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The emerging role of Rituximab in the treatment of large granular lymphocytic leukemia associated with rheumatoid arthritis: a single center experience

Year: 2024

Type: article

Abstract: A 51-year-old woman had a three-year history of polyarthritis of small joints and was diagnosed with RA in 2017.She was RF positive and ACPA negative and had moderate disease activity with a disease activity assessment-28 C-reactive protein (DAS 28-CRP) of 4.41.MTX was started but was soon stopped due to hepatic toxicity with concomitant treatment with isoniazid for latent tuberculosis (LT).Six months after, she developed leucopenia with neutropenia (490/mm 3). Toxic etiology was ruled out after stopping rifampicin, which she was taking at that time to treat LT.She had no fever and no history of recent infections.Full blood count showed a hemoglobin concentration of 13.2 g/dL and a platelet count of 150x10 9 /L.Total protein electrophoresis was normal.Viral serologies for human immunodeficiency virus (HIV), hepatitis virus B and C (HBV and HCV), Epstein-bar virus (EBV) and cytomegalovirus (CMV) were negative.The abdominal ultrasound did not reveal hepatomegaly or splenomegaly.LGL count in peripheral blood smear was 0.59x10 9 /L and neutrophils went down to as low as 90/mm 3 , requiring treatment with GCS-F.Lymphocyte surface markers on flow cytometry of peripheral blood smear reflected a constitutively activated T-cell phenotype (55% of lymphocytes were positive for CD3+/CD8+ and the majority of these had LGL features: CD57+, CD5+ diminished, CD45 R0+, CD28-, CD16-).Flow cytometry analysis of the TCR variable ABSTRACT Large Granular Lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder with a peculiar association with Rheumatoid Arthritis (RA).The most common feature is neutropenia and patients can have splenomegaly, resembling Felty' s Syndrome.These diseases have similar clinical and laboratory abnormalities, but the diagnosis of T-cell LGL (T-LGL) leukemia requires evidence of clonality.Even though T-LGL leukemia is indolent in most cases, inadequate treatment when it is indicated can lead to significant morbidity and mortality, mainly associated with recurrent infections.We present two clinical cases that emphasize the emerging role of Rituximab as an effective therapeutic option in patients with T-LGL and RA.

Source: ARP Rheumatology

DOI: 10.63032/dnpz5424