Immunoglobulin D Multiple Myeloma With Rapidly Progressing Renal Failure

Area of Science:

• Hematology
• Oncology
• Nephrology

Background:

• Immunoglobulin D (IgD) multiple myeloma (MM) is an extremely rare subtype of multiple myeloma, accounting for less than 2% of all cases.
• IgD MM is characterized by multiorgan involvement, with renal failure being a prominent and often defining feature.

Observation:

• A 62-year-old male smoker with hypertension presented with abdominal pain, constipation, and decreased urination.
• Laboratory results revealed significant renal impairment (S.Cr 5.99 mg/dL), anemia (hemoglobin 8.7 g/dL), and elevated serum free lambda light chains (8,947.6 mg/L) with a low κ/λ ratio (< 0.01).
• Serum IgD levels were markedly elevated at 27,300 mg/L, supporting the diagnosis of IgD MM.

Findings:

• Bone marrow biopsy confirmed plasma cell hypercellularity (70-80%) with 8% monoclonal IgD lambda plasma cells.
• Despite treatment with bortezomib and dexamethasone, followed by a bone marrow transplant, the patient remained dialysis-dependent, indicating persistent renal compromise.
• A solitary lytic lesion in the iliac crest was identified on skeletal survey.

Implications:

• This case underscores the aggressive nature of IgD multiple myeloma and its significant impact on renal function, often leading to long-term dialysis dependence.
• Despite advances in hematological control, the prognosis for IgD MM remains poor, particularly concerning renal outcomes.
• Early recognition and aggressive management strategies are crucial for patients with IgD MM to mitigate severe complications like irreversible kidney failure.