melphalan and Spondylitis--Ankylosing

Ankylosing spondylitis is an autoimmune disease with chronic inflammation of the spine and sacroiliac joints that is commonly treated with immunosuppressants including disease-modifying antirheumatic drugs and anti-tumor necrosis factor alpha therapy.. A 75-year-old female with active ankylosing spondylitis on treatment with etanercept was referred to us for newly diagnosed IgG kappa free light chain multiple myeloma. After failing induction with revlimid, bortezomib, and dexamethasone, she was initiated on carfilzomib. Following the achievement of adequate response to induction, she underwent an autologous hematopoietic stem cell transplant selected for CD34+ cells with melphalan 200mg/m. Although our patient had a coincident and unprecedented resolution of ankylosing spondylitis after receiving the hematopoietic stem cell transplant, this case consolidates the idea of transplant as a potential treatment option for ankylosing spondylitis and other rheumatological conditions.

Topics: Aged; Bortezomib; Dexamethasone; Female; Hematopoietic Stem Cell Transplantation; Humans; Lenalidomide; Melphalan; Multiple Myeloma; Remission Induction; Spondylitis, Ankylosing; Transplantation Conditioning; Transplantation, Autologous; Treatment Outcome

A 63-year-old man, known to have Bechterev's disease was admitted because of weight gain and nocturnal sweating. He also had signs of heart failure with progressive exertional dyspnoea. Many months previously numerous bleedings had occurred in the skin, predominantly the face (periorbital and perioral), the inguinal region and the penis.. Extensive diagnostic test failed to find any evidence of tumor. The Rumpel-Leede tourniquet test was positive, while platelet functions was normal, suggesting vascular disease. The skin biopsy showed many perivascular amyloid deposits (AL). Immunochemical differentiation also demonstrated the same amyloid in rectal and hepatic biopsies. But there was AA amyloid in a hepatic artery. Immunophoresis indicated a biclonal gammopahty of unknown significance.. These findings indicated the diagnosis of primary AL amyloidosis associated with a gammopathy of unknown significance and a secondary AA amyloidosis in the presence of chronic Bechterev s disease. The clinical picture also showed cardiac complications, predominantly heart failure and numerous previous myocardial infarctions without S-T elevations. Echocardiography, which revealed marked thickening of the left ventricle with a restrictive filling pattern, suggested cardiac co-morbidity. The patient underwent chemotherapy with melphalan and prednisone but had a sudden cardiac death.. In a case of bleeding of unknown cause systemic amyloidosis should be considered in the differential diagnosis. A tendency towards bleeding, as in this patient, may be the first sign of amyloidosis, which ist often diagnosed quite late in the course of the disease.

Topics: Amyloidosis; Death, Sudden, Cardiac; Diagnosis, Differential; Fatal Outcome; Glucocorticoids; Heart Failure; Hemorrhagic Disorders; Humans; Male; Melphalan; Middle Aged; Myocardial Infarction; Paraproteinemias; Prednisone; Spondylitis, Ankylosing

We present a 26-year-old man with edema, ascites and bloody diarrhea that later proved to be due to gastrointestinal and renal amyloidosis. Interestingly, he was also diagnosed as having ankylosing spondylitis,-possibly after a delay of 12 years. The obscure diagnosis and challenging treatment of secondary amyloidosis are further discussed.

Topics: Adult; Amyloidosis; Biopsy, Needle; Colchicine; Colonoscopy; Disease Progression; Drug Therapy, Combination; Fatal Outcome; Gastrointestinal Diseases; Humans; Immunohistochemistry; Male; Melphalan; Risk Assessment; Spondylitis, Ankylosing