OPEN IN READ APP
JOURNAL ARTICLE

Autologous Stem Cell Transplantation in Patients With Primary Systemic Amyloidosis: Experience of a Tertiary Hospital

M A Domínguez-Muñoz, C Calderón-Cabrera, M L Martino-Galiana, J Martín-Sánchez
Transplantation Proceedings 2015, 47 (9): 2661-4
26680066

BACKGROUND: Systemic immunoglobulin light-chain (AL) amyloidosis is a plasma cell dyscrasia that results from the deposition of insoluble fragments of immunoglobulin light or heavy chains. The subsequent disruption of organ function resulting from the extracellular deposition of these fragments ultimately leads to death. The median overall survival (OS) of patients ranges from 12 and 18 months down to 5 months in patients with cardiac involvement. Autologous hematopoietic stem cell transplantation (ASCT) is a treatment modality that achieves good response. The affected solid organ transplant (SOT) could improve performance status and have a favorable impact on survival.

METHODS: Retrospective analysis of 11 AL amyloidosis patients who received ASCT from 2005 to 2013, 2 of them also underwent SOT.

RESULTS: The 5-year OS depending on the number of organs involved (1 vs ≥2) was 100% versus 60% (P = .13). With a median follow-up of 4.8 years (range, 1.6-8), 81% of patients are alive maintaining complete hematologic response (n = 6) and very good partial response (n = 3). The 5-year progression-free survival was 80% (range, 42%-94%). Two patients underwent cardiac and renal transplantation as a bridge to ASTC. None of the double transplant patients has died.

CONCLUSION: ASCT is an effective treatment option in patients with AL amyloidosis. In those with advanced single organ damage, SOT should be considered to improve the clinical outcome.

Discussion

You are not logged in. Sign Up or Log In to join the discussion.

Trending Papers

Available on the App Store

Available on the Play Store
Remove bar
Read by QxMD icon Read
26680066
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"