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Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: One-year follow-up in five patient


, : Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: One-year follow-up in five patient. Blood 88(7): 2801-2806

The morbidity and lethality of AL amyloidosis is caused by the deposition of Ig light chains as fibrillar amyloid protein in vital organs, disrupting their function, and not by the generally low burden of clonal plasma cells that produce the paraproteins. Survival of patients with AL amyloidosis is no more than 1 to 2 years from the time of diagnosis with current management approaches. Clearly, more effective therapies are needed for this rapidly lethal disease. Five patients were treated with dose-intensive melphalan and blood stem cell support and followed for a period of 1 year. Patients were diagnosed with AL amyloidosis by tissue biopsy and categorized by performance status and organ involvement. Their plasma cell dyscrasias were evaluated with immunofixation electrophoresis of serum and urine specimens, quantitative serum Igs, and immunohistochemical staining of bone marrow biopsy specimens. After treatment with dose-intensive intravenous melphalan followed by infusion of autologous growth-factor-mobilized blood stem cells, clinical evaluations and plasma cell studies were repeated at 3 and 12 months. Three men and 2 women aged 38 to 53 years were treated. Median performance status (SWOG) was 2 (1 to 3), and clinical presentations included nephrotic syndrome (n = 1), symptomatic cardiomyopathy (n = 1), gastrointestinal involvement with polyneuropathy (n = 2), and hepatomegaly (n = 1). With a median follow-up of 13 months (12 to 17 months), all five patients are well and have shown stable or improved performance status and clinical remission of organ-related dysfunction, including a 50% reduction in daily proteinuria with no change in creatinine, reversal of symptoms of cardiomyopathy and reductions of posterior wall and septal thickening, reversal of polyneuropathy and gastric atony, and resolution of hepatomegaly by computed tomographic scan. In 3 of the 5 patients (60%) at 12 months after treatment, plasma cell dyscrasias could not be detected. Dose-intensive chemotherapy with intravenous melphalan and growth-factor-mobilized blood stem cell support is feasible therapy for patients with AL amyloidosis, even when there is clinical evidence of cardiac involvement. At least some patients with AL amyloidosis achieve complete remission of their plasma cell dyscrasia, improvement in performance status, and clinical remission of organ-specific disease after this form of treatment.

US$29.90

PMID: 8839879


Other references

Tallon, 1986: Layerwise structure in the fcc {111} solid-liquid interface. Physical Review Letters 57(11): 1328-1331

Radermacher, P.; Grote, H.; Susanto, F.; Reinauer, H., 1982: A method for the gas chromatographic determination of long and medium chain free fatty acids in serum. Free fatty acids in serum were determined by gas chromatography after esterification with boron tri-fluoride-methanol. Following prepurification of the serum lipid fractions by thin layer chromatography, the described analytical procedure selectiv...

Schull, T.L.; Brandow, S.L.; Dressick, W.J., 2001: Synthesis of symmetrical triarylphosphines from aryl fluorides and red phosphorus: Scope and limitations. The reaction of aryl fluorides with phosphide anion, generated in situ from the reduction of red phosphorus by lithium metal in liquid ammonia, gave symmetrical triarylphosphines in fair to good yields. Phosphonodiamide, sulfonamide, 2-oxazolyl, a...

Baranowska, B.; Zgliczyński, S., 1979: Prostatectomy--induced decrease in an enhanced serum testosterone concentration in patients with prostatic hyperplasia and its pathophysiological implications. Endokrynologia Polska 30(1): 75-87

Link, W.; Schill, B.; Barbera, A.C.; Cubero, J.I.; Filippetti, A.; Stringi, L.; Kittlitz, E. von; Melchinger, A.E., 1996: Comparison of intra- and inter-pool crosses in faba beans (Vicia faba L.). I. Hybrid performance and heterosis in Mediterranean and German environments. Two recently detected, promising CMS systems in faba bean have opened up new possibilities for hybrid breeding. This study was conducted to determine hybrid performance and heterosis of 33 intra-pool and 66 inter-pool crosses from a factorial of s...

Mear C.E.; Becher J.W., 1986: Geology of Ellenburger and Fusselman reservoirs, Midland Farms and Inez fields, Andrews County, Texas. Pages 97-106 1986

Zemskaya, A.A., 1971: Mites of the family Dermanyssidae Kolenati, 1859, in the fauna of the USSR. Eight species of Dermanyssid mites have been recorded on birds in the USSR, and include five that are well known: Dermanyssus gallinae (Redi), D. hirundinis (Herm.), D. passerinus Berl. & Trt., D. grochovskae Zemskaya and D. quintus Vitzthum. The...

Mothersill, I.W.; Hilfiker, P.; Kramer, G., 1999: Self-induced seizures, a conscious act?. Epilepsia 40(SUPPL 2): 24

Catalano, D.; D.B.Asio, A., 1959: Radiological aspects of hydranencephaly. Description of 2 cases. La Clinica Pediatrica 41: 912-924

Alexander, C.P., 1938: New or little-known Tipulidae from eastern Asia (Diptera). XXXVI-XL. Philipp. J Sci Manila : 66: 93-134