Diffuse Intestinal Involvement by Waldenström Macroglobulinemia: Clinical Case and Literature Review
DOI:
https://doi.org/10.24950/rspmi/CC/254/2/2018Keywords:
Immunoglobulin M, Intestinal Diseases, Malabsorption Syndromes, ; Waldenström MacroglobulinemiaAbstract
Gastrointestinal involvement in Waldenström macroglobulinemia is an extremely rare lymphoproliferative disorder. We describe a Waldenström macroglobulinemia patient presenting with diarrhea, dyspepsia, weight loss and malabsorption. Endoscopic studies revealed diffuse deposition of immunoglobulin M and kappa light chains in the mucosa of the small intestine. Later, on evaluation in the Haemato-Oncology clinic, the risk of death at 5 years was considered low, and thus no further therapeutic measures were proposed. The patient remained stable for three years, when she developed septic shock with rapidly progressive multiple organ failure and died a few hours after admission to hospital. The authors describe the case and elaborate a review of the literature on this pathology. We draw attention to this rare manifestation of an uncommon lymphoproliferative disorder to be considered in the differential diagnosis in a patient with a malabsorption syndrome.
Downloads
References
Pratz K, Dingli D, Smyrk T, Lust JA. Intestinal Lymphangiectasia with protein-Losing enteropathy in Waldenstrom Macroglobulinemia. Medicine. 2007;86:210-4.
Vijay A, Gertz M. Waldenstrom macroglobulinemia. Blood. 2007; 109:5096-103.
Gertz MA. Waldenstrom macroglobulinemia. Hematology. 2012;17 Suppl 1:S112-6.
Veloso F, Fraga J, Saleiro J. Macroglobulinemia and small intestinal disease. J Clin Gastrenterol. 1998; 10: 546-50.
Fonseca R, Hayman S. Waldenström macroglobulinaemia. Br J Haematol. 2007; 138:700-20.
García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A, et al. Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases. Br J Haematol. 2001; 115:575-82.
Benjamin M, Reddy S, Brawley OW. Myeloma and race: a review of the literature. Cancer Metastasis Rev. 2003; 22:87.
Royer RH, Koshiol J, Giambarresi TR, Vasquez LG, Pfeiffer RM, McMaster ML. Differential characteristics of Waldenström macroglobulinemia according to patterns of familial aggregation. Blood. 2010; 115:4464-71.
Linet MS, Humphrey RL, Mehl ES, Brown LM, Pottern LM, Bias WB, et al. A case-control and family study of Waldenstrom´s macroglobulinemia. Leukemia. 1993; 7:1363-9.
Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I, et al. Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. J Natl Cancer Inst. 2010; 102:557-67.
Aoki H, Takishita M, Kosaka M, Saito S. Frequent somatic mutations in D and/or JH segments of Ig gene in Waldenströms macroglobulinemia and chronic lymphocytic leukemia (CLL) with Richter´s syndrome but not in common CLL. Blood. 1995; 85:1913.
Ciric B, VanKeulen V, Rodriguez M, Kyle RA, Gertz MA, Pease LR. Clonal evolution in Waldenstrom macroglobulinemia highlights functional role of B-cell receptor. Blood. 2001; 97:321-3.
Schop RF, Kuehl WM, Van Wier SA, , Ahmann GJ, Price-Troska T, Bailey RJ, et al. Waldenström macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions. Blood. 2002; 100:2996-3001.
Kriangkum J, Taylor BJ, Treon SP, Mant MJ, Belch AR, Pilarski LM. Clonotypic IgM V/D/J sequence analysis in Waldenstrom macroglobulinemia suggests an unusual B-cell origin and an expansion of polyclonal B cells in peripheral blood. Blood. 2004; 104:2134-42.
Stone MJ, Merlini G, Pascual V. Autoantibody activity in Waldenstrom´s macroglobulinemia. Clin Lymphoma. 2005; 5: 225-9.
Dimopoulos MA, Panayiotidis P, Moulopoulos LA, et al. Waldenström´s macroglobulinemia: clinical features, complications, and management. J Clin Oncol. 2000; 18:214-26.
Fadil A, Taylor DE. The Lung and Waldenstrom´s Macroglobulinemia. South Med J. 1998;91: 681-5.
Veltman GA, van Veen S, Kluin-Nelemans JC. Renal disease in Waldenström´s macroglobulinaemia. Nephrol Dial Transplant. 1997; 12:1256 59.
Lindström FD, Hed J, Eneström S. Renal pathology of Waldenström´s macroglobulinaemia with monoclonal antiglomerular antibodies and nephrotic syndrome. Clin Exp Immunol. 1980; 41:196.
Civit T, Coulbois S, Baylac F, Taillandier L, Auque J. Macroglobulinemie de Waldenstrom et proliferation lymphoplasmocytaire cerebrale: le syndrome de Bing et Neel. A propos d’une nouvelle observation. Neurochirurgie. 1997; 43:245-9
Nobile-Orazio E, Marmiroli P, Baldini L, Spagnol G, Barbieri S, Moggio M, et al. Peripheral neuropathy in macroglobulinemia: incidence and antigen-specificity of M proteins. Neurology 1987; 37:1506-14.
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 16-1999. A 71-year-old man with progressive weakness and a gammopathy. N Engl J Med. 1999; 340:1661-9.
Rudnicki SA, Harik SI, Dhodapkar M, Barlogie B, Eidelberg D. Nervous system dysfunction in Waldenström´s macroglobulinemia: response to treatment. Neurology. 1998; 51:1210-3.
Baehring JM, Hochberg EP, Raje N, Ulrickson M, Hochberg FH. Neurological manifestations of Waldenström macroglobulinemia. Nat Clin Pract Neurol. 2008; 4:547-56.
Cobb MW, Domloge-Hultsch N, Frame JN, Yancey KB. Waldenström macroglobulinemia with an IgM-kappa antiepidermal basement membrane zone antibody. Arch Dermatol. 1992; 128:372.
Lowe L, Fitzpatrick JE, Huff JC, Shanley PF, Golitz LE. Cutaneous macroglobulinosis. A case report with unique ultrastructural findings. Arch Dermatol. 1992; 128:377-80.
Gressier L, Hotz C, Lelièvre JD, Carlotti A, Buffet M, Wolkenstein P, et al. Cutaneous macroglobulinosis: a report of 2 cases. Arch Dermatol. 2010; 146:65-9.
Gertz MA, Kyle RA. Hyperviscosity syndrome. J Intensive Care Med 1995; 10:128-9.
Kwann HC, Bongu A. The hiperviscosity syndromes. Semin Thromb Hemost; 1999;25:199-208.
Michael AB, Lawes M, Kamalarajan M. Cryoglobulinaemia as an acute presentation of Waldenstrom´s macroglobulinaemia. Br J Haematol. 2004; 124:565.
Terrier B, Jaccard A, Harousseau JL, Delarue R, Tournilhac O, Hunault-Berger M, et al. The clinical spectrum of IgM-related amyloidosis: a French nationwide retrospective study of 72 patients. Medicine. 2008; 87:99-109.
Owen RG, Treon SP, Al-Katib A. Clinicopathological definition of Waldenstrom´s macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom´s Macroglobulinemia. Semin Oncol. 2003; 30:110-5.
Swerdlow SH, Campo E, Harris NL, editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2008.
Kyle RA, Treon SP, Alexanian R. Prognostic markers and criteria to initiate therapy in Waldenstrom´s macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstroms Macroglobulinemia. Semin Oncol. 2003; 30:116.
Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P, et al. Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance? Clin Cancer Res. 2005; 11:1786-90.
Dhodapkar MV, Hoering A, Gertz MA, Rivkin S, Szymonifka J, Crowley J, et al. Long-term survival in Waldenstrom macroglobulinemia: 10-year follow-up of Southwest Oncology Group-directed intergroup trial S9003. Blood. 2009; 113:793-6.
Ansell SM, Kyle RA, Reeder CB, Fonseca R, Mikhael JR, Morice WG, et al. Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines. Mayo Clin Proc. 2010; 85:824-33.
Johnson SA, Birchall J, Luckie C, Oscier DG, Owen RG; Haemato-Oncology Task Force of the British Committee for Standards in Haematology. Guidelines on the management of Waldenström macroglobulinaemia. Br J Haematol 2006; 132:683-97.
Gertz MA. Waldenström macroglobulinemia: a review of therapy. Am J Hematol. 2005; 79:147.
Treon SP, Gertz MA, Dimopoulos M. Update on treatment recommendations from the Third International Workshop on Waldenstrom´s macroglobulinemia. Blood. 2006; 107:3442.
Szczepiorkowski ZM, Winters JL, Bandarenko N. Guidelines on the use of therapeutic apheresis in clinical practice--evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher. 2010; 25:83.
Dimopoulos MA, Gertz MA, Kastritis E. Update on treatment recommendations from the Fourth International Workshop on Waldenstrom´s Macroglobulinemia. J Clin Oncol. 2009; 27:120.
Dimopoulos MA, Alexanian R, Gika D, Tournilhac O, Leblond V, Morel P. Treatment of Waldenstrom´s macroglobulinemia with rituximab: prognostic factors for response and progression. Leuk Lymphoma 2004; 45:2057-8.
Gertz MA, Rue M, Blood E, Kaminer LS, Vesole DH, Greipp PR. Multicenter phase 2 trial of rituximab for Waldenström macroglobulinemia (WM): an Eastern Cooperative Oncology Group Study (E3A98). Leuk Lymphoma 2004; 45:2047-55.
Treon SP, Branagan AR, Hunter Z, Santos D, Tournhilac O, Anderson KC. Paradoxical increases in serum IgM and viscosity levels following rituximab in Waldenstrom´s macroglobulinemia. Ann Oncol. 2004; 15:1481-3.
Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, Zervas K, Tsatalas C, Kokkinis G, et al. Primary treatment of Waldenström macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol. 2007; 25:3344-9.
Treon SP, Ioakimidis L, Soumerai JD, Patterson CJ, Sheehy P, Nelson M, et al. Primary therapy of Waldenström macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05-180. J Clin Oncol. 2009; 27:3830-5.
Ghobrial IM, Xie W, Padmanabhan S, Badros A, Rourke M, Leduc R, et al. Phase II trial of weekly bortezomib in combination with rituximab in untrea-ted patients with Waldenström macroglobulinemia. Am J Hematol. 2010; 85:670-4.
Agathocleous A, Rohatiner A, Rule S, Hunter H, Kerr JP, Neeson SM, et al. Weekly versus twice weekly bortezomib given in conjunction with rituximab, in patients with recurrent follicular lymphoma, mantle cell lymphoma and Waldenström macroglobulinaemia. Br J Haematol. 2010; 151:346-53.
Steven TP. How I treat Waldenstrӧm macroglobulinemia. Blood. 2015; 126: 721-32
Treon SP, Soumerai JD, Branagan AR, Hunter ZR, Patterson CJ, Ioakimidis L, et al. Thalidomide and rituximab in Waldenstrom macroglobulinemia. Blood. 2008; 112:4452-7.
Leblond V, Johnson S, Chevret S, Copplestone A, Rule S, Tournilhac O, et al. Results of a randomized trial of chlorambucil versus fludarabine for patients with untreated Waldenström macroglobulinemia, marginal zone lymphoma, or lymphoplasmacytic lymphoma. J Clin Oncol. 2013; 31:301-7.
Leleu X, Soumerai J, Roccaro A, Hatjiharissi E, Hunter ZR, Manning R, et al. Increased incidence of transformation and myelodysplasia / acute leucemia in patients with Waldenstrom macroglobulinemia treated with nucleoside analogs. J Clin Oncol. 2009; 27:250-5.
Yang K, Tan J, Wu T. Alkylating agents for Waldenstrom´s macroglobulinaemia. Cochrane Database Syst Rev. 2009; CD006719.
Anagnostopoulos A, Hari PN, Pérez WS, Ballen K, Bashey A, Bredeson CN, et al. Autologous or allogeneic stem cell transplantation in patients with Waldenstrom´s macroglobulinemia. Biol Blood Marrow Transplant 2006; 12:845-54.
Garnier A, Robin M, Larosa F, Golmard JL, Le Gouill S, Coiteux V, et al. Allogeneic hematopoietic stem cell transplantation allows long-term complete remission and curability in high-risk Waldenström’s macroglobulinemia. Results of a retrospective analysis of the Société Française de Greffe de Moelle et de Thérapie Cellulaire. Haematologica. 2010; 95:950-5.
Sekhar J, Sanfilippo K, Zhang Q, Trinkaus K, Vij R, Morgensztern D. Waldenström macroglobulinemia: a surveillance, epidemiology, and end results database review from 1988 to 2005. Leuk Lymphoma. 2012; 53:1625-6.
Kristinsson SY, Eloranta S, Dickman PW, Andersson TM, Turesson I, Landgren O, et al. Patterns of survival in lymphoplasmacytic lymphoma/Waldenström macroglobulinemia: a population-based study of 1,555 patients diagnosed in Sweden from 1980 to 2005. Am J Hematol. 2013; 88:60-5.
Merlini G, Baldini L, Broglia C, Comelli M, Goldaniga M, Palladini G, et al. Prognostic factors in symptomatic Waldenstrom's macroglobulinemia. Semin Oncol. 2003; 30:211-5.
Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM, et al. Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. Br J Haematol 2006; 133:158-64.
Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J, et al. International prognostic scoring system for Waldenstrom macroglobulinemia. Blood. 2009; 113:4163-70.
Kastritis E, Kyrtsonis M, Hadjiiharissi E, Symeonidis A, Michalis E, Repoussis P, et al. Validation of the International Prognostic Scoring System (IPSS) for Waldenstrom´s macroglobulinemia (WM) and the importance of serum lactate dehydrogenase (LDH). Leuk Res. 2010;34:1340-3.
Downloads
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna
Open Access
