Primary AL amyloidosis
is a condition that falls under the umbrella of plasma cell dyscrasias.
There are currently no treatments approved for the treatment of AL amyloidosis
With a median survival time of less than 18 months following diagnosis and no approved therapies to address the organ damage caused by AL amyloidosis
, there is a significant need for new treatments for this devastating disease, said John Orloff, M.D., Executive Vice President and Head of Research & Development at Alexion.
The global incidence of amyloidosis is estimated at up to nine cases per million per year, with AL amyloidosis
the most prevalent type in developed countries.
Another important step after diagnosing AL amyloidosis
consists in identifying involvement of other organs.
Bird et al., "Guidelines on the diagnosis and investigation of AL amyloidosis
," British Journal of Haematology, vol.
(6-8) The most common association is systemic AL amyloidosis
, (9,10) but cases of diffuse alveolar-septal amyloidosis that are caused by systemic AA, systemic wild-type ATTR, and systemic hereditary ATTR amyloidosis have also been reported (Table 2).
Progressive thickening of skin during that time prompted for a biopsy which revealed AL amyloidosis
. Pulmonary function tests had shown restrictive lung disease and reduced gas transfer.
Although AL amyloidosis
and monoclonal immunoglobulin deposition disease (light-chain deposition disease and heavy-chain deposition disease, resp.) are both characterized by monoclonal immunoglobulin deposits in tissues, these diseases are distinguishable strictly by Congo red staining and characteristic appearance on electron microscopy.
(formerly called primary) is the commonest and most aggressive form.
Gillmore, "Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis
," Blood, vol.
Odiick's commitment to bettering other peoples' lives was recognized by fans, who gathered outside Vancouver General Hospital to show their respect when Odjick made it known through the Canucks website that he had been diagnosed with AL amyloidosis
, a rare terminal disease.
In AL amyloidosis
, the presence of a monoclonal immunoglobulin can usually be demonstrated in serum or urine samples.
Cardiac involvement is common in AL amyloidosis
and is associated with various treatment options and prognoses.5-7 In AL amyloidosis
the heart is affected in nearly 50% of cases.6 Previous reports indicated cardiac involvement as the cause of death in approximately 50% of patients with AL amyloidosis
Primary AL amyloidosis
has clonal B-cell dyscrasia, including multiple myelomas, B-cell lymphoma, Waldenstrom macroglobulinemia, or another plasma cell neoplasia.[sup.3,4] Bone marrow biopsy is required for all patients with AL amyloidosis
to exclude overt multiple myeloma.