Clinical Features and Treatment Response of Light Chain (AL) Amyloidosis Diagnosed in Patients With Previous Diagnosis of Multiple Myeloma

Madan, Sumit; Dispenzieri, Angela; Lacy, Martha Q.; Buadi, Francis K.; Hayman, Suzanne R.; Zeldenrust, Steven R.; Rajkumar, S. Vincent; Gertz, Morie A.; Kumar, Shaji

doi:10.4065/mcp.2009.0547

Cited by 75 publications

(70 citation statements)

References 20 publications

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“…Presence of BM-AD in MM is reported in 22–40% of patients8,9 and is more common than “true” symptomatic AL-MM. The former has no impact on survival and no connection with organ damage8,9 in contrast with AL-MM where survival is clearly impaired compared with MM without symptomatic AL 10. A recent study indicated that BM-AD in MM were present in only 1% of patients 11.…”

Section: Introductionmentioning

confidence: 99%

The risk of renal disease is increased in lambda myeloma with bone marrow amyloid deposits

Kozlowski

Montgomery

Befekadu

et al. 2017

JBM

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Background Light chain amyloidosis (AL) is a rare deposition disease and is present in 10–15% of patients with myeloma (MM). In contrast to symptomatic AL in MM, presence of bone marrow (BM) amyloid deposits (AD) in MM is not connected to kidney damage. Renal AD but not BM-AD occur mostly in MM with lambda paraprotein (lambda MM). Methods We investigated amyloid presence in BM clots taken at diagnosis in 84 patients with symptomatic MM and compared disease characteristics in MM with kappa paraprotein (kappa MM)/lambda MM with and without BM-AD. Results Lambda MM with BM-AD was compared with kappa MM without BM-AD, kappa MM with BM-AD, and lambda MM without BM-AD: lambda MM with BM-AD patients had a significantly higher mean creatinine level (4.23 mg/dL vs 1.69, 1.14, and 1.28 mg/dL, respectively) and a higher proportion presented with severe kidney failure (6/11 [55%] vs 6/32 [19%], 1/22 [5%], and 3/19 [16%], respectively). Proteinuria was more common in lambda MM with BM-AD patients compared with kappa MM without BM-AD patients (8/11 [73%] vs 5/32 [16%], respectively). Conclusion Kidney damage was more common in lambda MM with BM-AD indicating presence of renal AD.

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Section: Introductionmentioning

confidence: 99%

The risk of renal disease is increased in lambda myeloma with bone marrow amyloid deposits

Kozlowski

Montgomery

Befekadu

et al. 2017

JBM

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“…AL amyloidosis can occur in 10%–15% of patients with myeloma and is the most common cause of amyloid-associated GI tract involvement 3. It results from deposition of the protein in the submucosa and muscularis propria.…”

Section: Discussionmentioning

confidence: 99%

Multisystem amyloidosis as the unifying diagnosis for constipation, collapse and cardiomyopathy

McFarlane¹,

Bashford

Sah

et al. 2018

BMJ Case Reports

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Amyloidosis a rare disorder characterised by the deposition of amyloid protein aggregates in different organ systems throughout the body with resulting functional impairment of affected organs. It can present with localised or multisystemic deposits. Diagnosis is often delayed due to the non-specific nature of the symptoms. We present the case of a 59-year-old man with a 12-month history of non-specific symptoms. Investigations revealed positive gastritis. Blood tests showed only a normocytic anaemia and thrombocytopaenia. CT scan showed proximal sigmoid thickening. Biopsies were unremarkable. Echocardiogram and cardiac MRI scan showed restrictive cardiomyopathy. Congo red staining of gastric biopsies showed amyloid deposition. The patient had elevated serum kappa light chains and a bone marrow biopsy confirmed multiple myeloma and he was subsequently diagnosed with systemic light chain (AL) amyloidosis secondary to this. He was started on chemotherapy and parenteral nutrition; however, he deteriorated rapidly and so was started on palliative treatment and discharged home.

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“…Approximately 10 % of patients with multiple myeloma have concomitant amyloidosis and it often involves the heart, kidney, and nerves [28,29]. The prognosis for multiple myeloma with concomitant amyloidosis is poor and heart involvement is often associated with a rapidly progressive course and high mortality rate [28,30,31].…”

Section: Amyloidosismentioning

confidence: 99%

CT and MRI evaluation of cardiac complications in patients with hematologic diseases: a pictorial review

Kim

Jung

Kim

et al. 2015

Int J Cardiovasc Imaging

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Cardiac complications with hematologic diseases are not uncommon but it is difficult to diagnose, due to non-specific clinical symptoms. Prompt recognition of these potentially fatal complications by cardiac computed tomography (CT) or cardiac magnetic resonance imaging (MRI) may help to direct clinicians to specific treatments according to causes. Thrombosis is often related to central venous catheter use and is usually located at the catheter tip near the atrial wall. Differentiation of thrombosis from normal structure is possible with CT and, distinction of a thrombus from a tumor is possible on a delayed enhancement MRI with a long inversion time (500-600 ms). Granulocytic sarcoma of the heart is indicated by an infiltrative nature with involvement of whole layers of myocardium on CT and MRI. MRI with T2* mapping is useful in evaluating myocardial iron content in patients with hemochromatosis. Diffuse subendocardial enhancement is typically observed on delayed MRIs in patients with cardiac amyloidosis. T1 mapping is an emerging tool to diagnose amyloidosis. Myocardial abscess can occur due to an immunocompromised status. CT and MRI show loculated lesions with fluid density and concomitant rim-like contrast enhancement. Awareness of CT and MRI findings of cardiac complications of hematologic diseases can be helpful to physicians for clinical decision making and treatment.

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Clinical Features and Treatment Response of Light Chain (AL) Amyloidosis Diagnosed in Patients With Previous Diagnosis of Multiple Myeloma

Cited by 75 publications

References 20 publications

The risk of renal disease is increased in lambda myeloma with bone marrow amyloid deposits

The risk of renal disease is increased in lambda myeloma with bone marrow amyloid deposits

Multisystem amyloidosis as the unifying diagnosis for constipation, collapse and cardiomyopathy

CT and MRI evaluation of cardiac complications in patients with hematologic diseases: a pictorial review

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