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PubMed2004

[Sjögren's syndrome with solitary nodular pulmonary amyloidosis].

We describe a case of limited pulmonary amyloidosis with Sjögren syndrome. A 58-yr-old woman was referred to our hospital because of an abnormal chest radiograph (solitary small nodule) that was examined to investigate the cause of a persistent cough. A chest CT revealed a solitary small nodule in the left lower lung field. The specimens obtained by thoracoscopic surgery showed AL (kappa) amyloid deposits with lymphoplasmacytic infiltrate. Immunofixation of the serum and concentrated urine failed to demonstrate monoclonal immunoglobulins, and no amyloid deposits in the stomach were detected. She was subsequently diagnosed as having primary Sjögren's syndrome. Nodular pulmonary amyloidosis with Sjögren syndrome is very rare condition, and most cases present multiple nodules. As far as is known, this is the first report of a solitary nodule in pulmonary amyloidosis with Sjögren syndrome.

Amyloidosis↗
PubMed2004

Solitary spleen metastasis and amyloidosis in a patient with endometrial cancer.

BACKGROUND: In contrast to autopsy findings, solitary splenic metastases from solid tumors are extremely rare. It may occasionally be the first manifestation of recurrent solid cancers, and in particular of gynecologic malignancies. Secondary amyloidosis is also found in malignancy. CASE: A 52-year-woman originally diagnosed with a Stage IB, grade 2 endometrial carcinoma presented two and a half years later with a paroxysmal non-productive cough, weakness, loss of appetite and daily afternoon fever. On clinical examination wheezing on forced exhalation and painful splenomegaly was found. After an extensive work-up the patient underwent an explorative laparotomy and a splenectomy was performed. Histologic examination showed solitary spleen metastasis with amyloidosis. All symptoms resolved. As the patient had received a full course of postoperative irradiation after a total abdominal hysterectomy, six cycles of combination chemotherapy were administered. The patient remains free of recurrence, 46 months later. CONCLUSION: A case of solitary spleen metastasis with amyloidosis in a patient with endometrial cancer is presented.

Amyloidosis↗
PubMed1978

[Diagnostic problems of primary amyloidosis].

Surgical liver biopsy specimen of a women aged 58 with "biliary complaints" showed amyloid deposits of unknown nature. After a nearly two-years-course of the disease the patient died of cardiac and renal failure. Clinical findings and laboratory tests without abnormal serum globulins suggested primary amyloidosis. Morphologically amyloid was present in the heart, tongue, striated muscles and in many parenchymatous organs and endocrine glands. By electron microscopy no difference was revealed between the primary and secondary type, respectively. Differenciation between primary and secondary form of the amyloid could only be achieved by the demonstratin of resistance of the deposits against induced proteolysis with trypsin digestion under the polarization microscope. The same result was obtained in two cases of senile amyloidosis. The case presented indicates that increased level of pathologic globulins is not an obligatory phenomenon in primary amyloidosis.

Amyloid↗
PubMedDate not supplied

Perianal condyloma-like lesions in multiple myeloma associated amyloidosis.

Systemic types of amyloidosis include those associated with plasma cell dyscrasia, as in multiple myeloma. Here we describe a 57-year-old woman who was diagnosed as having multiple myeloma IgG lambda. Six months after the diagnosis of myeloma, mucocutaneous lesions began to develop, with ecchymoses in the body folds and eyelid and periorbital purpura. Pedunculated condylomatous tumours began to develop in the perianal area. The excisional biopsy of a perianal nodule revealed a faintly eosinophilic, amorphous material replacing almost the entire dermis, in association with ectatic, endothelial-lined vascular spaces. The dermal deposits showed affinity with Congo Red stain. There were no histopathological features typical of condylomata acuminata. A diagnosis of cutaneous myeloma-associated amyloidosis was established. To our knowledge, this is the second reported case of condyloma-like perianal lesions in multiple myeloma-associated amyloidosis.

Amyloidosis↗
PubMed2004

Therapeutic options in systemic AL amyloidosis.

Systemic amyloid light chain (AL) amyloidosis is a severe disease with unfavourable prognosis. Since the late 1970s different therapeutic modalities in AL amyloidosis have been investigated, trying to prolong survival. This review deals with the therapeutic modalities in AL amyloidosis to date, and highlights future perspectives.

Amyloidosis↗
PubMed2004

[Diagnosis and therapy for beta2-microglobulin amyloidosis in the K/DOQI guideline].

According to the Kidney disease outcomes quality initiative (K/DOQI) guideline, in order to decrease risk of beta2-microglobulin amyloidosis, noncuprophane (EVIDENCE), high-flux dialyzers (OPINION) should be used. No currently available therapy, except kidney transplantation, can stop disease progression of beta2-microglobulin amyloidosis or provide symptomatic relief (EVIDENCE). Screening for beta2-microglobulin amyloidosis, including measurement of serum levels of beta2-microglobulin, is not recommended (OPINION).

Adrenal Cortex Hormones↗
PubMed2004

[Cases report and literature review of laryngeal amyloidosis].

OBJECTIVE: To have a better understand about mechanism, classification, clinical manifestation, diagnosis and treatment of the laryngeal amyloidosis. METHOD: Among 11 cases of clinical material of patients suffering from laryngeal amyloidosis, 5 cases operated by indirect laryngoscope, 1 case by direct laryngoscope, 2 cases by laryngofissure, 1 case by vertical hemilaryngectomy with glottis reconstruction, 2 cases weren't operated. RESULT: Three cases lost follow-up, 2 cases were recurred, and 6 cases' condition remained stable. CONCLUSION: (1) Symmers' classification is generally accepted and has clinical value; (2) The Congo red staining is the classic and most specific histochemical test, the fibrils demonstrate characteristic apple-green birefringence when examined under polarized light microscopy, which provide basis on confirming the diagnosis. (3) Laryngeal amyloidosis usually indicated localized disease with systemic and secondary involvement uncommon, however, potential for an underlying disease state such as multiple myeloma, a full medical evaluation for systemic disease should be performed. (4) Management depends on the severity of the symptoms in regard to the individual patients and in some case, can be conservative observation. If disease is causing significant symptom or showing signs of increasing in size, surgical intervention must be carried out. (5) Recurrence maybe become manifested after several years and long-term follow-up is recommended.

Adolescent↗
PubMed2005

Early lymphocyte recovery predicts superior survival after autologous hematopoietic stem cell transplantation for patients with primary systemic amyloidosis.

PURPOSE: Absolute lymphocyte count recovery at day 15 (ALC-15) post-autologous stem cell transplantation (ASCT) is a powerful prognostic indicator for survival for multiple hematologic malignancies and metastatic breast cancer. The relationship of ALC-15 with clinical outcomes in primary systemic amyloidosis is unknown. EXPERIMENTAL DESIGN: We evaluated 145 consecutive patients with primary systemic amyloidosis who underwent ASCT at the Mayo Clinic from 1996 to 2003. The ALC-15 threshold was set at 500 cells/microL based on our previous observations. RESULTS: The median patient follow-up was 22 months (range, 3-87 months). Higher hematologic complete response was observed in patients with an ALC-15 > or = 500 cells/microL compared with patients with an ALC-15 < 500 cells/microL (41% versus 21%, P < 0.0008, respectively). The median overall survival and progression-free survival times were significantly better for the 59 patients that achieved an ALC-15 > or = 500 cells/microL compared with 86 patients with ALC-15 < 500 cells/microL (not reached versus 53 months, P < 0.0003 and not reached versus 27 months, P < 0.0001, respectively). Multivariate analysis showed ALC-15 to be an independent prognostic factor for overall survival and progression-free survival. CONCLUSIONS: ALC-15 > or = 500 cells/microL is associated with significantly improved clinical outcomes following ASCT in patients with primary systemic amyloidosis.

Adult↗
PubMed1992

[An unusual case of localized form of primary macroglobulinemia developing from a nodular primary pulmonary amyloidosis].

The authors reported here a case of primary pulmonary amyloidosis, which developed into a localized form of primary macroglobulinemia (PMG) 10 years later. A nodular shadow was pointed out on routine chest x-ray films of a 61-year-old Japanese male in 1977. In 1981, a diagnosis of nodular primary pulmonary amyloidosis was made by percutaneous lung biopsy. At that time, he suffered from signs and symptoms of chronic cold agglutinin disease (CCAD). Cold agglutinins were IgM-kappa antibodies. In 1986, serum immunoelectrophoresis demonstrated the presence of a small amount of monoclonal IgM-kappa. In 1987, the patient was readmitted because of pleural effusions. In the pleural effusion, the IgM level was 3,341 mg/dl and the titer of cold agglutinin was 32,000. Cytological examinations of pleural effusion showed the proliferation of lymphocytes, lymphoid cells and plasma cells. These cells showed the monoclonality of IgM-kappa by the peroxidase-antiperoxidase method. At post-mortem examination in March 1988, PA and PMG were found, but both lesions were localized only in the thoracic cavity. It is suggested that primary amyloidosis (PA) as well as CCAD and monoclonal gammopathy of undetermined significance may be one of the pre-neoplastic conditions of PMG.

Aged↗
PubMedDate not supplied

Primary AL (kappa-light chain) amyloidosis manifesting as peripheral neuropathy in a young male without increase in serum and urine immunoglobulin load: a diagnostic challenge.

Primary systemic or AL amyloidosis is a multisystem disorder characterized by diffuse extracellular infiltration of a fibrillar protein of monoclonal light chain origin (AL). Majority of the patients have monoclonal immunoglobulin in serum and/or urine and some have clonal proliferation of plasma cells in their bone marrow. This disease has the widest spectrum of organ involvement, most commonly affecting the kidneys, heart and liver. Involvement of peripheral nervous system is not infrequent and may be the presenting feature of the disease process. Thus, recognition of peripheral neuropathy and affecting the kidney as an early symptom of AL amyloidosis may widen the scope for therapeutic intervention. We describe here a rare case of primary amyloidosis (AL) kappa-light chain presenting with clinical features of peripheral neuropathy and affecting the kidney and heart at an early age of 18 years, hitherto unreported in literature. The case was further interesting as it was not associated with increased serum/urine immunoglobulins or plasma cells in bone marrow. Diagnosis was confirmed using immuno-electron microscopy on sural nerve biopsy.

Adolescent↗
PubMed1992

[Bullous poikilodermatitic amyloidosis of the skin with junctional bulla development in IgG light chain plasmacytoma of the lambda type. Histology, immunohistology and electron microscopy].

A 75-year-old patient presented with bullae, poilokilodermatic skin and nail dystrophy as signs of systemic amyloidosis 1 year before an IgG myeloma of the lambda type was diagnosed. The skin lesions appeared at mechanically irritated locations on the trunk and at the tensor sites of the extremities. Histology showed a subepidermal blister and necrotic keratinocytes in the epidermis. There were amyloid deposits in the papillary dermis perivascular, and in the deep dermis around eccrine glands and in arrectores pilorum muscles. Polyclonal antibodies allowed classification of the deposits as amyloid composed of lambda light-chain immunoglobulins. Electron microscopy revealed globoid deposits of non-branching filaments typical of amyloid. The blister was formed at the level of the lamina lucida, with the lamina densa at the base of the bottom of the bulla. So far, junctional blister formation in bullous amyloidosis of the skin has been described only once. Our case is the second report of this blister type, and to our knowledge the first published report of a junctional blister in myeloma-associated systemic amyloidosis.

Actin Cytoskeleton↗
PubMedDate not supplied

[Conformational and intermolecular interactions of proteins in amyloidosis].

A conception on amyloidosis as a key factor of neuronal death in neurodegenerative diseases is stated. Experimental evidence is presented that amyloidosis is caused by alterations in the activity of a number of enzymes as well as conformational changes in pathogenic proteins. Arguments for amyloidosis as the universal biological mechanism of specific elimination of neurons showing changed metabolic and physiological status of cell differentiation are adduced. The final pattern of cell death seems to differ cardinally from that in both apoptosis and necrosis.

Amyloid↗
PubMedDate not supplied

Accumulation of Tc99m-DMSA-3 in the spleen in a case of multiple myeloma with associated amyloidosis.

We describe a case of a 58-year-old male with longstanding hypertension and Type 2 diabetes mellitus who developed sudden onset renal impairment. The first clue to the possible presence of amyloidosis in this case was provided by the radionuclide renal cortical scan performed with trivalent dimercapto succinic acid (Tc99m-DMSA-3), which revealed intense tracer uptake in the spleen suggesting amyloid deposit. Further workup to ascertain the cause of amyloidosis led to the diagnosis of multiple myeloma. We conclude that in cases of extra-renal or splenic accumulation of Tc99m-DMSA-3, a diagnosis of amyloidosis should be considered, in an appropriate clinical setting.

Amyloidosis↗
PubMedDate not supplied

Interleukin-6 (IL-6) -174 G/C polymorphism in familial Mediterranean fever patients with and without amyloidosis.

Familial Mediterranean fever (FMF) is a recessive disorder characterized by attacks of fever and inflammation. A sustained inflammatory reaction is observed in the disease course, and cytokine levels such as interleukin (IL)-1, IL-6 and tumor necrosis factor-alpha (TNF-alfa) are shown to increase during and between the attacks. In this study, we investigated the role of the functionally important IL-6 -174 G/C polymorphism in the clinical outcome of FMF and amyloidosis. One hundred and fifty-six FMF patients (80 with amyloidosis) and 90 healthy controls were studied. The genotype distributions and allele frequencies of the patients and the controls were found to be similar, and the differences between the groups were not statistically significant. The results show that IL-6 -174 G/C polymorphism is not associated with FMF and amyloidosis. The increase observed in cytokine levels during and between the attacks is more likely due to the inflammatory nature of the disease.

Amyloidosis↗
PubMedDate not supplied

Co-existent crescentic glomerulonephritis and renal amyloidosis: a case report and literature review.

Co-existent crescentic glomerulonephritis and renal amyloidosis have only rarely been reported thus far. To our knowledge, only about eighteen convincing cases (with >50% crescents) have been described, mostly in the context of rheumatoid arthritis. In this report, we add a case from our institution and review the existing literature, including a proposed mechanism of crescent formation in this setting. Although the relationship between crescentic glomerulonephritis, renal amyloidosis, and rheumatoid arthritis needs further investigation, we suggest that one should consider the development of crescentic glomerulonephritis with amyloidosis in patients with long-standing rheumatoid arthritis and worsening renal function.

Amyloidosis↗
PubMedDate not supplied

Primary gastric lymphoma and Helicobacter pylori infection with gastric amyloidosis.

Primary gastric lymphoma, an uncommon gastric tumor caused by infection with Helicobacter pylori, is rarely associated with gastric amyloidosis. Chronic bacterial infection is known to cause amyloidosis. We report a 53-year-old man who had an antral and duodenal mass with narrowing and ulceration on endoscopy and CT scan; endoscopic biopsy revealed gastric amyloidosis. Rapid urease test and serology for H. pylori were positive. Histology of resected specimen of distal stomach revealed primary gastric lymphoma, amyloid deposits and spiral organisms suggestive of H. pylori. Rectal biopsy was negative for amyloid. He remained well on follow-up after surgery and eradication of H. pylori.

Amyloidosis↗
PubMed2006

Primary cutaneous nodular amyloidosis in a patient with Sjögren's syndrome.

A 71-year-old woman with a history of Sjögren's syndrome presented for evaluation of a waxy nodule present on the scalp for 6 months. Histopathologic examination revealed deposition of homogenous eosinophilic material throughout the reticular dermis consistent with amyloidosis. Primary cutaneous nodular amyloidosis is a rare phenomenon characterized by the deposition of immunoglobin light chains by a clonal plasma cell population. Patients need to be monitored for progression to systemic amyloidosis or plasma cell dyscrasias.

Aged↗
PubMed2006

Primary pulmonary amyloidosis associated with multiple myeloma.

Amyloidosis is a syndrome characterized by the deposition of an insoluble proteinaceous material in the extracellular matrix of one or several organs. Respiratory tract involvement with amyloid is rare and deposition of lower respiratory tract has been recognized in a variety of situations with different presentations. Primary idiopathic amyloidosis may be a diagnostic problem because of its low incidence and its variable manifestations. We report herein a case with multiple myeloma presenting diffuse interstitial infiltration, in which pulmonary AL type amyloidosis was diagnosed through transbronchial lung biopsy.

Amyloidosis↗