Successful Treatment of Immunoglobulin D Myeloma by Bortezomib and Dexamethasone Therapy

Sekiguchi, Naohiro; Takezako, Naoki; Nagata, Akihisa; Wagatsuma, Miyuki; Noto, Satoshi; Yamada, Kazuo; Miwa, Akiyoshi

doi:10.2169/internalmedicine.50.5977

Cited by 4 publications

(6 citation statements)

References 20 publications

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“…Over the last decade, multiple randomized trials have shown the superiority of novel immunomodulatory agents (thalidomide) and proteasome inhibitors (bortezomib) in combination with ASCT have shown over standard therapy (melphalan, vincristine, adriamycin, and dexamethasone) [9]. …”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin D Multiple Myeloma With Rapidly Progressing Renal Failure

et al. 2015

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Immunoglobulin D (IgD) multiple myeloma (MM) is a very rare form of myeloma affecting less than 2% of all myeloma patients. It has a multiorgan involvement with renal failure being the key feature. We present here a case of IgD MM in a 62-year-old white male, smoker with past medical history of hypertension, who presented to emergency department with complaints of lower abdominal pain, constipation and decreased urination. Physical exam was unremarkable. Laboratory investigation showed S.Cr 5.99 mg/dL, hemoglobin 8.7 g/dL and corrected S.Ca 10.6 mg/dL. Urine dipstick showed 100 protein and TP/Cr ratio was 23. Serology was positive for serum free lambda chain level of 8,947.6 mg/L as well with free κ/λ ratio < 0.01. The results of serum and urine electrophoresis and immunofixation were also supportive of diagnosis of IgD MM. IgD level was remarkably elevated (27,300 mg/L) too. CT scan of abdomen/pelvis was negative for obstructive uropathy. Skeletal survey showed a solitary lytic lesion in the iliac crest. His kidney function deteriorated next day requiring hemodialysis. The bone marrow biopsy was positive for plasma cell hypercellularity (70-80%) and flow cytometry showed 8% monoclonal IgD lambda plasma cells. The patient was started on bortezomib and dexamethasone and he underwent bone marrow transplant 6 months later. He is doing well hematologically now but he remains dialysis-dependent. IgD MM is a very rare disease affecting younger population with poor prognosis; patients often end up on hemodialysis despite better control of the hematological component.

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Section: Discussionmentioning

confidence: 99%

Immunoglobulin D Multiple Myeloma With Rapidly Progressing Renal Failure

et al. 2015

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“…En relación con las anormalidades citogenéticas, éstas son variables (3,11,13) y algunos de los pacientes pueden tener un cariotipo normal (8,13), aunque no se ha descrito el efecto que tienen en el pronóstico de los pacientes con mieloma secretor de IgD.…”

Section: Discussionunclassified

“…Tradicionalmente, se ha descrito como de mal pronóstico cuando se compara con los tipos más comunes (secretor de IgG, IgA o de cadenas ligeras), con una media de sobrevida global entre 12 y 17 meses. Se postula que su pronóstico adverso se debe en gran parte a que es un reto diagnóstico y en la mayoría de los casos se diagnostica en etapas avanzadas de la enfermedad (7,8).…”

Section: Introductionunclassified

“…El mieloma secretor de IgD tiene características de laboratorio y en las manifestaciones clínicas que lo hacen único (4). El diagnóstico es particularmente difícil porque aproximadamente en 40% de los casos no se observa pico monoclonal en la electroforesis de proteínas en suero (7,8) y en los pacientes restantes el pico monoclonal es discreto; además a causa de la proteinuria monoclonal se puede confundir con un mieloma secretor de cadenas ligeras.…”

Section: Introductionunclassified

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Mieloma de células plasmáticas secretor de IgD, un reto diagnóstico. Reporte de un caso

et al. 2013

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RESUMEN El mieloma de células plasmáticas secretor de IgD es una neoplasia de células plasmáticas poco frecuente y agresivo, generalmente afecta individuos más jóvenes que los demás mielomas. Es de difícil diagnóstico, ya que no se observa el pico de proteína monoclonal característico de los mielomas; no obstante, la inmunofijación, la cuantificación de IgD y el estudio de orina son pruebas de gran utilidad para su diagnóstico. Con respecto a las manifestaciones clínicas, es frecuente anemia, falla renal y múltiples lesiones óseas; además, la enfermedad tiene un curso agresivo, con poca respuesta a la quimioterapia convencional. A continuación, se describe un caso de una mujer de 57 años con antecedentes de dolor óseo y múltiples lesiones líticas óseas, quien fue diagnosticada con mieloma secretor de IgD a partir de la electroforesis de proteínas en suero, la inmunofijación en suero y orina, la cuantificación de IgD y el estudio medular. ABSTRACT Immunoglobulin D plasma cell myeloma is a rare and aggressive plasma cell disorder, which usually occurs in younger patients than other myelomas. Immunoglobulin D multiple myeloma is usually misdiagnosed because of the lack of a typical monoclonal protein spike; however, immunofixation electrophoresis, Immunoglobulin D serum levels and urine analysis are highly useful for diagnosis of the disease. Regarding clinical manifestations, patients commonly have anemia, renal failure, and multiple bone lesions. In addition, patients show an aggressive clinical course with poor response to conventional treatment and unfavorable prognosis. We report a 57 years-old female who presented with bone pain and multiple osteolytic bone lesions; according to serum protein electrophoresis, serum and urine immunofixation, IgD serum levels, and bone marrow biopsy, a final diagnosis of immunoglobulin D plasma cell myeloma was made. KEY WORDS Multiple myeloma, immunoglobulin D, renal insufficiency

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“…He was treated with two cycles of VAD therapy [28 days cycles of vincristine (0.4 mg/kg) with doxorubisin (9 mg/m 2 ) by continuous intravenous infusion for 4 days, and dexamethasone (40 mg/kg) by intravenous infusion for 4 days] as an induction treatment, by which he achieved partial response. The patient had his first recurrence in 2009 and received BD therapy consisting of Bor and Dex [6] and achieved a very good partial response. In 2014, enlargement of the mediasinum without any symptoms was noted on a chest X-ray.…”

Section: Casementioning

confidence: 99%