• TRAPS


**Figure 1.** *Renal AA amyloidosis, Congo red 100×.*

#### **Figure 2.**

*Rheumatoid arthritis, complicated by renal AA amyloidosis with nephrotic syndrome.*


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**Figure 5.**

*Renal Amyloidosis*

**Figure 3.**

**Figure 4.**

*DOI: http://dx.doi.org/10.5772/intechopen.81677*

*Castleman's disease, unfixed gross specimen.*

*Castleman's disease, atypical lymphoid tissue, hematoxylin & eosin 100×.*

*Sclerosing angiomatoid nodular transformation of the spleen. Formalin-fixed gross specimen.*

#### **Table 3.**

*Spectrum of the diseases, associated with AA amyloidosis, personal data, unpublished.*

*Amyloid Diseases*

**Figure 1.**

**Figure 2.**

*Renal AA amyloidosis, Congo red 100×.*

*Rheumatoid arthritis, complicated by renal AA amyloidosis with nephrotic syndrome.*

*Spectrum of the diseases, associated with AA amyloidosis, personal data, unpublished.*

**Associated disease Patients (N) %** Rheumatoid arthritis 64 44.1 Ankylosing spondylitis 16 11.0 Psoriatic arthritis 7 4.8 Crohn's disease/ulcerative colitis 3 2.0 Sarcoidosis 1 0.7 Mediterranean fever 14 9.6 Hyper-IgD syndrome 1 0.7 Bronchiectasis 10 6.8 Osteomyelitis 7 4.8 Paraplegia 6 4.1 Tuberculosis 4 2.7 Chronic cutaneous ulcers 3 2.0 Cystic fibrosis 1 0.7 Lung tumors 3 2.0 Hodgkin's lymphoma 2 1.4 Castleman's disease 2 1.4 Sclerosing angiomatoid nodular transformation of the spleen 1 0.7 Total 145 100

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**Table 3.**

**Figure 3.** *Castleman's disease, unfixed gross specimen.*

**Figure 4.** *Castleman's disease, atypical lymphoid tissue, hematoxylin & eosin 100×.*

#### *Amyloid Diseases*

#### **Figure 6.**

*Sclerosing angiomatoid nodular transformation of the spleen. PAS 100×.*

#### **Figure 7.** *Renal AA amyloidosis, Congo red 100×, polarized light.*

**Figure 8.** *Renal AA amyloidosis, serum amyloid A, immunoperoxidase 100×.*

## **3. AL amyloidosis**

The precursor proteins of the fibrils in AL amyloidosis are monoclonal immunoglobulin light chains, produced by plasma cell clone. AL amyloidosis, which is the most prevalent type of systemic amyloidosis in the Western countries, sometimes

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**Figure 10.**

**Figure 9.**

*Renal AL amyloidosis, Congo red 100×.*

*Renal AL amyloidosis, Congo red 100×, polarized light.*

*Renal Amyloidosis*

over time [19–21].

(**Figures 12–17**).

*DOI: http://dx.doi.org/10.5772/intechopen.81677*

formerly it was known as "primary" [15–18].

is associated with B cell lymphoproliferative disorders—multiple myeloma, Waldenström macroglobulinemia and non-Hodgkin lymphomas [9–14]. However usually AL amyloidosis is associated with low-grade plasma cell clone and do not meet the criteria for multiple myeloma or lymphoplasmacytic lymphoma, therefore

macroglobulinemia, and 102—with AL amyloidosis ("primary").

sites of amyloid deposition in the kidney (**Figures 9–11**).

In the real practice, among 128 patients with biopsy-proven AL amyloidosis, followed in our unit, 25 were diagnosed with multiple myeloma, 1—with Waldenström

Kidneys and heart are the main sites of involvement in AL amyloidosis with the occurrence up to 70% of cases. Renal involvement typically presents with proteinuria or NS, which is manifested in more than 50% of patients at the time of diagnosis, and impaired kidney function progressing towards ESRD in about 20% of cases

AL amyloidosis is diagnosed by demonstration of monoclonal deposits in the

Kidney biopsy is usually indicated for significant proteinuria and/or renal insufficiency in patients with signs and symptoms of heart, liver, tongue, intestine, peripheral and autonomous nervous system and soft tissues damage

Monoclonal protein studies should be performed to match the monoclonal protein in circulation with the monoclonal deposits in the kidney (**Figure 18**).

#### *Renal Amyloidosis DOI: http://dx.doi.org/10.5772/intechopen.81677*

*Amyloid Diseases*

**Figure 6.**

**Figure 7.**

*Sclerosing angiomatoid nodular transformation of the spleen. PAS 100×.*

*Renal AA amyloidosis, Congo red 100×, polarized light.*

*Renal AA amyloidosis, serum amyloid A, immunoperoxidase 100×.*

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**Figure 8.**

**3. AL amyloidosis**

The precursor proteins of the fibrils in AL amyloidosis are monoclonal immunoglobulin light chains, produced by plasma cell clone. AL amyloidosis, which is the most prevalent type of systemic amyloidosis in the Western countries, sometimes

is associated with B cell lymphoproliferative disorders—multiple myeloma, Waldenström macroglobulinemia and non-Hodgkin lymphomas [9–14]. However usually AL amyloidosis is associated with low-grade plasma cell clone and do not meet the criteria for multiple myeloma or lymphoplasmacytic lymphoma, therefore formerly it was known as "primary" [15–18].

In the real practice, among 128 patients with biopsy-proven AL amyloidosis, followed in our unit, 25 were diagnosed with multiple myeloma, 1—with Waldenström macroglobulinemia, and 102—with AL amyloidosis ("primary").

Kidneys and heart are the main sites of involvement in AL amyloidosis with the occurrence up to 70% of cases. Renal involvement typically presents with proteinuria or NS, which is manifested in more than 50% of patients at the time of diagnosis, and impaired kidney function progressing towards ESRD in about 20% of cases over time [19–21].

AL amyloidosis is diagnosed by demonstration of monoclonal deposits in the sites of amyloid deposition in the kidney (**Figures 9–11**).

Kidney biopsy is usually indicated for significant proteinuria and/or renal insufficiency in patients with signs and symptoms of heart, liver, tongue, intestine, peripheral and autonomous nervous system and soft tissues damage (**Figures 12–17**).

Monoclonal protein studies should be performed to match the monoclonal protein in circulation with the monoclonal deposits in the kidney (**Figure 18**).

**Figure 9.** *Renal AL amyloidosis, Congo red 100×.*

**Figure 10.** *Renal AL amyloidosis, Congo red 100×, polarized light.*

Different treatment regimens had been used since 1997, when melphalan was introduced—melphalan and prednisone (MP), melphalan and dexamethasone (MD), and high dose melphalan with autologous stem cell transplantation (ASCT).

#### **Figure 11.**

*Renal AL amyloidosis, light chain lambda, immunofluorescence 100×.*

#### **Figure 12.**

*AL amyloidosis, electrocardiogram, low-voltage waves in all leads.*

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**Figure 16.**

*AL amyloidosis, "racoon eye" symptom.*

*AL amyloidosis, "shoulder pad" symptom.*

*Renal Amyloidosis*

**Figure 14.**

**Figure 15.**

*AL amyloidosis, macroglossia.*

*DOI: http://dx.doi.org/10.5772/intechopen.81677*

**Figure 13.** *AL amyloidosis, echocardiogram, myocardial mirror-like appearance.*

*Amyloid Diseases*

**Figure 11.**

**Figure 12.**

*Renal AL amyloidosis, light chain lambda, immunofluorescence 100×.*

*AL amyloidosis, electrocardiogram, low-voltage waves in all leads.*

*AL amyloidosis, echocardiogram, myocardial mirror-like appearance.*

Different treatment regimens had been used since 1997, when melphalan was introduced—melphalan and prednisone (MP), melphalan and dexamethasone (MD), and high dose melphalan with autologous stem cell transplantation (ASCT).

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**Figure 13.**

**Figure 14.** *AL amyloidosis, macroglossia.*

**Figure 15.** *AL amyloidosis, "shoulder pad" symptom.*

**Figure 16.** *AL amyloidosis, "racoon eye" symptom.*

**Figure 17.** *AL amyloidosis, spontaneous subcutaneous hemorrhages.*

**Figure 18.** *Serum electrophoresis, M-spike.*

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*Renal Amyloidosis*

regimens (**Figure 19**) [23].

**4. Conclusions**

**Acknowledgements**

**Conflict of interest**

**Author details**

Elena Zakharova

*DOI: http://dx.doi.org/10.5772/intechopen.81677*

Currently recommended treatment for AL amyloidosis, including cyclophosphamide-thalidomide-dexamethasone (CTD), bortezomib-dexamethasone (BD), cyclophosphamide-bortezomib-dexamethasone(CBD) regimens with relatively fast hematological response were adopted from multiple myeloma treatment protocols [22]. In our experience of treatment of systemic "primary" AL amyloidosis with kidney involvement using different regimens over almost three decades, cumulative survival did not differ statistically between melphalan-based and bortezomib-based

Clinical diagnosis of AA and AL systemic amyloidosis, most often affecting kidneys, is based on the presence of proteinuria or nephrotic syndrome and impaired kidney function in patients with extrarenal manifestations. Kidney biopsy is crucial for the diagnostics, and while Congo red staining with examination of Congopositive material in the polarized light is confirmative for amyloidosis as such, immunofluorescence and immunohistochemistry technics are helpful to distinguish AA and AL types. Differential diagnostics of AA and AL types guides the treatment strategies. In cases when neither AA nor AL amyloidosis are confirmed, one should consider rare types of amyloidosis, based on the presence of renal involvement—

Author thanks doctors Olga Vorobova, Ekaterina Stolyarevich, Vladimir Bedin,

Mikhail Tavobilov, Evgeny Shutov, Eugene Nikitin, Marina Rybakova and Igor

Miloserdov for their help in diagnostics and treatment of the patients.

ALECT2, AapolA I, II and IV, AFib or ALys amyloidosis.

Author declares no conflict of interests.

Postgraduate Education, Moscow, Russian Federation

\*Address all correspondence to: helena.zakharova@gmail.com

provided the original work is properly cited.

© 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

City Clinical Hospital n.a. S.P. Botkin, Russian Medical Academy of Continuous

**Figure 19.** *Treatment results in 49 patients with AL amyloidosis, personal data [23].*

*Renal Amyloidosis DOI: http://dx.doi.org/10.5772/intechopen.81677*

Currently recommended treatment for AL amyloidosis, including cyclophosphamide-thalidomide-dexamethasone (CTD), bortezomib-dexamethasone (BD), cyclophosphamide-bortezomib-dexamethasone(CBD) regimens with relatively fast hematological response were adopted from multiple myeloma treatment protocols [22]. In our experience of treatment of systemic "primary" AL amyloidosis with kidney involvement using different regimens over almost three decades, cumulative survival did not differ statistically between melphalan-based and bortezomib-based regimens (**Figure 19**) [23].
