Fealty syndrome
Overview
A rare complication of long-term rheumatoid arthritis which involves an enlarged spleen and blood abnormalities.
Symptoms
* Malaise * Fatigability * Anorexia * Weight loss * Joint pain * Joint deformity * Recurrent infection * Recurrent mouth infection * Sinusitis * Bronchitis * Furunculosis * Leg ulcers * Dragging sensation in left upper abdominal quadrant * Pain in left upper abdominal quadrant * Rheumatoid arthritis * Enlarged spleen * Enlarged liver * Swollen lymph nodes * Pallor * Brown pigmentation on extremities * Reduced number of neutrophils in blood * Reduced white blood cell count * Low blood platelet level * Reduced granular white blood cells in blood * Anemia * Blood vessel inflammation * Fever
Causes
* Mononucleosis * Congestive heart failure * Portal hypertension –Most often secondary to cirrhosis * Hepatitis * Hereditary spherocytosis * Sickle cell disease * Thalassemia major * Polycythemia vera * Malaria * Tuberculosis * Other infections: Mycobacterium avium complex, HIV, CMV, RMSF * Endocarditis o Malignancy (e.g., leukemia, lymphoma, metastases) o –Massive enlargement of the spleen usually signifies a lymphoproliferative or myeloproliferative disorder * Systemic lupus erythematosus * Felty's syndrome (rheumatoid arthritus, splenomegaly, and granulocytopenia) * Splenic hemangioma, hamartoma, or cyst * Trauma * Splenic vein thrombosis * Less common causes (“zebras”) include Gaucher's disease, amyloidosis, kala-azar (visceral leishmaniasis), schistosomiasis, rickets, syphilis, babesiosis, typhoid fever, histoplasmosis, and toxoplasmosis
Treatment
* Infectious etiologies require appropriate antibiotic regimens * Leukemia and lymphoma are treated with combination chemotherapy * Systemic lupus erythematosus and rheumatoid arthritis are treated with steroids and/or cytotoxic agents * Hemolytic anemia is treated with steroids * Splenectomy may be required for patients with traumatic spleen injury with persistent bleeding; patients without a spleen are at increased risk of sepsis and should receive regular pneumococcal and Haemophilus influenzae vaccinations