The atypical presentation of Felty syndrome, even without arthritis symptoms, needs further evaluation. Timely diagnosis of neutropenia and splenomegaly in patients with rheumatoid arthritis without joint symptoms is crucial for a better prognosis. Despite the rarity of the condition, clinicians should have a high index of suspicion, and multidisciplinary collaboration between rheumatology, hematology, and other specialists is required for accurate diagnosis.

Felty syndrome (FS) is a disorder that develops after a long history of erosive rheumatoid arthritis and presents with splenomegaly and neutropenia. In addition to joint deformities, FS causes a variety of extra-articular manifestations such as vasculitis, skin lesions, and lymphadenopathy. FS is also reported to cause non-cirrhotic portal hypertension which may result in variceal bleeding. FS is usually treated by disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate. Herein, we report a case of rapidly deteriorating FS and a severe relapsing neutropenia only a few weeks after discontinuation of methotrexate and other available DMARDs. The patient presented with a fever and a multi-drug resistant gangrenous ulcer consistent with ecthyma gangrenosum. The patient was also found to have hepatosplenomegaly and portal hypertension. The case was managed with antibiotics and symptomatic treatments only as DMARDs were either unavailable or not affordable by the patient. However, the patient\'s condition did not improve. This case highlights that DMARDs are considered an essential part of preventing infections due to FS neutropenia. Patients with FS should continue DMARDs for life to avoid the relapse of their condition.

Felty\'s syndrome (FS) is a disorder wherein patients with rheumatoid arthritis develop splenomegaly, neutropenia, and in some cases, portal hypertension without underlying cirrhosis. Esophageal variceal bleeding is a complication of FS in patients with portal hypertension. In contrast to splenectomy, few reports exist on the management of variceal bleeding with endoscopic therapy. Moreover, the long-term outcome has not been reported. We present a patient with esophageal variceal bleeding due to portal hypertension secondary to Felty\'s syndrome. The patient was followed up for two years postendoscopy intervention. Literature review was performed and the histological features of portal hypertension in FS are discussed. The patient presented with a typical triad of rheumatoid arthritis (RA), splenomegaly, and neutropenia and was diagnosed as Felty\'s syndrome in 2012. She was admitted to our hospital in September 2017 for esophageal variceal bleeding. At the time of admission, her liver function test was normal. Abdominal CT showed no signs of cirrhosis and portal vein obstruction. Liver biopsy further excluded diagnosis of cirrhosis and supported the diagnosis of porto-sinusoidal vascular disease (PSVD), which was previously named as noncirrhotic idiopathic portal hypertension (NCIPH). An upper abdominal endoscopy revealed gastric and esophageal varices. A series of endoscopies was performed to ligate the esophageal varices. The patient was followed up for two years and did not show rebleeding. In conclusion, comorbid PSVD might be a cause of portal hypertension in FS patients. The present case had excellent outcome in two years, which supported the use of endoscopic therapy for the management of variceal bleeding in FS patients. Further large prospective study is needed to confirm the findings.

Large granular lymphocyte (LGL) leukemia is an indolent lymphoproliferative malignancy which dysregulates humoral immunity and underlies the myriad autoimmune phenomena. We describe a 62-year-old woman with Felty\'s syndrome who developed a severe bleeding diathesis. Laboratory evaluation demonstrated acquired inhibitors to both factor VIII (FVIII) and fibrinogen, likely secondary to T-cell LGL leukemia. After a complicated course, the patient\'s inhibitors were extinguished with rituximab and high-dose corticosteroids. Bleeding was controlled with alternating FEIBA (factor eight inhibitor bypassing activity) and recombinant activated FVII. This report reviews the literature comparing the efficacy of various treatment modalities for both disorders. To our knowledge, this is the first reported case of a patient with LGL leukemia acquiring an inhibitor to FVIII or fibrinogen.

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OBJECTIVE: To review and summarize the information available on the effectiveness and safety of biological therapies in refractory Felty\'s syndrome (FS).
METHODS: We describe a case of FS with severe neutropenia and recurrent bacterial infections unresponsive to disease-modifying antirheumatic drug treatment and long-term administration with granulocyte colony-stimulating factor, in which treatment with rituximab (RTX) was useful and resulted in a sustained neutrophil response. Current evidence on the use of biological therapies in FS is also analyzed through a systematic review of the English-language literature, based on a PubMed search.
RESULTS: Available data on the use of biological therapies in refractory FS are based only on several case reports and are limited to the use of RTX and some anti-tumor necrosis factor α agents (etanercept, infliximab, and adalimumab). Including the case described here, data are available on 8 patients treated with RTX. A sustained increase in the absolute neutrophil count (>1500/mm(3)) was observed in 62.5% (5/8) of these patients after 1 cycle of treatment. In most of them, the hematological response was accompanied by a parallel improvement in biological markers of inflammation and other clinical manifestations of FS (arthritis, recurrent infections, systemic symptoms, etc). After a median follow-up of 9 months (range, 6-14), only 1 of these patients relapsed and neutropenia reappeared; in this patient, retreatment was rapidly effective. No significant adverse events related to RTX therapy were reported. Experience with anti-tumor necrosis factor agents is limited to 6 patients, none of whom presented any sustained increase in neutrophil count.
CONCLUSIONS: Although it is not yet possible to make definite recommendations, the global analysis of all cases reported to date only supports the use of RTX as a second-line therapy in patients with refractory FS.

OBJECTIVE: Bleeding esophageal varices are a common complication of portal hypertension in patients with underlying liver disease. Often patients with hepatic cirrhosis have hypersplenism with thrombocytopenia and leukopenia. Felty\'s syndrome is a disorder where patients with rheumatoid arthritis develop splenomegaly, neutropenia, and on rare occasions, portal hypertension without underlying cirrhosis.
METHODS: We present a case of a patient with portal hypertension secondary to Felty\'s syndrome and discuss the importance of recognizing this condition since the treatment of choice is surgical management with splenectomy. A review of the literature and underlying liver histologic features are discussed.
RESULTS: Medical and surgical management of patients with Felty\'s syndrome is different from those with portal hypertension due to cirrhosis.
CONCLUSIONS: Splenectomy is the treatment of choice for complications of portal hypertension in patients with Felty\'s Syndrome.

Hyperviscosity syndrome (HVS) is characterised by high serum viscosity and the involvement of multiple organs, commonly causing retinal haemorrhage, bleeding diathesis, pulmonary hypertension, congestive heart failure (CHF), neurologic deficits and death. It has been reported that HVS is mostly encountered in Waldenström\'s macroglobulinaemia (80%-90% of all HVS cases) and occasionally in multiple myeloma. HVS in patients with connective tissue diseases (CTD) has rarely been reported. Of 28 cases of HVS reported in patients with CTD, 19 were with seropositive rheumatoid arthritis (RA). However, only six of these 19 cases had Felty\'s syndrome. Here we report another case of HVS in a patient with RA as well as Felty\'s syndrome.

Felty\'s syndrome is a rare but serious extra-articular manifestation of rheumatoid arthritis. Morbidity as well as mortality are increased on account of greater susceptibility to infectious agents. We report on two patients suffering from Felty\'s syndrome who were successfully treated by cyclophosphamide. A review of the literature with special regard to treatment of Felty\'s syndrome is given.

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