Case Presentation: A 70 year old female with a history of diabetes presented to the hospital for evaluation of pancytopenia and neutropenic fever. She was found to have pancytopenia on routine blood work. about 2 months prior to presentation. She complained of intermittent fever and chills for 1 week. She denied any specific complaints including cough, headache, dysuria, myalgias, nausea, vomiting or diarrhea. Physical exam revealed a temperature of 100.8 F, normal blood pressure and pulse. She had conjunctival pallor but remainder of the exam was normal. Laboratory evaluation demonstrated a WBC of 1 K/mm3 with absolute neutrophil count of 0.4, hemoglobin 9 g/dl, platelet count 90 K, reticulocyte count 2.8 %, ESR 100 mm/hr and CRP 5. Vitamin B12, folic acid, Iron and ferritin levels were normal. Infectious work up including urinalysis, blood culture were negative and a chest X-ray without infiltrate. Hospital course was significant for persistent intermittent low grade fevers. Repeat urine and blood (including fungal) cultures were negative. An extensive work up was done including hepatitis panel, parvovirus, CMV/EBV , HIV testing, SPEP, ANA, Rheumatoid factor (RF), anti-cyclic citrullinated peptide (CCP), abdominal imaging and bone marrow biopsy. ANA was positive (1:640), RF 3519 IU/ml, anti-CCP 266 u/ml. Abdominal imaging revealed normal liver, splenomegaly without any other signs of portal hypertension. Bone marrow biopsy showed normocellular marrow with maturing trilineage hematopoiesis. Rest of the workup was negative. Patient was diagnosed with non-articular felty’s syndrome and was initiated on rituximab with gradual improvement in ANC counts.
Discussion: Differential diagnoses for pancytopenia with splenomegaly include portal hypertension/cirrhosis, infections like EBV, autoimmune disorders (SLE/Rheumatoid arthritis), lymphoma, myeloproliferative neoplasms, storage diseases like Gaucher’s etc.., Felty’s syndrome is a triad of Rheumatoid arthritis (RA), splenomegaly and neutropenia. ANC below 2000/microL is required for the diagnosis and other causes of neutropenia should be excluded. Patient’s with Felty’s usually have severe erosive, seropositive RA for more than 10 years. Non Articular Felty’s syndrome is extremely rare. It is diagnosed in patients with positive RF/CCP in the absence of erosive RA disease after ruling out other etiologies for pancytopenia/splenomegaly. RA may manifest later. Literature review shows about 10 cases of non-articular felty’s syndrome reported so far. While some patients remained free of joint disease, most of them developed erosive joint disease up to 10 years after diagnosis. Management of Non-Articular Felty’s syndrome is similar to Felty’s and involves use of disease modifying antirheumatic drugs, methotrexate, rituximab , corticosteroids etc., Hydroxychloroquine and sulfasalazine are not studied in patients with felty’s syndrome. Patients with marked granulocytopenia and life-threatening infections in whom the granulocytopenia needs to be reversed quickly are treated with Granulocyte-colony stimulating factor.
Conclusions: Felty’s syndrome should be considered in patients presenting with pancytopenia, splenomegaly, elevated RF ,CCP after excluding other causes. It can manifest in patients without joint disease. Felty’s Syndrome can be an initial presentation of rheumatoid arthritis with manifestation of joint disease in future.