Results (Outcome and Follow up):
After the treatment started, her neutrophil count improved. On her
follow-up appointments, her CBC and WBC remained normal, and she
reported improvement in her fatigue symptoms. She has not had any
hospital admissions or life threatening infections, and nonspecific
symptoms improved considerably.
Discussion :
Felty syndrome is an extracellular articular manifestation of rheumatoid
arthritis. The lifetime risk of developing Felty syndrome (FS) for a
patient with rheumatoid arthritis (RA) has been estimated to be
approximately 1-3 percent (1). It is primarily diagnosed through
clinical evaluation based on the presence of persistent neutropenia and
enlarged spleen in chronic RA. It is crucial to emphasize this criterion
because diagnosing the condition can be challenging when arthritis
symptoms are absent (4,8). Arthritis usually occurs for almost ten or
more years before neutropenia is diagnosed (6). Beyond this triad,
clinical presentations of Felty’s syndrome can involve anemia, low
platelet counts (thrombocytopenia), recurrent bacterial infections, skin
ulcers, unexplained portal hypertension, and an elevated risk for the
development of blood-related cancers like non-Hodgkin’s and Hodgkin’s
lymphoma (3). Though uncommon, documented cases in the literature
highlight instances of FS occurring without concurrent articular RA
(Table 2). We have presented such a case and briefly reviewed the
existing literature.
Table 2 shows a few cases, including ours, in which Felty syndrome was
diagnosed before or simultaneously with the onset of arthritis symptoms.
(Separate File)
Felty syndrome was found to be more common in females. All the patients
presented with neutropenia and splenomegaly, and some of them had
arthritis initially as well, as shown in the table.
Pathophysiology of neutropenia in Felty syndrome involves both cellular
and humoral immunity. It includes the development of autoantibodies
against granulocyte colony-stimulating factor (G-CSF) and
polymorphonuclear neutrophils (PMN), resulting in apoptosis of
neutrophils and neutropenia (13).
There are no specific criteria for diagnosing felty syndrome. It is a
clinical diagnosis of rheumatoid arthritis with splenomegaly and
neutropenia. In patients who present without arthritis, positive RF and
anti-CCP antibodies can point towards the possibility of rheumatoid
arthritis, like in our patient. High-titer RF and anti-CCP have a
specificity of 99.5% for RA (15). When the patient tested positive for
both factors, she was referred to rheumatology and diagnosed with RA.
Felty syndrome does not have a specific curative treatment. Literature
needs guidelines in the management of Felty syndrome, although a few
articles have highlighted the different strategies. Treatment primarily
focuses on improving neutropenia with the treatment of rheumatoid
arthritis, which is the underlying cause (8). Methotrexate and rituximab
are the preferred DMARDs in patients with FS (11). Numerous case reports
have demonstrated that rituximab can induce a sustained and complete
response in autoimmune cytopenias, particularly in cases of Felty’s
Syndrome (7, 10, 14). In our case, the patient responded well to the
Rituximab treatment. Glucocorticoids can temporarily increase
neutrophils as they cause the release of non-segmented neutrophils into
the circulation (12). The role of splenectomy is small, although it has
been used in refractory cases.
Our patient did not have arthritis; instead, she presented with
remarkable fatigue, severe neutropenia in labs, and mild splenomegaly on
imaging. Further workup showed high-titer rheumatoid factor and positive
anti-CCP antibody. The neutropenia normalized with the use of a high
dose of steroids but later relapsed. She was then started on rituximab
therapy, which improved her neutrophils. However, the patient needs to
be closely monitored for neutrophils and the development of arthritis.
While our case report and brief literature review provide valuable
insights into Felty syndrome’s atypical presentation and management, it
is essential to acknowledge that these findings may only sometimes
apply. This limits our report, as it may not apply to all the cases due
to individual differences in presentation and response to treatment.
Felty syndrome is a rare presentation of RA, but persistent neutropenia
can lead to life-threatening infections. Therefore, it is necessary to
check for RA and early refer to rheumatology even in the absence of
arthritis symptoms. Early detection of Felty syndrome is crucial for
further management and prevention of life-threatening infections.
Conclusions :
In conclusion, this case highlights the present atypical manifestation
of Felty Syndrome, underscoring the challenges in managing this rare
condition. The case findings emphasize the importance of considering
diverse clinical manifestations of FS. We also emphasize the importance
of ruling out other causes of neutropenia and splenomegaly. If left
untreated, neutropenia can lead to the development of life-threatening
infections. Awareness among healthcare professionals, particularly
hematologists, is crucial for timely recognition and appropriate
intervention, ensuring optimal care for patients with this complex
autoimmune condition.