studies, up to 52% of rheumatoid arthritis (RA) patients will have splenomegaly due to various reasons. Those, with leukopenia, will be diagnosed with Felty’s syndrome (FS). However, a percentage of these patients will in fact have large granular lymphocyte leukemia, which in RA is almost always T-cell type (T-LGLL).
Report: A male patient born in 1963, came to our Clinic in 2016 due to a 5-year history of symmetric polyarthritis. At the time he had increased inflammatory markers, leukopenia, positive rheumatoid factor, cyclic citrullinated peptide antibodies and positive ANA and anti DNA antibodies. He also had hepatosplenomegaly. There was a dilemma whether it was RA with FS, RA associated with systemic lupus (RUPUS) or even a paraneoplastic disorder due to microcytic anemia and weight loss. After a thorough workup, including CT scans, sternal puncture and bone marrow biopsy (showing a polyclonal T-lymphocyte hyperplasia), a diagnosis of FS was made. He was treated with methotrexate (MTX) and prednisone. However neutropenia, lymphocytosis and hepatosplenomegaly persisted. A second broad CT scan revealed a multifocal lymphadenopathy with nodes up to 2 centimeters in size. A repeated bone marrow biopsy was characteristic of a lymphoproliferative disorder. A T-cell clonality test was performed from peripheral blood and the patient was diagnosed with T-LGLL. Although the initial recommended treatment for T-LGLL associated with autoimmune disease is MTX with prednisone, the patient did not respond to this treatment. Tofacitinib was also tried, with lacking effect, and at the moment a trial with rituximab is planned.
Conclusion: About one third of T-LGLL patients have RA. However only 0.6% of RA patients have LGLL. A strong connection between these entities and HLA-DR4 has been observed. LGLL is in most cases a chronic, indolent disease, without a standardised treatment protocol. Symptomatic patients and patients with associated autoimmune diseases require therapy, most oft en MTX with prednisone as the inital treatment. This case is an example that all neutropenic RA patients with splenomegaly should be carefuly evaluated, especially if the triad of FS occurs early in the disease course, and also that thanks to clonality tests, a percentage of FS will most likely be reclassifi ed to LGLL.
References:
Annals of Rheumatic Disease, UpToDate

Keywords

Hrčak ID:

213081

URI

https://hrcak.srce.hr/213081

Publication date:

5.12.2018.

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