A longitudinal evaluation of health-related quality of life in patients with AL amyloidosis: associations with health outcomes over time.

Light chain (AL) amyloidosis is a rare disease associated with significant, irreversible organ dysfunction and high case fatality. An observational study was conducted to assess health-related quality of life (HRQoL) in patients treated for AL amyloidosis between 1994 and 2014 with both high dose melphalan and stem cell transplantation (HDM/SCT) or non-SCT chemotherapy regimens. The SF-36v1® Health Survey (SF-36) was administered to assess HRQoL during clinic visits. Analysis of variance was used to compare pre- and post-treatment HRQoL within each treatment group to an age- and gender-adjusted general population (GP) normative sample. Cox proportional hazard models were fit to examine associations between pre-treatment levels of HRQoL and mortality within 1 and 5 years after initiating specific treatment regimens (HDM/SCT: n = 402; non-SCT chemotherapy regimens: n = 172). Among patients who received HDM/SCT, there were significant improvements following treatment in vitality, social functioning, role-emotional and mental health. Worse pre-treatment SF-36 physical component scores were associated with a greater risk of mortality in both treatment groups and follow-up periods (P ≤ 0·005 for both). [Correction added on 20 October 2017, after first online publication: This P value has been corrected]. Using HRQoL assessments in every physician visit or treatment may provide valuable insights for treating rare conditions like AL amyloidosis.

Optimized patient-trajectory for patients undergoing treatment with high-dose chemotherapy and autologous stem cell transplantation.

PURPOSE: Before, during and after autologous hematopoietic stem cell transplantation (HD-ASCT) patients suffer from significant loss of physical function, and experience multiple complications during and after hospitalization. Studies regarding safety and feasibility of physical exercise interventions for patients undergoing treatment with HD-ASCT are missing. METHODS: Forty patients referred to HD-ASCT treatment, suffering from multiple myeloma, lymphoma or amyloidosis aged 23-70 years were enrolled in a prospective longitudinal study. The study consisted of a home-based exercise program for use in the ambulatory setting and supervised exercise sessions Monday to Friday for 30-40 minutes during admission. Safety of the exercise program and physical tests were assessed by using a weekly questionnaire and report of inadvertent incidences. Adherence to the home-based exercise program was reported by using a patient diary, weekly questionnaire and count of daily attendance in supervised sessions during hospital stay. Data collection was scheduled shortly after diagnosis, admission, discharge and eight weeks after discharge. Success criteria were: no severe adverse events in relation to exercise program and assessments; performance of three days of physical exercises during ambulatory period and hospital stay and 150 minutes of weekly physical activity. RESULTS: Of the 25 patients who completed the exercise program during the ambulatory period prior to HD-ASCT a mean weekly attendance to home exercises of 5.3 (± 2.8) days and a median weekly physical activity of 240 (± 153.8) minutes was found. During hospital stay the median attendance was 9 (± 3.9) days of 10 (± 6.9) possible. Two months after discharge the patients reported a median weekly physical activity of 360 (2745.5) minutes. No severe adverse events in relation to the exercise program or assessments were reported. CONCLUSION: Based on the enrolled number of patients the physical exercise intervention for patients undergoing HD-ASCT seems promising regarding feasibility and safety.

Amiloidosis asociada a hemodiálisis como causa de subluxación atlo-axoidea anterior / Amyloidosis associated to hemolialysis as casuse of anterior atlo-axoid subluxation

La amiloidosis por depósito de beta 2-microglobulina constituye una complicación frecuente en los pacientes con insuficiencia renal crónica tratados con hemodiálisis durante largos períodos de tiempo. Se caracteriza por una alta incidencia de afectación poliarticular, desarrollando una espondiloartropatía destructiva que afecta predominantemente a la columna cervical, y que en casos graves puede provocar una subluxación atlo-axoidea. Se presenta el caso clínico de un paciente varón de 54 años de edad afectado de amiloidosis asociada a hemodiálisis, que presentó dolor cervical y rigidez resistente a tratamiento conservador. Tras realizar una radiografía lateral de columna cervical en máxima flexión y TAC cervical, se diagnosticó una subluxación atlo-axoidea anterior. El paciente fue intervenido quirúrgicamente, estando actualmente asintomático (AU)