Immunoglobulin D amyloidosis: a distinct entity

Gertz, Morie A.; Buadi, Francis K.; Hayman, Suzanne R.; Dingli, David; Dispenzieri, Angela; Greipp, Philip R.; Kumar, Shaji; Lacy, Martha Q.; Lust, John A.; Leung, Nelson; Rajkumar, S. Vincent; Russell, Stephen J.; Zeldenrust, Steven R.; Mıkhael, Joseph; Roy, Vivek; Kyle, Robert A.

doi:10.1182/blood-2011-06-358895

Cited by 17 publications

(10 citation statements)

References 25 publications

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“…Despite these studies, two case reports were identified in the literature of patients with IgD MM achieving complete remission at eight and 21 years follow-up [ 5 ], indicating that some patients do very well. A case series of 53 patients in 2012 showed that IgD MM is associated with a lower incidence of renal amyloidosis, higher serum albumin, and lower urine protein levels [ 2 ]. Where other IgM and IgA myelomas have a high concentration of serum proteins and M components, patients with IgD MM have low M component proteins, which can be undetectable in some cases.…”

Section: Discussionmentioning

confidence: 99%

“…Renal amyloidosis was found in 36% versus 58% of these two groups, respectively (P = 0.05). Survival outcomes were found not to be different among IgD amyloidosis as compared to IgG, IgA, or light chain myeloma amyloidosis [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…The condition is more common in males and compared with other MM isotypes, IgD MM is characterized by an aggressive clinical course with worse overall survival (OS) and a high frequency of complications [ 1 ]. The incidence of hypercalcemia and amyloid light chain (AL) amyloidosis is more common in IgD MM than in other myelomas [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

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Immunoglobulin D Multiple Myeloma: A Rare Variant

MacDougall¹,

Niazi²,

Rehan³

et al. 2022

Cureus

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Immunoglobulin D multiple myeloma (IgD MM) is a rare isotype of multiple myeloma (MM), comprising less than 2% of all cases. It is often associated with advanced disease at the time of diagnosis, an aggressive clinical course, and shorter overall survival (OS) than other subtypes of MM. There is an increased frequency of undetectable or small monoclonal (M-) protein levels on electrophoresis, hypercalcemia, anemia, lytic bony lesions, and renal failure. However, given the rarity of the disease, there are few cases of IgD MM described in the literature. Given the very small amount of IgD immunoglobulins, they may form very small or undetectable M spike on electrophoresis, making the diagnostic error in diagnosing this specific subgroup very easy. Treatment for MM has seen significant advancement, especially over the last decade, with the advent of medications such as proteasome inhibitors, immunomodulatory agents, and monoclonal antibodies. It is important to understand how IgD MM responds to these newer agents and why this disease continues to be associated with poor outcomes despite advancements in treatment. Small clinical studies on patients with IgD MM show better outcomes following a combination of high-dose chemotherapy (HDCT) and autologous stem cell transplant (ASCT) compared to standard chemotherapy. Given the rarity of the disease, there are no large studies done to see the effectiveness of these treatments, and most of the data are derived from small case series. We report a case of IgD kappa MM that was incidentally discovered following a traumatic bicycle accident. The patient started treatment with bortezomib and dexamethasone (Vd) as an inpatient while he was in the rehabilitation unit and was later switched to bortezomib, dexamethasone, and lenalidomide (VRd) as an outpatient. He has now completed seven cycles and successfully underwent autologous hematopoietic stem cell transplantation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Immunoglobulin D Multiple Myeloma: A Rare Variant

MacDougall¹,

Niazi²,

Rehan³

et al. 2022

Cureus

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“…IgD amyloidosis is also rare, and in two recent large studies, serum IgD monoclonal protein was identified in ,1% of patients with AL amyloidosis. 18,22 Gertz et al 18 and Roussel et al 22 suggested a lower frequency of renal and cardiac involvement in IgD amyloidosis, although the overall survival of patients was similar to AL amyloidosis.…”

Section: Igd and Monoclonal Gammopathymentioning

confidence: 99%

“…However, IgD can be detected by immunofixation or immunodiffusion using antisera to IgD. 18 IgD monoclonal gammopathy is extremely rare. 8,18 Although IgD heavy-chain deposition associated with an IgD monoclonal gammopathy has not been described, both IgD myeloma and IgD amyloidosis have been described in the literature.…”

Section: Igd and Monoclonal Gammopathymentioning

confidence: 99%

IgD Heavy-Chain Deposition Disease

Royal

Quint²,

Leblanc

et al. 2015

Journal of the American Society of Nephrology

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Monoclonal Ig deposition disease (MIDD) is a rare complication of monoclonal gammopathy characterized by deposition of monoclonal Ig light chains and/or heavy chains along the glomerular and tubular basement membranes. Here, we describe a unique case of IgD deposition disease. IgD deposition is difficult to diagnose, because routine immunofluorescence does not detect IgD. A 77-year-old man presented with proteinuria and renal failure, and kidney biopsy analysis showed a nodular sclerosing GN with extensive focal global glomerulosclerosis, tubular atrophy, and interstitial fibrosis. Immunofluorescence was negative for Ig deposits, although electron microscopy showed deposits in the glomeruli and along tubular basement membranes. Laser microdissection of glomeruli and mass spectrometry of extracted peptides showed a large spectra number for IgD, and immunohistochemistry showed intense glomerular and tubular staining for IgD. Together, these findings are consistent with IgD deposition disease. Bone marrow biopsy analysis showed 5% plasma cells, which stained for IgD. The patient was treated with bortezomib and dexamethasone, which resulted in improvement of hematologic parameters but no improvement of renal function. The diagnosis of IgD deposition disease underscores the value of laser microdissection and mass spectrometry in further evaluating renal biopsies when routine assessment fails to reach an accurate diagnosis. A 77-year-old man of Eastern European descent presented with proteinuria and severe renal insufficiency 6 months ago. The patient had numerous comorbidities, which included chronic obstructive pulmonary disease associated with pulmonary hypertension and bronchiectasis, coronary artery disease, mild aortic stenosis, arterial hypertension, and dyslipidemia.At presentation, the patient complained of fatigue, weight loss, and lower extremity swelling. His BP was normal, and other than the edema, the physical examination was unremarkable. His serum creatinine level was 2.2 mg/dl (194 mmol/L), with an eGFR of 29 ml/min. Additional evaluation showed nephrotic range proteinuria of 5.3 g/d. Microscopic examination of the urine revealed .100 red blood cells per high-power field. Other significant laboratory findings included a mild normocytic anemia with a hemoglobin concentration of 11.4 g/dl and a decreased serum albumin at 2.5 g/dl. Serum calcium level was normal. Serological studies for antinuclear antibody and antineutrophil cytoplasmic antibodies were negative, and complement levels (C3 and C4) were in the normal range.Serum protein electrophoresis showed no M spike on initial evaluation. However, the serum protein immunofixation studies revealed a monoclonal l-band. Serum l-free light-chain level was elevated at 2303 mg/L, with a k:l ratio of 0.02. A 24-hour urine collection revealed the presence of a monoclonal l-light chain. Bone studies did not show any lytic bone lesion.A bone marrow biopsy was performed, and it showed normal cellularity with a mild monoclonal plasmacytosis (5%) and a nega...

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A case of benign immunoglobulin D monoclonal gammopathy of undetermined significance with 26 years of follow‐up

Anderson,

Bladé,

Kyle

2024

eJHaem

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The presence of a serum immunoglobulin D (IgD) monoclonal protein (M‐protein) is seen in < 1% of patients with monoclonal gammopathies and is usually indicative of a malignant plasma cell disorder. Only a few cases of well‐documented benign monoclonal gammopathy of undetermined significance (MGUS) of IgD subtype have been reported, and only 2 of those had over 5 years of follow‐up at the time they were reported. Herein we describe longer‐term follow‐up of one of those 2 patients who has subsequently passed away from unrelated causes but never developed multiple myeloma or amyloidosis after 26 years of follow‐up. Although IgD MGUS is extremely rare, this case confirms that presence of an IgD M‐Protein is not always synonymous with a malignant plasma cell process.

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Immunoglobulin D amyloidosis: a distinct entity

Cited by 17 publications

References 25 publications

Immunoglobulin D Multiple Myeloma: A Rare Variant

Immunoglobulin D Multiple Myeloma: A Rare Variant

IgD Heavy-Chain Deposition Disease

A case of benign immunoglobulin D monoclonal gammopathy of undetermined significance with 26 years of follow‐up

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