#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Waldenström macroglobulinemia – clinical manifestations and differential diagnosis and prognosis of the disease


Authors: Z. Adam 1;  J. Šmardová 2;  V. Ščudla 3
Authors‘ workplace: Interní hematoonkologické klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc. 1;  Ústav patologie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jirka Mačák, CSc. 2;  III. interní klinika Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vlastimil Ščudla, CSc. 3
Published in: Vnitř Lék 2007; 53(12): 1325-1337
Category: Review

Overview

Waldenström macroglobulinemia is defined by the presence of IgM type monoclonal immunoglobulin and histological prove of lymphoplasmocytary lymphoma in the bone marrow. Clinical symptoms of the disease depend on the pressure on the bone marrow by the malignant clone with subsequent cytopenia, extramedullary infiltration and toxic manifestations of monoclonal immunoglobulin. 6q deletion and absence of translocation in the sphere of the heavy immunoglobin chain gene, which is otherwise typical for other lymphoproliferative disorders, is the typical cytogenetic abnormality. The text describes the symptoms of the disease and the issues of its differential diagnosis.

Keywords:
Waldenström macroglobulinemia – IgM type multiple myeloma


Sources

1. Waldenström J. Incipient myelomatosis or essential hyperglobulinemia with fibrinogenopenia. A new syndrome? Acta Med Scand 1944; 117: 217-247.

2. Desikan KR, Li Z, Jaganath S. Waldenströms makroglobulinemia: current therapy and future approaches. Bio Drugs 2002; 16: 201-207.

3. Johnson SA, Birchall J, Luckie C et al. Guidelines on management of Waldenström’s macroglobulinemia. Brit J Haematol 2006; 132: 687-697.

4. Johnson SA. Advances in the treatment of Waldenström’smacroglobulinemia. Exp Rev Anticancer Treatment 2006; 6: 329-334.

5. Klán J, Doležalová I, Pelíšková D et al. Waldenströmova makroglobulinémie jako model imunopatologie ve stáří. Geriatria 2004; 10: 18-27.

6. Treon SP, Hunter ZR, Aggarwal A et al. Characterization of familiar Waldenström’smacroglobulinemia. Ann Oncol 2006; 17: 488-494.

7. Tichý M, Urban P, Matěja P et al. Laboratorní analýza souboru 3 049 monoklonálních imunoglobulinů. Klin Biochem Metabol 2002; 10: 257-261.

8. Tichý M. Viscosity of paraproteinemic séra. Acta Med 1996; 39: 41-43.

9. Tichý M. Laboratorní analýza monoklonálních imunoglobulinů (paraproteinů). Český Těšín: Finidr 1997.

10. Rencová E, Malý J, Bláha M et al. Dynamika sítnicových změn v závislosti na léčbě Waldenströmovy choroby. Čs Oftal 1993; 49: 3-7.

11. Stalnikiewicz L, Carrote-Lefebvre I, Detourmignies L et al. Prognostic factors in Waldenström’smacroglobulinemia. Description of the complication during the evolution - preliminary results on 101 patients. Semin Oncol 2003; 30: 216-219.

12. Shihabi ZK Cryoglobulins: An important but neclected clinical test. Ann Clin Lab Science 2006; 36: 395-408.

13. Tichý M, Hrnčíř Z, Urban P et al. Monoklonální imunoglobuliny. Klin Biochem Metabol 2004; 12: 84-87.

14. Tichý M. Monoklonální gamapatie. Labor Aktuel CS 2000; 7-10.

15. Daoud MS, Lust JA, Kyle RA. Monoclonal gammapaties and associated skin disorders. J Amer Acad Dermatol 1999; 40: 507-535.

16. Dimopoulos MA, Kyle RA, Anagnastopoulos A et al. Diagnosis and management of Wadenström’s macroglobulinemia. J Clin Oncol 2005; 23: 1564-1577.

17. Bryce AH, Kyle RA, Dispenzieri A et al. Natural history of therapy of 66 patients with mixed cryoglobulinemia. Amer J Hematol 2006; 81: 511-518.

18. Kyle RA, Gahrton JP. The spektrum of IgM monoclonal gammopathy in 430 cases. Mayo Clin Proc 1987; 62: 719-731.

19. Stone MJ, Merlini G, Pascal V. Autoantibody activity in Waldenströms macroglobulinaemia. Clin Lymphoma 2005; 5: 225-229.

20. Bonnetblanc JM, Bedane CH, Fazol J et al. Tumoral macroglobulinosis of the skin in situ release of IgM. Acta Dermatovenereol 2006; 86: 63-64.

21. Dimopoulos MA, Panayiotis P, Moulapoulos LA et al. Waldenström’s makroglobulinemia: Clinical features, complication and management. J Clin Oncol 2000; 18: 214-226.

22. Gertz MA, Kyle RA. Amyloidosis with IgM gammapathies. Semin Oncol 2003; 30: 325-328.

23. Gertz MA. Natural history and therapy of 66 patients with mixed cryoglobulinemia. Amer J Hematol 2006; 81: 511-518.

24. Tichý M. Primární amyloidóza. Lék zpr Lék Fak Univ Karlovy Hr Králové 1999; 44: 99-107.

25. Schimonová M, Zatloukal P, Havlíček F et al. Amyloidóza plic při Waldenstromövě chorobě. Stud Pneumol Phtiseol 1998; 58: 72-74.

26. Okuda M, Okuda Y, Obuta T et al. Primary lung involvement with amyloid deposition in Waldenström macroglobulinaemia. Respirology 2004; 9: 414-418.

27. Gardyn J, Schwartz A, Gal R et al. Waldenström’s macroglobulinaemia associated with AA amyloidosis. Int J Hematol 2001; 74: 76-78.

28. Gisseroth O, Landais C, Cremades S et al. Amyloid arthropathy and Waldenström’s macroglobulinemia. Joint Bone Spine 2006; 73: 456-458.

29. Sanchorawala V, Blanchard E, Seldin DC et al. AL-amyloidosis associated with B cell lymphoproliferative disorders: frequency and treatment outcomes Amer J Hematol 2006; 81: 692-695.

30. Cesana C, Barbarami L, Miqueleiz S et al. Clinical characteristics and outcome of imunoglobulin-M related disorders. Clin Lymphoma 2005; 5: 261-264.

31. Levine T, Pestron A, Florence J et al. Peripheral neuropaties in Waldenström’s macroglobulinemia. J Neurol Neurosurg Psychiatry 2006; 77: 224-228.

32. Pestronk A. Peripheral neuropathy in Waldenström’s macroglobulinemia. J Neurol Neurosurg Psychiatr 2006; 77: 224-228.

33. Simovic D, Gorson, KC, Ropper AH. Comparison of IgM-MGUS and IgG MGUS polyneuropathy. Acta Neurol Scand 1998; 97: 194-200.

34. Garcia-Pacheco H, Khan A, Venka KK Rapidly progressive glomerulonefritis in a patients with Waldenström’smacroglobulinemia. Clin Nephrol 2005; 64: 396-399.

35. Bridoux F, Sirac Ch, Hugue V et al. Fanconi syndrome induced by monoclonal Vkappa3 light chain in Waldenström’s macroglobulinemia. Amer J Kidny disease 2005; 45: 749-757.

36. Yokote T, Akioka T, Oka S et al. Cutaneous infiltration with Waldenström macroglobulinemia. Leukemia Res 2006; 30: 1207-1210.

37. Autier J, Buffet M, Pinquier L et al. Cutaneeous Waldenströms macroglobulinaemia with deck chair sign treated with cyclophosphamide. J Am Acad Dermatol 2005; 52: 45-47.

38. Cerna M, Risik K, Jakubikova N. Lymfolazmocytoidní imunocytom - Waldenströmova choroba s postihnutím pluc. Stud Pneumol Phtiseol CS 1989; 49: 198-202.

39. Pozdechova A, Virsik K, Tichá M et al. Intrathorakalne prejavy Waldenströmovej makroglobulinémie. Stud Pneumol Phtiseol CS 1980; 40: 599-604.

40. Kyrtsonis MC, Angelopoulou MK, Kontopidou FN et al. Primary lung involvement in Waldenström’s macroglobulinemia. Acta Hametol 2001; 105: 92-96.

41. Kyrtsonis MC, Vassilakopoulos TP, Angelopoulou MK et al. Waldenstöm’s macroglobulinemia: clinical course and prognostic factors in 60 patients. Ann Hematol 2001; 80: 722-727.

42. Malý J, Tichý M, Blaha M et al. A case of acute Waldenström macroglobulinemia. Sbor Ved Praci Lék Fak Univ Karlovy Hrad Kr 1983; 26: 165-173.

43. Civit T, Coulbouis S, Baylac F et al. Waldenström macroglobulinaemia and cerebral lymphoplamocytic proliferation. Neurochirurgie 1997; 43: 245-249.

44. Jondeau K, Alterescu R, Franc B et al. Unusual evolution of Waldenström’s macroglobulinemia into ostolytic myeloma. Eur J. Haematol 2006; 77: 74-79.

45. Lin P, Buesco R, Wilson C et al. Wandenström macroglobulinaemia involving extramedulary sites. Amer J Surg Patol 2003; 27: 1104-1113.

46. Lin P, Hao S, Handy BC et al. Lymphoid neoplasms associated with IgM paraprotein. Am J Clin Pathol 2005; 123: 200-205.

47. Slavin ChM, Seymour J Progressive multifocal leucoencefalopahty complicating Waldenström’s macroglobulinemia. Leukemia Lymphoma 2003; 44: 1819-1821.

48. Facon T, Brouillard M, Duhamel A et al. Prognostic factors in Waldenström’s macroglobulinaemia: a report of 167 cases. J Clin Oncol 1993; 11: 1553-1558.

49. Gobi PG, Bettini R, Montecucco C et al. Study of prognosis in Waldenström’s macroglobulinaemia. Blood 1994; 83: 1452-1459.

50. Morel P, Monconduit M, Jacomy D et al. Prognostic factors in Waldenström’s macroglobulinaemia. A report on 232 patients with the description of a new scoring system and its validation on 253 other patients. Blood 2000; 96: 852-858.

51. Morel, P, Duhamel A, Gobbi P et al. International Prognostic Scoring system for Waldenström’s Macroglobulinemia. Blood 2006; 108: 42a.

52. Owen RG, Barrans SL, Richards SJ et al. Waldenström’s macroglobulinemia: Developement of diagnostic criteria and identification of prognostic factors. Am J Clin Pathol 2001; 116: 420-428.

53. Owen RG, Parapia LA, Higginson J et al: Clinicopathological correlates of IgM paraproteinemias. Clin Lymphoma 2000; 1: 39-43.

54. Owen RG, Treon SP, Al-Katib A et al. Clinicopathological definition of Waldenström’s macroglobulinaemia. Consensus panel recommendation from the Second International Workshop on Waldenström’smacroglobulinemia. Semin Oncol 2003; 30: 567-584.

55. Owen RG, Treon SP, AL-Katib A et al. Clinicopathological definition of Waldenström’s macroglobulinemia. Semin Oncol 2003; 30: 110-115.

56. Garcia-Sanz R, Montoso S, Torrequebrada A et al. Waldenström’s macroglobulinemia. Presenting features and outcome in a series of 217 patients. Br J Haematol 2001; 115: 575-582.

57. Rajkumar SV Monoclonal gammopathy of unknown significance, Waldenström’s macroglobulinaemia, AL-amyloidosis and related plasma cell disorders. Diagnosis and treatment. Mayo Clin Proc 2006; 81: 893-703.

58. Remstein ED, Hanson CA, Kyle RA et al. Despite apparent morphologic and immunophenotypic heterogenity Waldenoström´s macroglobuliniemia is consistently composed of cell showing morpholic continuum of small lymphocytes, plasmocytoid lymphocytes and plasma cells. Semin Oncol 2003; 30: 182-186.

59. Rajkumar V, Haman S, Greip FR. Angiogenesis in Waldenström’s macroglobulinaemia. Semin Oncol 2003; 30: 262-264.

60. Konoplev S, Madeiro J, Bueso-Ramos CE et al. Immunophenotypic profile of lymfoplasmocytic lymphoma/Waldenström’s macroglobulinaemia. Am J Clin Pathol 2005; 124: 414-420.

61. Hunter ZR, Branagan AR, Mannin R et al. CD5, CD10 and CD23 expression in Waldenström’smacroglobulinaemia. Clin Lymphoma 2004; 5: 246-249.

62. San Miguel JF, Vidriales MB, Ocio E et al. Immunopfenotypic analysis of Waldenström’s macroglobulinaemia. Semin Oncol 2003; 30: 187-195.

63. Sahota SS, Forconi F, Ottensmeier CH et al. Origins of the malignant clone in typical Wandenström´s macroglobulinaemia. Semin Oncol 2003; 30: 136-141.

64. Kriangkum J, Taylor BJ, Strachan E et al. Impaired class switch recombination in Waldenström’smacroglobulinemia despite apparently norme CSR machinery. Blood 2006; 107: 2920-2927.

65. Kriangkum J, Tailor BJ, Reimann T et al. Origins of Waldenström’s macroglobulinemia: does it arise from a unusual B cell precursor? Clinical Lymphoma 2005; 5: 217-219.

66. Rollett RA, Wilkinson EJ, Gonzalez D et al. Immunoglobulin Haevy Chain sequence analysis in Waldenström’s macroglobulinemia and immunoglobulin M monoclonal gammapathy of undetermined significance. Clin Lymphoma Myeloma 2006; 7: 70-72.

67. Calasanz MJ, Cigudosa JC, Odero MD et al. Cytogenetic analysis of 280 patients with multiple myeloma and releated disordes. Genes chromosomes Cancer 1997; 18: 84-93.

68. Louviaux I, Michaux L, Hagemeier A et al. Cytotenetic abnormalities in Waldenström’sisease. Blood 1998; 92 (Suppl 1): Abstr. 3776.

69. Mansoor A, Medeiros LHJ, Weber DM et al. Cytogenetic findings in lymfoplasmocytic lymphoma. Chromosomal abnormalities are associated with polymorphous subtype and an aggressive clinical course. Amer J Clinic Pathol 2001; 116: 543-549.

70. Schop RFJ, Jalal SM, Van Wier SA et al. Deletions of 17p13.1 and 13q14 are uncomon in Waldenström macroglobulinemia clonal cells and mostly seen at the time of disease progression. Cancer Genetics Cytogenetics 2002; 132: 55-60.

71. Schop RFJ, Krehl WM, Van Wier SA et al. Waldenström’s macroglobulinemia: neoplastic cells lack IgH translocation but have frequent 6q deletions. Blood 2002; 100: 2996-3001.

72. Schop RFJ, Van Wier SA, Xu R et al. 6q deletion discriminates Waldenström’s macroglobulinemia from IgM monoclonal gammapathy of undetermined significance. Cancer Genetics Cytogenetics 2006; 169: 150-153.

73. Treon SR, Morton C, Braverman E et al. Deletion in 6q21-22 are commonly present in patients with familiar and non-familiar Waldenström’s macroglobulinemia. And IgM monoclonal gammapathy of unknown significance. Blood 2003; 102: Abstr. 2526.

74. Shaw GR, Kronberg DL. P53 deletion but not trisomy 12 are adverse in B cell lymphoproliferative disorders. Cancer Genetics Cytogenetics 2000; 119: 146-154.

75. Chang H, Saymour S, Li D et al. Analysis of IgH translocation chromosome 13q14 and 17p13.1 (pp3) deletions by fluorescent in situ hybridization in Waldenström’s makroglobulinemia. Leukemie 2004; 18: 1160-1162.

76. Chang WJ, Schop RF, Price-Troska T et al. Gene expression profiling of Waldenström’smacrogobulinemia reveals phenotype more similar to chronic lymphocytic leukemia then multiple myeloma. Blood 2006; 108: 2755-2763

77. Wong KF. Waldenström’s macroglobulinaemia with karyotypic aberrations involving both homologous 6q. Cancer Genet Cytogenet 2001; 124: 137-139

78. Ocio, EM, Hernánez JH, Mateo G et al. Immunophenotypic and cytogenetic comparison of Waldenström’s macroglobulinemia and splenic marginal zone lymphoma. Clin Lymphoma 2005; 5: 241-245.

79. Avet-Loiseau H, Garand R, Lodé L et al. 14g32 translocations discriminate IgM multiple myelom from Waldenström’s macroglobulinemia. Semin Hematom 2003; 30: 153-155.

80. Johnson SA. Waldenström macroglobulinaemia. Rev Clin Exp Hematol 2002; 6: 421-334.

81. Avet-Loiseau H, Facon T, Grosbois B et al. Oncogenesis in multiple myeloma: 14q32 and 13q chromosom abnormalities are non randomly distributed but correlate with natural history immunological features and clinical presentation. Blood 2002; 99: 2185-2191.

82. Konduri K, Sahota SS, Babbage G et al. Imunoglobulin M myeloma Evaluation of molecular features and cytokine expression. Clin Lymphoma 2005; 5: 285-289.

83. Pilarski L. Impaired recombination in Waldenström’s macroglobulinemia. Blood 2006; 107: 2920-2927

84. Ocio EM, Schop RFJ, Gonzales B et al. 6q deletion in Waldenström macroglobulinaemia is associated with features of adverse prognosis. Brit J Haematol 2006; 136: 80-86.

85. Owen RG. Immunoglobulin heavy chain sequences analysis in Waldenström’smacroglobulinemia and imunoglobulin M monoclonal gammopathy of undetermined significance. Clin Lymphoma Myeloma 2006; 7: 70-72.

86. Ackroyd S, O’Connor SJM, Owen RG Rarity of IgH translocations in Waldenström’smacroglobulinaemia. Cancer Genet Cytogenet 2005; 163: 77-80.

87. Cook JR, Aguilera NI, Reshmi S et al. Deletion of 6q is not a characteristic marker of nodal lymphoplasmocytic lymphoma. Cancer Genet Cytogenet 2005; 162: 85-88.

88. Ščudla V. Význam sérových hladin volných lehkých řetězců imunoglobulinů v diagnostice a hodnocení aktivity mnohočetného myelomu. Vnitř Lék 2005; 51: 1249-1259.

89. Tichý M, Friedecký B, Vávrová J et al. Standardizace biochemických laboratorních vyšetření u mnohočetného myelomu. Klin Biochem Metabol 2006; 14: 8-13.

90. Tichý M, Gregor J, Holečková J et al. C-reaktivní protein. Labor Aktuell CS 2002; 8-12.

91. Tichý M, Hrnčír M, Mráček J. Simultaneous occurrence of IgM lambda wit pyroprecipitation properties and cryoglobulin in the serum of patient with Waldenström’smacroglobulinemia. Neoplasma 1978; 25: 741-744.

92. Tichý M, Stulík M, Kovářová H et al. Použití dvojrozměrné chromatografie k analýzy monoklonálních lehkých řetězců imunoglobulinů v moči. Voj Zdrav Listy 1994; 63: 79-83.

93. Tichý M. Paraproteinémie nejasného původu. Klin Biochem Metabol 1998; 6: 172-175.

94. Tichý M. Stanovení monoklonálních imunoglobulinů a jejich fragmentů u monoklonálních gamapatií. Vnitř Lék 2005; 51: 1225-1227.

95. Sakalová A, Mistrík M, Škultétyová D et al. Waldenströmova makroglobulinémie, aký je súčasný stav? Hemat Transfuz 1999; 9: 56-60.

96. Kyle RA, Rajkumar S, Therneau TM et al. Prognostic factors and prediction of outcome of immunoglobulin M monoclonal gammopathy of undetermined significance. Clin Lymphoma 2005; 5: 257-260.

97. Kyle RA, Rajkumar SV. Monoclonal Gammapaties of undertermined significance. Hematol Oncol Clin North Amer 1999; 13: 1181-1201.

98. Kyle RA, Treon SP, Alexanian R et al. Prognostic markers and criteria to initiate therapy in Waldenström’smacroglobulinemia. Consensus panel recommendation from the second international Workshop on Waldenström’smacroglobulinemia. Semin Oncol 2003; 30: 116-120.

99. Klán J, Topinková E, Pelíšková D et al. Waldenströmova makroglobulinémie v geriatrické praxi. Čes Geriat Rev 2004; 2: 40-44.

100. Ghobrial IR, Fonseca R, Gertz MA et al. Prognostic model for disease specific and overall mortality newly diagnosed symptomatic patients with Waldenström’smacroglobulinaemia. Brit J Haematol 2006; 133: 158-1654.

101. Alexanian R, Weber D, Delasalle F et al. Asymptomatic Waldenström’s macroglobulinemia. Semin Oncol 2003; 30: 206-210.

102. Cesana C, Miquelei S, Bernuzzi P et al. Smouldering Waldenstrom macroglobulinaemia: Factors predicting evolution to symptomatic disease. Semin Oncol 2003; 30: 206-210.

103. Morra E, Cesana C, Klersy C et al. Predictive variables for malignant transformation on 452 patients with asymptomatic IgM monoclonal gammopathy. Semin Oncol 2003; 30: 172-177.

104. Morra E, Cesana C, Klersy C et al. Prognostic factors for transformation in asyptomatic imunoglobulin IgM monoclonal gammapathies Clinical Lymphoma 2005; 5: 256-269.

105. Baldini L, Goldaniga M, Guffanti A et al. Imunoglobulin M monoclonal gammapathy of undetermined significance and indolent Waldenström’s macroglobulinaemia recognize the same determinants of evolution into symptomatic lymphoid disorders: A proposal for a commmon prognostic scoring system. J Clin Oncol 2005; 23: 4662-4668.

106. Baldini L, Goldaniga M, Guffanti A et al. Imunoglobulin M monoclonal gammapathy of undetermined significance and indolent Waldenström’s macroglobulinaemia recognize the same determinants of evolution into symptomatic lymphoid disorders: A proposal for a commmon prognostic scoring system. J Clin Oncol 2005; 23: 4662-4668.

107. Dhodapkar MV, Jacobson JL, Gertz MA et al. Prognostic factors and response to fludarabine therapy in Waldenström’s macroglobulinemia: an update of a US Intergroup trial. Semin Oncol 2003; 30: 220-225.

108. Dimopoulos MA, Gika D, Zervas K et al. The international staging system for multiple myeloma is applicable in symptomatic Waldenström’s macroglobulinaemia. Leukemia Lymphoma 2004; 45:1809-1813.

109. Merlini G, Baldini L, Broglia C et al. Prognostic factors in symptomatic Waldenström’s macroglobulinaemia. Semin Oncol 2003; 30: 211-215.

110. Nagan S, Rohatiner AZS, Matthews J et al. Waldenström’s macoglobulinaemia: A restrospective analysis of 40 patients from 1972-2001. Semin Oncol 2003; 30: 236-238.

111. Pangalis GA, Kyrtsonis MCh, Kontopidu FN et al. Differential diagnosis of Waldenström’s macroglobulinemia and other B cell disorders. Clin Lymphoma 2005; 5: 235-240.

112. Schnitzler L, Hurez D, Verret JL et al. Chronic urtica and hyperostosis in macroglobulinemia. Ann Dermatol Venereol 1989; 116: 547-550.

113. Tichý M. Turbidimetrické stanovení lehkých řetězců imunoglobulinů. Labor Aktuel CS 1998; 6-10.

Labels
Diabetology Endocrinology Internal medicine

Article was published in

Internal Medicine

Issue 12

2007 Issue 12

Most read in this issue
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#