నైరూప్య

A rare cause of hypersplenism; splenic amyloidosis induced by familial mediterranean fever

Pervin Ozkan Kurtgoz, Ibrahim Guney, Suleyman Karakose & Edip Erkus

Background: Familial Mediterranean Fever (FMF) is an inflammatory disease characterized by fever and serositis attacks. The most important complication of the disease is the development of AA type amyloidosis. Amyloid most often accumulates in the kidney, liver, spleen and heart. Splenomegaly may be seen secondary to inflammatory response without developing amyloidosis in FMF patients. Although splenic amyloidosis has an asymptomatic nature but in the literature several cases of spontaneous splenic rupture due to splenic amyloidosis have been reported. There are no reported cases of hypersplenism due to AA type amyloidosis in the literature so that our case has importance in this respect. Case summary: A 30-year-old female patient with diagnosis of FMF and proteinuria for 3 years was admitted to the nephrology outpatient clinic because of weakness, palpitation and swelling of the legs. She had anemia and thrombocytopenia. She had a recent history of hospitalization due to frequent erythrocyte transfusion requirements. Bone marrow related diseases and immunologic causes were excluded during the evaluation of bicytopenia. Patients history revealed that she had amyloid deposition in kidney. The patient's need for frequent blood transfusion was attributed to hypersplenism. Splenectomy was performed and amyloid deposition was detected in the spleen. After splenectomy, hemoglobin and platelet counts were increased. Conclusion: We present a case with hypersplenism secondary to AA type amyloidosis and who were treated with splenectomy. If cytopenia and splenomegaly were detected in FMF patients then hypersplenism must be considered and splenectomy must be kept in mind for the treatment.