Multicentric Castleman's disease associated with renal amyloidosis and pure red cell aplasia

 A 50-year-old man was admitted suffering from severe anemia and renal dysfunction. He had been admitted for the first time at the age of 49, and was diagnosed with multicentric Castleman's disease (MCD) and secondary amyloidosis. At that time, marked erythroid hypoplasia was demonstrated by both aspiration and biopsy of bone marrow. A diagnosis of pure red-cell aplasia (PRCA) was made. Immunosuppressive agents improved his symptoms and laboratory data. We report here a very rare case of PRCA following MCD and amyloidosis, and with reference to the literature, we discuss the relation between MCD and related diseases. Received: February 12, 1998 / Accepted: June 17, 1998     

A fatal case of primary cutaneous gamma–delta T‐cell lymphoma complicated by HLH and cardiac amyloidosis

Gamma–delta T‐cell lymphomas (GD‐TCL) are rare and rapidly fatal neoplasms that are often associated with Hemophagocytic Lymphohistiocytosis (HLH), a syndrome of fevers, cytopenias, and multiorgan failure that often leads to a rapid death. We report the first case demonstrating an association between GD‐TCL, HLH, and cardiac amyloidosis, presenting a novel mechanism for rapid deterioration in these patients.     

CHOLESTASIS AND RENAL FAILURE IN A PATIENT WITH SECONDARY AMYLOIDOSIS

Amyloid, was first described in the 19th century by Virchow, which means starch or cellulose. Extracellular deposition of this unique protein fibrils in tissues, often leading organ dysfunction is known as amyloidosis. In systemic amyloidosis, amyloid fibrils may deposit in different organs including kidneys, heart and liver. Although liver is effected frequently, clinical liver disease is rare. There are a few cases that secondary amyloidosis presented with hepatic involvement and cholestasis which seems to be limited to primary amyloidosis. Hepatic amyloidosis with cholestasis may be a predictor of involvement of other organs and thus of poor prognosis. We report a case of a 67 year old man with renal failure and prominent cholestasis due to AA amyloid deposition in the liver.     

Amyloid goiter related with Crohn's disease: A rare association: Amyloid goiter secondary to Crohn's disease

INTRODUCTION

Amyloid goiter (AG) is characterized by enlargement of the thyroid gland as a result of extensive amyloid deposition in a bilateral and diffuse manner.

PRESENTATION OF CASE

A 58-year-old male patient was diagnosed of Crohn''s Disease (CD). He was admitted to our clinic with complaint of respiratory distress and rapid growth swelling in the neck. Ultrasound examination revealed huge multinodular goiter on both sides of thyroid gland. We performed bilateral total thyroidectomy. Pathological evaluation revealed AG.

DISCUSSION

Amyloid leads to degeneration in tissues, thereby disrupts the function of the relevant organs. It is important to distinguish AG from other reasons of goiter, particularly thyroid medullary cancer that can cause amyloid deposition in thyroid gland. Secondary amyloidosis frequently involves thyroid gland at microscopic level, but rarely causes goiter. An analysis of current literature revealed that only few cases of AG occurred secondary to CD. Herein we presented a case of AG who has rapidly growing goiter that associated with CD.

CONCLUSION

AG must be kept in mind in case of rapidly growing goiter, especially in patients with chronic inflammatory bowel diseases.     

Successful Long-Term Outcome of the First Combined Heart and Kidney Transplant in a Patient with Systemic AL Amyloidosis

Simultaneous cardiac and renal involvement is associated with a particularly poor prognosis in patients with AL amyloidosis (AL-A). We report the first case of a successful long-term outcome of combined heart and kidney transplantation not followed by autologous stem cell transplantation in a patient with systemic AL-A. The recipient was a 46-year-old man with end-stage renal failure associated with serious cardiac involvement in the context of AL-A. Before transplantation, two courses of oral melphalan plus prednisone induced partial hematologic remission, as shown by the decrease in circulating free light chain with no improvement of renal or heart function. The patient underwent combined heart and kidney transplantation as a rescue treatment. During the follow-up period (36 months), plasma cell dyscrasia remains in complete remission, with normal free lambda light chain levels and no recurrence of amyloid deposition on heart and kidney grafts. This case report demonstrates that combined heart and kidney transplantation not systematically associated with stem cell transplantation may be considered an additional therapeutic option in AL-A patients with severe organ dysfunction and partial hematologic remission.     

Adverse Prognostic Factors for Morbidity and Mortality During Peripheral Blood Stem Cell Mobilization in Patients with Light Chain Amyloidosis

Patients with immunoglobulin light chain (AL) amyloidosis undergoing peripheral blood hematopoietic stem cell (PBSC) mobilization for autologous hematopoietic stem cell transplantation (auto-HCT) can experience significant morbidity and mortality. The purpose of this study was to characterize the adverse events and identify prognostic factors associated with the development of morbidity and mortality in patients with AL amyloidosis who had begun PBSC mobilization for auto-HCT. A retrospective study was performed in 101 consecutive patients with AL amyloidosis who underwent PBSC mobilization for auto-HCT between January 2006 and December 2013. A composite primary endpoint of morbidity and mortality during PBSC mobilization was used. Forty-one patients (41%) experienced at least 1 adverse event, including 4 deaths during PBSC mobilization. Adverse events included in this composite endpoint were cardiac events, thromboembolic events, bleeding events, unplanned hospitalization, weight gain?>2% necessitating diuretic intervention, and death. Low serum albumin levels, elevated N-terminal pro-brain natriuretic peptide, and increased interventricular septal thickness were significantly associated with the composite primary endpoint (P?=?.024, .001, and .006, respectively). The median progression-free survival from the start of PBSC mobilization was 4.7 years, and the median overall survival was 6.5 years. In general, PBSC mobilization is associated with minimal complications, but patients with AL amyloidosis can experience more frequent and severe complications, such as volume overload and weight gain. Careful patient selection is warranted in patients with AL amyloidosis before proceeding to PBSC mobilization and auto-HCT.     

Human apolipoprotein A-I Gly26Arg stimulation of inflammatory responses via NF-kB activation: Potential roles in amyloidosis?

The cascade of molecular events leading to Human apolipoprotein A–I (apoA–I) amyloidosis is not completely understood, not even the pathways that determine clinical manifestations associated to systemic protein deposition in organs such as liver, kidney and heart. About twenty natural variants of apoA–I were described as inducing amyloidosis, but the mechanisms driving their aggregation and deposition are still unclear. We previously identified that the mutant Gly26Arg but not Lys107-0 induced the release of cytokines and reactive oxygen species from cultured RAW 264.7 murine macrophages, suggesting that part of the pathogenic pathway could elicit of an inflammatory signal. In this work we gained deep insight into this mechanism and determined that Gly26Arg induced a specific pro-inflammatory cascade involving activation of NF-κB and its translocation into the nucleus. These findings suggest that some but not all apoA–I natural variants might promote a pro-oxidant microenvironment which could in turn result in oxidative processing of the variants into a misfolded conformation.