The presented rare form of double myeloma disease with initial renal insufficiency underscores the importance of careful observation and teamwork that can alter the course of this serious disease. [ncbi.nlm.nih.gov]

The cases presented here indicate that disease progression and relapse may be associated with selective outgrowth of a specific FLC producing clone. [learningcenter.ehaweb.org]

The present authors report the case of a 64-yr-old female with LCDD characterised by asymptomatic airway involvement. [erj.ersjournals.com]

Bowman's capsule may contain a material that is identical to that present in the center of the nodules. [jasn.asnjournals.org]

Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report. J Med Case Rep. 2007. 1:187. [Medline]. Gallo G, Goñi F, Boctor F, Vidal R, Kumar A, Stevens FJ. Light chain cardiomyopathy. [emedicine.medscape.com]

• Sepsis

  Three weeks later, she developed worsening respiratory insufficiency, ARDS, sepsis, respiratory arrest, and cardiac arrest resulting in death. [neurology.org]

  The use of high dose chemotherapy followed by ASCT is associated with toxicities such as mucositis, sepsis, bacteremia, and diarrhea. In general, LCDD patients are younger; thus, ASCT should be considered in these patients. [emedicine.medscape.com]

• Camping

  I am back to working out, running, playing ball with my 11 year old, hiking, camping, and all the things I loved to do before I got sick. Now i enjoy them even more. At one point my kidney functions were near 12% and are now at about 50%. [cancercompass.com]

  Dodrecht, Boston, London, Kluwer Academic Publishers, 2003; pp. 95–120 ↵ Grogan TM, Van Camp B, Kyle RA, Müller-Hemerlink HK, Harris NL. Plasma cell neoplasm. In : Jaffe ES, Lee Harris N, Stein H, eds. Tumours of Haematopoietic and Lymphoid Tissues. [erj.ersjournals.com]

• Recurrent Infection

  […] disorders (dyscrasias) such as multiple myeloma, primary amyloidosis, and related diseases or to monitor the effectiveness of treatment When you have bone pain, fractures, anemia (with normal size and shape red blood cells (normocytic)), kidney disease, and recurrent [labtestsonline.org]

• Hyperactivity

  In this capacity, it would demonstrate hyperactivity of the B cells that make the autoantibodies. Ig-fLCs were also found to be elevated in 1/3 patients with rheumatoid arthritis and 1/5 patients with systemic sclerosis. [mastattack.org]

Extensive workup showed a faint gamma region band (SPEP); free kappa light chains (urine IFE); kappa level 4165 with kappa/lambda ratio 737.17 (serum free light chains); generalized myopathic MUAPs with fibrillations (EMG), and severe bilateral CTS (EMG [neurology.org]

On the other hand, the FLC ratio is abnormal in all patients with LCDD, 1, 6, 7 suggesting that serum FLC measurement should be performed in the diagnostic workup of adults presenting with renal disease, independent of the presence of a serum or urine [bloodjournal.org]

Testing for the level of ALP (an enzyme called “alkaline phosphatase”) should be included in your regular blood lab workup. An ALP test measures the amount of this enzyme in the blood. [amyloidosis.org]

Laboratory Studies The laboratory workup for light-chain deposition disease (LCDD) includes the following: Routine serum chemistries to evaluate hepatic and renal function Complete blood cell (CBC) count to assess for anemia Serum and urine electrophoresis [emedicine.medscape.com]

• Hyperuricemia

  […] creatinine (48 m/Lg, 100 mg/L, 121 mg/l) and BUN (0.58 g/L, 1, 20 g/L, 1.82 g/l) in less than a month, the renal failure associated with hyperproteinemia of 81 g/l, a serum albumin of 39.53 g /l, elevated α2 and β2 globulin (11.83 g/l and 7.78 g/l), hyperuricemia [omicsonline.org]

  Hypercalcemia and hyperuricemia can occur as a result of myeloma and can contribute to renal failure. In addition, the myeloma cells can directly infiltrate the kidneys resulting in dysfunction. [cancer.ucsf.edu]

• Hypogammaglobulinemia

  Serum protein electrophoresis usually is normal or shows hypogammaglobulinemia. Bence Jones proteinuria (type κ ) is present in 10 to 15% of patients. [merckmanuals.com]

If others confirm our data, BD may become the preferred initial treatment for patients with LCDD. Copyright© 2009 Ferrata Storti Foundation [doi.org]

The treatment continued with hemodialysis 3 times per week with chemotherapy protocol bortezomib, doxorubicin, dexamethasone. [ncbi.nlm.nih.gov]

Treatment may include chemotherapy with a drug called Bortezomib ; autologous stem cell transplantation ; immunomodulatory drugs; and/or kidney transplant. [rarediseases.info.nih.gov]

This case supports the observation of Hobbs that patients with Bence-Jones myeloma may have a poorer prognosis than those with otherwise typical IgG or IgA myeloma. [ncbi.nlm.nih.gov]

Myeloma tubulopathy lesions may be present and in this case seems to be associated with a poor prognosis. Despite its rarity, the prognosis of this disease is poor because as than half of the patients die or progress toward ESRD within 2 years. [omicsonline.org]

Although the prognosis for long term kidney health is poor, there are some studies which have shown that some patients have done relatively well. [unckidneycenter.org]

Fewer mature plasma cells indicate a poorer prognosis. Bone marrow microvessel density: A high degree of new blood vessel development in the bone marrow indicates active tumor growth and thus a poorer prognosis. [emedicinehealth.com]

Miscellaneous diseases of uncertain etiology. In : Travis WD, Colby TV, Koss MN, Rosado-de-Christenson ML, Müller NL, King TE, eds. Non Neoplastic Disorders of the Lower Respiratory Tract. [erj.ersjournals.com]

Etiology Deletions, insertions or point mutations in the constant 1 (CH1) domain of the IgH are acquired during the process of somatic hypermutation. [atlasgeneticsoncology.org]

Etiology, Epidemiology and Pathophysiology Etiology The cause or causes of myeloma are unknown, but there is some evidence to support a number of theories of its origin, including viral, genetic, and exposure to toxic chemicals, the most notable being [cancer.ucsf.edu]

In a group of 13 patients with acute or chronic renal failure of various etiologies, including secondary amyloidosis, and end-stage renal failure on hemodialysis, FLC concentrations were increased in all patients, but the K/L FLC ratio was within the [clinchem.aaccjnls.org]

(Goossens et al., 1998) Epidemiology Mu HCD affects predominantly Caucasian men in their 5th -6 th decades. [atlasgeneticsoncology.org]

[…] represent an extramedullary manifestation of multiple myeloma while relapsing after chemotherapy. [21] LCDD can also complicate other B-cell lymphomas, such as lymphoplasmacytic lymphoma, marginal-zone lymphoma, and chronic lymphocytic lymphoma. [29] Epidemiology [emedicine.medscape.com]

Spain [7] 6 2/4 37-74 Europe [2] 63 40/23 58 USA [6] 34 17/17 • MIDD : 57,4 ± 2,12 • MIDD+ tubulopathy myeloma : 67,1 ± 3,92 Senegal 3 1/2 • LCDD : 61 • LCDD + tubulopathy myeloma : 47 et 69 Table 1: Monoclonal Immunoglobulin Deposition Disease (MIDD) epidemiology [omicsonline.org]

Epidemiology It is estimated that in 2017, approximately 30,280 new cases of multiple myeloma will be identified in the U.S., representing 1% of all malignancies and 10% of all hematological malignancies. [cancer.ucsf.edu]

Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy. Clin J Am Soc Nephrol. 2006 Nov. 1(6):1342-50. [Medline]. Buxbaum JN, Chuba JV, Hellman GC, Solomon A, Gallo GR. [emedicine.medscape.com]

Islet amyloid and type 2 diabetes; from molecular misfolding to islet pathophysiology. Biochim Biophys Acta. 2001;1537:179-203. Sanchorawala V, Wright DG, Seldin DC, et al. [rarediseases.org]

Serum κ and λ light chains are elevated in Crohn’s models and using an experimental blocker prevents these bowel symptoms. [mastattack.org]

In most cases, plasmapheresis is used in combination with corticosteroids and immunosuppressive drugs to prevent production of abnormal proteins or to treat the underlying disease. [ncbi.nlm.nih.gov]

Platelets, in combination with certain plasma proteins, help produce blood clots, which prevent bleeding. [emedicinehealth.com]

Experiments have demonstrated that SecB does not recognize leader peptides at all [ Randall 1990 ], but instead recognizes some feature of incompletely folded MBP, which vanishes in the mature MBP, and that SecB and the leader peptide each serve to prevent [cureffi.org]

Commonly, preventative antivirals (acyclovir or Valtrex) and anti-Pneumocystis carinii (Septra or dapsone) medications are given if corticosteroids are used in the treatment regimen. [cancer.ucsf.edu]