Heredofamilial Amyloidosis.

A variety of familial forms of amyloidosis have been described. Most of them are rare and occur in limited geographic areas. The most common and best studied is an autosomal recessive

condition called familial Mediterranean fever.[172] This is a febrile disorder of unknown cause characterized by attacks of fever accompanied by inflammation of serosal surfaces, including

peritoneum, pleura, and synovial membrane. This disorder is encountered largely in individuals of Armenian, Sephardic Jewish, and Arabic origins. It is associated with widespread tissue

involvement indistinguishable from reactive systemic amyloidosis. The amyloid fibril proteins are made up of AA proteins, suggesting that this form of amyloidosis is related to the

recurrent bouts of inflammation that characterize this disease. The gene for familial Mediterranean fever has been cloned, and its product is called pyrin (for its relation to fever). Although

its exact function is not known, it has been suggested that pyrin is responsible for regulating acute inflammation, presumably by inhibiting the function of neutrophils.[173] The relationship

of this mutation to the disease is not understood.

In contrast to familial Mediterranean fever, a group of autosomal dominant familial disorders is characterized by deposition of amyloid predominantly in the nerves—peripheral and

autonomic. These familial amyloidotic polyneuropathies have been described in different parts of the world. As mentioned previously, in all of these genetic disorders, the fibrils are made

up of mutant transthyretins (ATTR).

Localized Amyloidosis.

Sometimes, amyloid deposits are limited to a single organ or tissue without involvement of any other site in the body. The deposits may produce grossly detectable nodular masses or be

evident only on microscopic examination. Nodular (tumor-forming) deposits of amyloid are most often encountered in the lung, larynx, skin, urinary bladder, tongue, and the region about

the eye. Frequently, there are infiltrates of lymphocytes and plasma cells in the periphery of these amyloid masses, raising the question of whether the mononuclear infiltrate is a response

to the deposition of amyloid or instead is responsible for it. At least in some cases, the amyloid consists of AL protein and may therefore represent a localized form of immunocyte-derived

amyloid.

Endocrine Amyloid.

Microscopic deposits of localized amyloid may be found in certain endocrine tumors, such as

medullary carcinoma of the thyroid gland, islet tumors of the pancreas, pheochromocytomas, and undifferentiated carcinomas of the stomach, and in the islets of Langerhans in patients

with type II diabetes mellitus. In these settings, the amyloidogenic proteins seem to be derived either from polypeptide hormones (e.g., medullary carcinoma) or from unique proteins (e.g.,

islet amyloid polypeptide).

Amyloid of Aging.

Several well-documented forms of amyloid deposition occur with aging.[174] Senile systemic amyloidosis refers to the systemic deposition of amyloid in elderly patients (usually in their

seventies and eighties). Because of the dominant involvement and related dysfunction of the heart, this form was previously called senile cardiac amyloidosis. Those who are symptomatic

present with a restrictive cardiomyopathy and arrhythmias. The amyloid in this form is composed of the normal TTR molecule. In addition to the sporadic senile systemic amyloidosis,

another form, affecting predominantly the heart, that results from the deposition of a mutant form of TTR has also been recognized. Approximately 4% of the black population in the

United States is a carrier of the mutant allele, and cardiomyopathy has been identified in both homozygous and heterozygous patients. The precise prevalance of patients with this mutation

who develop clinically manifest cardiac disease is not known.

Pathogenesis.

Amyloidosis results from abnormal folding of proteins, which are deposited as fibrils in extracellular tissues and disrupt normal function. Misfolded proteins are often unstable and selfassociate,

ultimately leading to the formation of oligomers and fibrils that are deposited in tissues. The reason diverse conditions are associated with amyloidosis may be that each of these

conditions results in excessive production of proteins that are prone to misfolding. The proteins that form amyloid fall into two general categories: (1) normal proteins that have an inherent

tendency to fold improperly, associate and form fibrils, and do so when they are produced in

Figure 6-54Proposed schema of the pathogenesis of the major forms of amyloid fibrils.

Figure 6-55Amyloidosis of the kidney. The glomerular architecture is almost totally obliterated by the massive accumulation of amyloid.

Figure 6-56Cardiac amyloidosis. The atrophic myocardial fibers are separated by structureless, pink-staining amyloid (arrows).

References

1. Janeway CA, Jr, Medzhitov R: Innate immune recognition. Annu Rev Immunol 20:197, 2002.

2. Takeda K, Kaisho T, Akira S: Toll-like receptors. Ann Rev Immunol 21:335, 2003.

3. Cyster JG: Chemokines and cell migration in secondary lymphoid organs. Science 286:2098, 1999.

4. Mebius RE: Organogenesis of lymphoid tissues. Nat Rev Immurol 3:292, 2003.

5. Davis MM, et al: Ligand recognition by alpha beta T cell receptors. Annu Rev Immunol 16:523, 1998.

6. Hennecke J, Wiley DC: T cell receptor-MHC interactions up close. Cell 104:1, 2001.

7. Weiss A: Structure and function of the T cell antigen receptor. J Clin Invest 86:1015, 1990.

8. Hayday AC: gd cells: a right time and a right place for a conserved third way of protection. Annu Rev Immunol 18:975, 2000.

9. Lenschow DJ, Walunas TL, Bluestone JA: CD28/B7 system of T cell costimulation. Annu Rev Immunol 14:233, 1996.

10. von Andrian UH, Mackay CR: T-cell function and migration. Two sides of the same coin. N Engl J Med 343:1020, 2000.

11. Abbas AK, et al: Functional diversity of helper T lymphocytes. Nature 383:787, 1996.

12. Clark EA, Ledbetter JA: How B and T cells talk to each other. Nature 367:425, 1994.

13. Clark LB, Foy TM, Noelle RJ: CD40 and its ligand. Adv Immunol 63:43, 1996.

14. Mellman I, Steinman RM: Dendritic cells: specialized and regulated antigen processing machines. Cell 106:255, 2001.

15. Banchereau J, et al: Immunobiology of dendritic cells. Annu Rev Immunol 18:767, 2000.

16. Cerwenka A, Lanier LL: Natural killer cells, viruses and cancer. Nat Rev Immunol 1:41, 2001.

17. Bjorkman PJ: MHC restriction in three dimensions: a view of T cell receptor/ligand interactions. Cell 89:167, 1997.

18. Germain RN: MHC-dependent antigen processing and peptide presentation: providing ligands for T lymphocyte activation. Cell 76:287, 1994.

19. Hammer J, Sturniolo T, Sinigaglia F: HLA class II peptide binding specificity and autoimmunity. Adv Immunol 66:67, 1997.

20. Kay AB: Allergy and allergic diseases. N Engl J Med 344:30, 109, 2001.

21. Gould HJ, et al.: The biology of IgE and the basis of allergic disease. Annu Rev Immunol 21:579, 2003.

22. Costa JJ, et al: The cells of the allergic response: mast cells, basophils, and eosinophils. JAMA 278:1815, 1997.

23. Romagnani S: Cytokines and chemoattractants in allergic inflammation. Mol Immunol 38:881, 2002.

24. Murphy KM, Reiner SL: The lineage decisions of helper T cells. Nat Rev Immunol 2:933, 2002.

25. Kawakami T, Galli SJ: Regulation of mast-cell and basophil function and survival by IgE. Nat Rev Immunol 2:773, 2002.

26. Robinson DS, Kay AB, Wardlaw AJ: Eosinophils. Clin Allergy Immunol 16:43, 2002.

27. Ono SJ: Molecular genetics of allergic diseases. Annu Rev Immunol 18:347, 2000.

28. Kemp SF, Lockey RF: Anaphylaxis: a review of causes and mechanisms. J Allergy Clin Immunol 110:341, 2002.

29. Baumann U, Schmidt RE: The role of Fc receptors and complement in autoimmunity. Adv Exp Med Biol 495:219, 2001.

30. Russell JH, Ley TJ: Lymphocyte-mediated cytotoxicity. Annu Rev Immunol 20:323, 2002.

31. Heeger PS: T-cell allorecognition and transplant rejection: a summary and update. Am J Transpl 3:525, 2003.

32. Libby P, Pober JS: Chronic rejection. Immunity 14:387, 2001.

33. Pascual M, et al: Strategies to improve long-term outcomes after renal transplantation. N Engl J Med 346:580, 2002.

34. Vogelsang GB, Lee L, Bensen-Kennedy DM: Pathogenesis and treatment of graft-versus-host disease after bone marrow transplant. Annu Rev Med 54:29, 2003.

35. Kumar V, et al: Role of murine NK cells and their receptors in hybrid resistance. Curr Opin Immunol 19:52, 1997.

36. Ruggeri L, et al: Effectiveness of donor natural killer cell reactivity in mismatched hematopoietic transplants. Science 295:2097, 2002.

37. Kyewski B, et al: Promiscuous gene expression and central T-cell tolerance: more than meets the eye. Trends Immunol 23:364, 2002.

38. Anderson MS, et al: Projection of an immunological self shadow within the thymus by the AIRE protein. Science 298:1395, 2002.

39. Van Parijs L, Abbas AK: Homeostasis and self-tolerance in the immune system: turning lymphocytes off. Science 280:243, 1998.

40. Walker LS, Abbas AK: The enemy within: keeping self-reactive T cells at bay in the periphery. Nat Rev Immunol 2:11, 2002.

41. Goodnow CC, et al: Self-tolerance checkpoints in B lymphocyte development. Adv Immunol 59:279, 1995.

42. Schwartz RH: T cell anergy. Ann Rev Immunol 21:305, 2003.

43. Shevach EM: CD4+ CD25+ suppressor T cells: more questions than answers. Nat Rev Immunol 2:389, 2002.

44. Ramsdell F: Foxp3 and natural regulatory T cells: key to a cell lineage? Immunity 19:165, 2003.

45. Siegel RM, et al: The multifaceted role of Fas signaling in immune cell homeostasis and autoimmunity. Nat Immunol 1:469, 2000.

46. Nagata S: Fas ligand-induced apoptosis. Annu Rev Genet 33:29, 1999.

47. Marsden VA, Strasser A: Control of apoptosis in the immune system. Ann Rev Immunol 21:71, 2003.

48. Marrack P, Kappler J, Kotzin BL: Autoimmune disease: why and where it occurs. Nat Med 7:899, 2001.

49. Kamradt T, Mitchison NA: Tolerance and autoimmunity. N Engl J Med 344:655, 2001.

50. Encinas JA, Kuchroo VK: Mapping and identification of autoimmunity genes. Curr Opin Immunol 12:691, 2000.

51. Wakeland EK, et al: Delineating the genetic basis of systemic lupus erythematosus. Immunity 15:397, 2001.

52. Nelson BH: Interleukin-2 signaling and the maintenance of self-tolerance. Curr Dir Autoimmun 5:92, 2002.

53. Ravetch JV, Bolland S: IgG Fc receptors. Annu Rev Immunol 19:275, 2001.

54. Vanderlugt CL, Miller SD: Epitope spreading in immune-mediated diseases: implications for immunotherapy. Nat Rev Immunol 2:85, 2002.

55. Ruiz-Irastorza G, et al: Systemic lupus erythematosus. Lancet 357:1027, 2001.

56. Hahn BH: Antibodies to DNA. New Engl J Med 338:1359, 1998.

57. Keren DF: Antinuclear antibody testing. Clin Lab Med 22:447, 2002.

58. Galli M, et al: Antiphospholipid antibodies: predictive value of laboratory tests. Thromb Hemost 78:75, 1997.

59. Arnout J: Antiphospholipid syndrome: diagnostic aspects of lupus anticoagulants. Thromb Haemost 86:83, 2001.

60. Levine JS, Branch DW, Rauch J: The anti-phospholipid syndrome. New Engl J Med 346:752, 2002.

61. Gaffney PM, Moser KL, Graham RR, Behrens TW: Recent advances in the genetics of systemic lupus erythematosus. Rheum Dis Clin North Am 28:111, 2003.

62. Criswell LA, Amos CI: Update on genetic risk factors for systemic lupus erythematosus and rheumatoid arthritis. Curr Opin Rheumatol 12:85, 2000.

63. Bolto M, Walport MJ: C1q, autoimmunity and apoptosis. Immunobiology 205:395, 2002.

64. Rubin RL: Etiology and mechanisms of drug-induced lupus. Curr Opin Rheumatol 11:357, 1999.

65. White S, Rosen A: Apoptosis in systemic lupus erythematosus. Curr Opin Rheumatol 15:557, 2003.

66. Shlomchik MJ, Craft JE, Mamula MJ: From T to B and back again: positive feedback in systemic autoimmune disease. Nat Rev Immunol 1:147, 2001.

66A. Nakken B, et al: T-helper cell tolerance to ubiquitous nuclear antigens. Scand J Immunol 58:478, 2003.

67. Tsao BP: The genetics of human systemic lupus erythematosus. Trends Immunol 24:595, 2003.

68. Belmart HM, Abramson SB: Pathology and pathogenesis of vascular injury in SLE. Arthritis Rheum 39:9, 1996.

69. Cameron JS: Lupus nephritis. J Am Soc Nephrol 10:413, 1999.

70. Moore PM, Lisak RP: Systemic lupus erythematosus: immunopathogenesis of neurologic dysfunction. Springer Semin Immunopathol 17:43, 1995.

71. Moder KG, Miller TD, Tazelaar HD: Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc 74:275, 1999.

72. Iliopoulos AG, Toskos GC: Immunopathogenesis and spectrum of infection in SLE. Semin Arthritis Rheum 25:318, 1996.

73. Donnelly AM, et al: Discoid lupus erythematosus. Australas J Dermatol 36:3, 1995.

74. Patel P, Werth V: Cutaneous lupus erythematosus: a review. Dermatol Clin 20:373, 2002.

75. Fox RI, Stern M, Michelson P: Update in Sjögren syndrome. Curr Opin Rheumatol 12:391, 2000.

76. Jonsson R, Haga HJ, Gordon TP: Current concepts on diagnosis, autoantibodies and therapy in Sjögren's syndrome. Scand J Rheumatol 29:341, 2000.

77. Hang LM, Nakamura RM: Current concepts and advances in clinical laboratory testing for autoimmune diseases. Crit Rev Clin Lab Sci 34:275, 1997.

78. Gordon TP, et al: Autoantibodies in primary Sjögren's syndrome: new insights into mechanisms of autoantibody diversification and disease pathogenesis. Autoimmunity 34:123, 2001.

79. Sumida T, et al: TCR in Sjögren syndrome. Br J Rheumatol 36:622, 1997.

80. Haneji N, et al: Identification of a-fodrin as a candidate autoantigen in primary Sjögren syndrome. Science 276:604, 1997.

80A. Hansen A, Lipsky PE, Domer T: New concepts in the pathogenesis of Sjogren syndrome: many questions, fewer answers. Curr Opin Rheumatol 15:556, 2003.

81. James JA, Harley JB, Scofield RH: Role of viruses in systemic lupus erythematosus and Sjögren syndrome. Curr Opin Rheumatol 13:370, 2001.

82. Manthorpe R, et al: Primary Sjögren syndrome: diagnostic criteria, clinical features, and disease activity. J Rheumatol 24 (suppl 50):8, 1997.

83. Kahaleh MB, LeRoy EC: Autoimmunity and vascular involvement in systemic sclerosis (SSc). Autoimmunity 31:195, 1999.

84. Scaletti C, et al: Microchimerism and systemic sclerosis. Int Arch Allergy Immunol 125:196, 2001.

85. Jimenez SA, et al: Pathogenesis of scleroderma: collagen. Rheum Dis Clin North Am 22:647, 1996.

86. Tan FK, Arnett FC: Genetic factors in the etiology of systemic sclerosis and Raynaud phenomenon. Curr Opin Rheumatol 12:511, 2000.

87. Harvey GR, McHugh NJ: Serologic abnormalities in systemic sclerosis. Curr Opin Rheumatol 11:495, 1999.

88. Mitchell H, et al: Scleroderma and related conditions. Med Clin North Am 81:129, 1997.

89. Hoffman RW, Greidinger EL: Mixed connective tissue disease. Curr Opin Rheumatol 12:386, 2000.

90. Smolen JS, Steiner G: Mixed connective tissue disease: to be or not to be? Arthritis Rheum 41:768, 1998.

91. Sneller MC, Fauci AS: Pathogenesis of vasculitis syndromes. Med Clin North Am 81:221, 1997.

92. Group WHOS: Primary immunodeficiency diseases. Clin Exp Immunol 109 (suppl):1, 1997.

93. Buckley RH: Primary immunodeficiency diseases: dissectors of the immune system. Immunobiol Rev 185:206, 2002.

94. Ochs HD, Smith CID: X-linked agammaglobulinemia: a clinical and molecular analysis. Medicine 75:287, 1996.

95. Satterthwaite AB, Witte ON: The role of Bruton's tyrosine kinase in B-cell development and function: a genetic perspective. Immunol Rev 175:120, 2000.

96. Spickett GP, et al: Common variable immunodeficiency: how many diseases? Immunol Today 18:325, 1997.

97. Burrows PD, Cooper MD: IgA deficiency. Adv Immunol 65:245, 1997.

98. Ramesh N, et al: The hyper-IgM (HIM) syndrome. Springer Semin Immunopathol 19:383, 1998.

99. Durandy A, Honjo T: Human genetic defects in class-switch recombination (hyper-IgM syndromes). Curr Opin Immunol 13:543, 2001.

100. Epstein JA: Developing models of DiGeorge syndrome. Trends Genet 17:S13, 2001.

101. McDermid HE, Morrow BE: Genomic disorders on 22q11. Am J Hum Genet 70:1077, 2002.

102. Sugamura K, et al: The interleukin-2 receptor gamma chain: its role in the multiple cytokine receptor complexes and T cell development in XSCID. Annu Rev Immunol 14:179, 1996.

103. Leonard WJ: Cytokines and immunodeficiency diseases. Nat Rev Immunol 1:200, 2001.

104. Resta R, Thompson LF: SCID: the role of adenosine deaminase deficiency. Immunol Today 18:371, 1997.

105. Reith W, Mach B: The bare lymphocyte syndrome and the regulation of MHC expression. Annu Rev Immunol 19:331, 2001.

106. Huber J, et al: Pathology of congenital immunodeficiencies. Semin Diagn Pathol 9:31, 1992.

107. Fischer A, Hacein-Bey S, Cavazzana-Calvo M: Gene therapy of severe combined immunodeficiencies. Nat Rev Immunol 2:615, 2002.

108. Parkman R, et al: Gene therapy for adenosine deaminase deficiency. Annu Rev Med 51:33, 2000.

109. Snapper SB, Rosen FS: The Wiskott-Aldrich syndrome protein (WASP): roles in signaling and cytoskeletal organization. Annu Rev Immunol 17:905, 1999.

110. Snapper SB, Rosen FS: A family of WASPs. N Engl J Med 348:350, 2003.

111. Walport MJ: Complement. First of two parts. N Engl J Med 344:1058, 2001.

112. Walport MJ: Complement. Second of two parts. N Engl J Med 344:1140, 2001.

113. Frank MM: Complement deficiencies. Pediatr Clin North Am 47:1339, 2000.

114. Carugati A, et al: C1-inhibitor deficiency and angioedema. Mol Immunol 38:161, 2001.

115. Rosse WF: New insights into paroxysmal nocturnal hemoglobinuria. Curr Opin Hematol 8:61, 2001.

116. Royce RA, et al: Sexual transmission of HIV. N Engl J Med 336:1072, 1997.

117. Goodnough LT, Shander A, Brecher ME: Transfusion medicine: looking to the future. Lancet 361:161, 2003.

118. Mofenson LM, McIntyre JA: Advances and research directions in the prevention of mother-to-child HIV-1 transmission. Lancet 355:2237, 2000.

119. Cardo DM, et al: A case control study of HIV seroconversion in health care workers after percutaneous exposure. N Engl J Med 337:1485, 1997.

120. Frankel AD, Young JA: HIV-1: fifteen proteins and an RNA. Annu Rev Biochem 67:1, 1998.

121. Letvin NL, Walker BD: Immunopathogenesis and immunotherapy in AIDS virus infections. Nat Med 9:861, 2003.

122. Berger EA, Murphy PM, Farber JM: Chemokine receptors as HIV-1 coreceptors: roles in viral entry, tropism, and disease. Annu Rev Immunol 17:657, 1999.

123. Littman DR: Chemokine receptors: keys to AIDS pathogenesis? Cell 93:677, 1998.

124. LaBranche CC, et al: HIV fusion and its inhibition. Antiviral Res 50:95, 2001.

125. O'Brien SJ, Moore JP: The effect of genetic variation in chemokines and their receptors on HIV transmission and progression to AIDS. Immunol Rev 177:99, 2000.

126. Kinter A, et al: Chemokines, cytokines and HIV: a complex network of interactions that influence HIV pathogenesis. Immunol Rev 177:88, 2000.

127. Greene WC, Peterlin BM: Charting HIV's remarkable voyage through the cell: Basic science as a passport to future therapy. Nat Med 8:673, 2002.

128. Haase AT: Population biology of HIV-1 infection: viral and CD4+ T cell demographics and dynamics in lymphatic tissues. Annu Rev Immunol 17:625, 1999.

129. Hazenberg MD, et al: T cell depletion in HIV-1 infection: how CD4+ T cells go out of stock. Nat Immunol 1:285, 2000.

130. McCune JM: The dynamics of CD4+ T-cell depletion in HIV disease. Nature 410:974, 2001.

131. Grossman Z, et al: CD4+ T-cell depletion in HIV infection: are we closer to understanding the cause? Nature Medicine 8:319, 2002.

132. Wolthers KC, et al: T cell telomere length in HIV-1 infection: no evidence for increased CD4+ T cell turnover. Science 274:1543, 1996.

133. Gougeor M-L: Apoptosis as an HIV strategy to escape immune attack. Nat Rev Immunol 3:392, 2003.

134. Shearer GM: HIV-induced immunopathogenesis. Immunity 9:587, 1998.

135. Blankson JN, Persaud D, Siliciano RF: The challenge of viral reservoirs in HIV-1 infection. Annu Rev Med 53:557, 2002.

136. Steinman RM, et al: The interaction of immunodeficency viruses with dendritic cells. Curr Top Microbiol Immunol 276:1, 2003.

137. van Kooyk Y, Geijtenbeck TB: DC-SIGN: escape mechanisms for pathogens. Nat Rev Immunol 3:697, 2003.

138. Cohen OJ, et al: Studies on lymphoid tissue from HIV-infected individuals: implications for the design of therapeutic strategies. Springer Semin Immunopathol 18:305, 1997.

139. Power C, Johnson RT: Neuroimmune and neurovirological aspects of human immunodeficiency virus infection. Adv Virus Res 56:389, 2001.

140. Tardieu M, Boutet A: HIV-1 and the central nervous system. Curr Top Microbiol Immunol 265:183, 2002.

141. Stevenson M: HIV-1 pathogenesis. Nat Med 9:853, 2003.

142. Kahn JO, Walker BD: Acute human immunodeficiency virus type 1 infection. N Engl J Med 339:33, 1998.

143. McMichael AJ, Rowland-Jones SL: Cellular immune responses to HIV. Nature 410:980, 2001.

144. Gandhi RT, Walker BD: Immunologic control of HIV-1. Annu Rev Med 53:149, 2002.

145. Mellors JW, et al: Prognosis in HIV-1 infection predicted by the quantity of virus in plasma. Science 272:1167, 1996.

146. Piguet V, Trono D: Living in oblivion: HIV immune evasion. Semin Immunol 13:51, 2001.

147. Johnson WE, Desrosiers RC: Viral persistance: HIV's strategies of immune system evasion. Annu Rev Med 53:499, 2002.

148. Klenerman P, Wu Y, Phillips R: HIV: current opinion in escapology. Curr Opin Microbiol 5:408, 2002.

149. CDC: Centers for Disease Control and Prevention: 1993 revised classification system and expanded surveillance definition for AIDS among adolescents and adults. MMWR 41(RR-

17):1, 1992.

150. Furrer H, Fux C: Opportunistic infections: an update. J HIV Ther 7:2, 2002.

151. Gold JWM, et al: Management of the HIV-infected patient: Part II. Med Clin North Am 81:299, 1997.

152. Kovacs JA, Masur H: Prophylaxis against opportunistic infections in patients with human immunodeficiency virus infection. N Engl J Med 342:1416, 2000.

153. Barnes PF, Lakey DL, Burman WJ: Tuberculosis in patients with HIV infection. Infect Dis Clin North Am 16:107, 2002.

154. Boshoff C, Weiss R: AIDS-related malignancies. Nat Rev Cancer 2:373, 2002.

155. Scadden DT: AIDS-related malignancies. Annu Rev Med 54:285, 2003.

156. Judde JG, Lacoste V, Briere J, et al: Monoclonality or oligoclonality of human herpesvirus 8 terminal repeat sequences in Kaposi's sarcoma and other diseases. J Natl Cancer Inst

92:729, 2000.

157. Ensoli B, et al: Biology of Kaposi's sarcoma. Eur J Cancer 37:1251, 2001.

158. Moore PS, Chang Y: Molecular virology of Kaposi's sarcoma-associated herpesvirus. Philos Trans R Soc Lond B Biol Sci 356:499, 2001.

159. Boshoff C: Coupling herpesvirus to angiogenesis. Nature 391:24, 1998.

160. Knowles DM, Pirog EC: Pathology of AIDS-related lymphomas and other AIDS-defining neoplasms. Eur J Cancer 37:1236, 2001.

161. Carbone A: Emerging pathways in the development of AIDS-related lymphomas. Lancet Oncology 4:22, 2003.

162. Shah KV: Human papillomavirus and anogenital cancers. N Engl J Med 337:1386, 1997.

163. Knowles DM: Immunodeficiency-associated lymphoproliferative disorders. Mod Pathol 12:200, 1999.

164. McMichael AJ, Hauke T: HIV vaccines 1983–2003. Nat Med 9:874, 2003.

165. Robinson HL: New hope for an AIDS vaccine. Nat Rev Immunol 2:239, 2002.

166. Pepys MB: Pathogenesis, diagnosis and treatment of systemic amyloidosis. Philos Trans R Soc Lond B Biol Sci 356:203, 2001.

167. Merlini G, Bellotti V: Molecular mechanisms of amyloidosis. New Engl J Med 349:583, 2003.

168. Plante-Bordeneuve V, Said G: Transthyretin related familial amyloid polyneuropathy. Curr Opin Neurol 13:569, 2000.

169. DeArmond SJ: Cerebral amyloidosis in prion diseases. Int J Exp Clin Invest 7:3, 2000.

170. Falk RH, Comenzo RL, Skinner M: The systemic amyloidoses. N Engl J Med 337:898, 1997.

171. Harrison CJ, et al: Translocations of 14q32 and deletions of 13q14 are common chromosomal abnormalities in systemic amyloidosis. Br J Haematol 117:427, 2002.

172. Drenth JP, van der Meer JW: Hereditary periodic fever. N Engl J Med 345:1748, 2001.

173. Touitou I: The spectrum of Familial Mediterranean Fever (FMF) mutations. Eur J Hum Genet 9:473, 2001.

174. Cornwell GG, et al: The age related amyloids: a growing family of unique biochemical substances. J Clin Pathol 48:984, 1995.

175. Dobson CM: Protein folding and its links with human disease. Biochem Soc Symp:1, 2001.

176. Guy CD, Jones CC: Abdominal fat pad aspiration biopsy for tissue confirmation of systemic amyloidosis: specificity, positive predictive value, and diagnostic pitfalls. Diagn

Cytopathology 24:181, 2001.

177. Gilmore JD, et al: Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of serum amyloid A protein. Lancet 358:24, 2001.

Date: 2016-04-22; view: 1181