Head and Neck Pathology

, Volume 6, Issue 1, pp 111–120 | Cite as

Crystal-Storing Histiocytosis: Report of a Case, Review of the Literature (80 Cases) and a Proposed Classification

  • Snjezana Dogan
  • Leon Barnes
  • Wilhelmina P. Cruz-Vetrano
North American Society of Head and Neck Pathology Companion Meeting, March 18, 2012, Vancouver, British Colombia

Abstract

We report a case of crystal storing histiocytosis (CSH) of the upper lip and cheek in a 51-year-old woman and review the clinicopathologic features of 80 cases in the literature. These occurred in 41 men and 39 women with a respective mean age of 59 and 61 years (range 17–81 years). Forty-six patients (58%) had localized CSH, and, of these, 16 (35%) occurred in the head and neck, with the most common site being the eye/orbit. The remaining 34 patients (42%) had generalized CSH primarily involving bone marrow, liver, lymph nodes, spleen and/or kidney. Regardless of whether the CSH was localized or generalized, the vast majority of patients (90%) had an underlying lymphoproliferative or plasma cell disorder, especially multiple myeloma, lymphoplasmacytic lymphoma, or monoclonal gammopathy of undetermined significance. In 7 cases (8.8%), the CSH was associated with a variety of benign disorders, often with an inflammatory background, and no evidence of a clonal lymphoproliferative or plasma cell disorder. Treatment and prognosis varied according to the underlying disease. A classification of CSH based on etiology and/or associated disease and chemical composition of the crystal is proposed, rare non-immunoglobulin variants of CSH are discussed, and a differential diagnosis of other potentially confusing lesions is provided.

Keywords

Crystal-storing histiocytosis Intracellular crystals Immunoglobulin crystals Histiocytes 

Notes

Acknowledgments

We thank Raymond E. Felgar, MD, PhD of the Division of Hematopathology, University of Pittsburgh Medical Center, Presbyterian Hospital for reviewing this case, Jay Arlick, DMD, oral surgeon, Clearfield Hospital, Clearfield, Pennsylvania, who removed the lesion and provided some evaluation and follow-up and Jacinthe Chenevert, MD, Division of Endocrine and Head and Neck Pathology, University of Pittsburgh Medical Center, Presbyterian Hospital for collecting some published data.

References

  1. 1.
    Kapadia SB, Enzinger FM, Heffner DK, Hyams VJ, Frizzera G. Crystal-storing histiocytosis associated with lymphoplasmacytic neoplasms. Report of three cases mimicking adult rhabdomyoma. Am J Surg Pathol. 1993;17:461–7.PubMedCrossRefGoogle Scholar
  2. 2.
    Lebeau A, Zeindl-Eberhart E, Muller E-C, Muller-Hocker J, Jungblut PR, Emmerich B, Lohrs U. Generalized crystal-storing histiocytosis associated with monoclonal gammopathy: molecular analysis of a disorder with rapid clinical course and review of literature. Blood. 2002;100:1817–27.PubMedGoogle Scholar
  3. 3.
    Jones D, Bhatia VK, Krausz T, Pinkus GS. Crystal-storing histiocytosis : a disorder occurring in plasmacytic tumors expressing immunoglobulin kappa light chain. Hum Pathol. 1999;30:1441–8.PubMedCrossRefGoogle Scholar
  4. 4.
    Gebrail F, Knapp M, Perrotta G, Cualing H. Crystalline histiocytosis in hereditary cystinosis. Arch Pathol Lab Med. 2002;126(9):1135.PubMedGoogle Scholar
  5. 5.
    Lewis JT, Candelora JN, Hogan RB, Briggs FR, Abraham SC. Crystal-storing histiocytosis due to massive accumulation of Charcot-Leyden crystals: a unique association producing colonic polyposis in a 78-year old woman with eosinophilic colitis. Am J Surg Pathol. 2007;31:481–5.PubMedCrossRefGoogle Scholar
  6. 6.
    Sukpanichnant S, Hargrove NS, Kachintorn U, Manatsathit S, Chanchairujira T, Siritanaratkul N, Akaravipuyh T, Thakerngpol K. Clofazimine-induced crystal-storing histiocytosis producing chronic abdiminable pain in a leprosy patient. Am J Surg Pathol. 2000;24:129–35.PubMedCrossRefGoogle Scholar
  7. 7.
    Bosman C, Camassei FD, Boldrini R, Piro FR, Saponara M, Romeo R, Corsi A. Solitary crystal-storing histiocytosis of the tongue in a patient with rheumatoid arthritis and polyclonal hypergammaglobulinemia. Arch Pathol Lab Med. 1998;122:920–4.PubMedGoogle Scholar
  8. 8.
    Colby TV, Koss MN, Travis WD. Tumors of the lower respiratory tract. Washington, DC: Armed Forces Institute of Pathology; 1995. Atlas of tumor pathology, 3rd series, fascicle 13.Google Scholar
  9. 9.
    Coupland SE, Foss HD, Hummel M, Stein H. Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis. Ophthalmology. 2002;109:105–10.PubMedCrossRefGoogle Scholar
  10. 10.
    De Alba Campomanes AG, Rutar T, Crawford JB, Seiff S, Goodman D, Grenert J. Crystal-storing histiocytosis and crystalline keratopathy caused by monoclonal gammopathy of undetermined significance. Cornea. 2009;28(9):1081–4.PubMedCrossRefGoogle Scholar
  11. 11.
    De Lastours V, Papo T, Cazals-Hatem D, Eden A, Feydy A, Belmatoug N, Chauveheid MP, Lidove O, Fantin B. Bone involvement in generalized crystal-storing histiocytosis. J Rheumatol. 2006;33(11):2354–8.PubMedGoogle Scholar
  12. 12.
    El Hamel C, Thierry A, Trouillas P, Bridoux F, Carrion C, Quellard N, Goujon JM, Aldigier JC, Gombert JM, Cogné M, Touchard G. Crystal-storing histiocytosis with renal Fanconi syndrome: pathological and molecular characteristics compared with classical myeloma-associated Fanconi syndrome. Nephrol Dial Transplant. 2010;25(9):2982–90. Epub 2010 Mar 31.PubMedCrossRefGoogle Scholar
  13. 13.
    Fairweather PM, Williamson R, Tsikleas G. Pulmonary extranodal marginal zone lymphoma with massive crystal storing histiocytosis. Am J Surg Pathol. 2006;30:262–7.PubMedCrossRefGoogle Scholar
  14. 14.
    Farooq U, Bayerl MG, Abendroth CS, Verma C, Talamo G. Renal crystal storing histiocytosis in a patient with multiple myeloma. Ann Hematol. 2009;88:807–9.PubMedCrossRefGoogle Scholar
  15. 15.
    Friedman MT, Molho L, Valderrama E, Kahn LB. Crystal-storing histiocytosis associated with a lymphoplasmacytic neoplasm mimicking adult rhabdomyoma. Arch Pathol Lab Med. 1996;120:1133–6.PubMedGoogle Scholar
  16. 16.
    Galed-Placed I. Immunoglobulin crystal-storing histiocytosis in a pleural effusion from a woman with IgA kappa multiple myeloma: a case report. Acta Cytol. 2006;50:539–41.PubMedCrossRefGoogle Scholar
  17. 17.
    Garcia JF, Sanchez E, Lloret E, Martin J, Piris MA. Crystal-storing histiocytosis and immunocytoma associated with multifocal fibrosclerosis. Histopathology. 1998;33:459–64.CrossRefGoogle Scholar
  18. 18.
    Grossniklaus HE, Stulting RD, L’Hernault N. Corneal and conjunctival crystals in paraproteinemia. Hum Pathol. 1990;21:1181–3.PubMedCrossRefGoogle Scholar
  19. 19.
    Harada M, Shimada M, Fukayama M, Kaneko T, Kitazume K, Weiss SW. Crystal-storing histiocytosis associated with lymphoplasmacytic lymphoma mimicking Webel Christian disease: immunohistochemical, ultrastructural, and gene-rearrangement studies. Hum Pathol. 1996;27(1):84–7.PubMedCrossRefGoogle Scholar
  20. 20.
    Ionescu DN, Pierson DM, Qing G, Li M, Colby TV, Leslie KO. Pulmonary crystal-storing histiocytoma. Arch Pathol Lab Med. 2005;129:1159–63.PubMedGoogle Scholar
  21. 21.
    Joo M, Kwak JE, Chang SH, Kim H, Chi JG, Moon YS, Kim KM. Localized gastric crystal-storing histiocytosis. Histopathology. 2007;51(1):116–9. Epub 2007 May 31.PubMedCrossRefGoogle Scholar
  22. 22.
    Kaminsky IA, Wang AM, Olsen J, Schechter S, Wilson J, Olson R. Central nervous system crystal-storing histiocytosis: neuroimaging, neuropathology, and literature review. AJNR Am J Neuroradiol. 2011;32(2):E26–8. Epub 2010 Jan 28.PubMedCrossRefGoogle Scholar
  23. 23.
    Kaufmann O, Hansen A, Deicke P, Burmester GR, Dietel M. Subcutaneous crystal-storing histiocytosis associated with lymphoplasmacytic lymphoma (immunocytoma). Pathol Res Pract. 1996;192:1148–51.PubMedCrossRefGoogle Scholar
  24. 24.
    Kazzaz B, Dewar A, Corrin B. An unusual pulmonary plasmacytoma. Histopathology. 1992;21:285–7.PubMedCrossRefGoogle Scholar
  25. 25.
    Keane C, Gill D. Multi-organ involvement with crystal-storing histiocytosis. Br J Haematol. 2008;141(6):750. Epub 2008 Apr 22.PubMedCrossRefGoogle Scholar
  26. 26.
    Kornstein MJ, deBlois GG, Williams ME. Unusual biclonal plasma cell dyscrasia with crystallike inclusions producing colonic tumors (plasma cell polyposis) and terminating in T-cell lymphoma. Arch Pathol Lab Med. 1992;116(2):168–72.PubMedGoogle Scholar
  27. 27.
    Kusakabe T, Watanabe K, Mori T, Iida T, Suzuki T. Crystal-storing histiocytosis associated with MALT lymphoma of the ocular adnexa: a case report with review of literature. Virchows Arch. 2007;450(1):103–8. Epub 2006 Nov 17.PubMedCrossRefGoogle Scholar
  28. 28.
    László R, Degrell P, Kellermayer M, Bollmann D, Egyed M, Seress L, Pajor L. Crystal-storing histiocytosis associated with only one of two consecutive, but genetically unrelated B-cell lymphomas. Pathol Res Pract. 2009;205(4):273–8. Epub 2008 Dec 21.PubMedCrossRefGoogle Scholar
  29. 29.
    Lee WS, Kim SR, Moon H, Choe YH, Park SJ, Lee HB, Jin GY, Chung MJ, Lee YC. Pulmonary crystal-storing histiocytoma in a patient without a lymphoproliferative disorder. Am J Med Sci. 2009;338(5):421–4.PubMedCrossRefGoogle Scholar
  30. 30.
    Lesesve JF, Bronowicki JP, Galed-Placed I. Crystal-storing histiocytosis in ascites from a patient with IgM kappa lymphoplasmacytic lymphoma. Cytopathology. 2011;22(3):207–8. doi: 10.1111/j.1365-2303.2010.00823.PubMedCrossRefGoogle Scholar
  31. 31.
    Llobet M, Castro P, Barcelo C, Trull JM, Campo E, Bernadó L. Massive crystal-storing histiocytosis associated with low-grade malignant B-cell lyanphoma of MALT-type of the parotid gland. Diagn Cytopathol. 1997;17:148–52.PubMedCrossRefGoogle Scholar
  32. 32.
    Martin AW, Carstens PH, Yam LT. Crystalline deposits in ascites in a case of cryoglobulinemia. Acta Cytol. 1987;31(5):631–6.PubMedGoogle Scholar
  33. 33.
    Mennemeyer R, Hammar SE, Cathey WJ. Malignant lymphoma with intracytoplasmic IgM crystalline inclusions. N EnglJ Med. 1974;291:960–3.CrossRefGoogle Scholar
  34. 34.
    Mullen B, Chalvardjian A. Crystalline tissue deposits on a case of multiple myeloma. Arch Pathol Lab Med. 1981;105(2):94–7.PubMedGoogle Scholar
  35. 35.
    Padmalatha C, Warner TF, Hafez GR. Pseudo-Gaucher cell in IgMk plasmacytoid lymphoma. Am J Surg Pathol. 1981;5(5):501–5.PubMedCrossRefGoogle Scholar
  36. 36.
    Papla B, Spolnik P, Rzenno E, Zduńczyk A, Rudzki Z, Okoń K, Szczepański W, Dabroś W, Stachura J. Generalized crystal-storing histiocytosis as a presentation of multiple myeloma: a case with a possible pro-aggregation defect in the immunoglobulin heavy chain. Virchows Arch. 2004;445:83–9.PubMedGoogle Scholar
  37. 37.
    Pinkerton RM, Robertson DM. Corneal and conjunctival changes in dysproteinemia. Invest Ophthalmol. 1969;8:357–64.PubMedGoogle Scholar
  38. 38.
    Pitman SD, Wang J, Serros ER, Zuppan C. A 70-year-old woman with acute renal failure. Crystal-storing histiocytosis. Arch Pathol Lab Med. 2006;130:1077–8.PubMedGoogle Scholar
  39. 39.
    Pock L, Stuchlik D, Hercogova J. Crystal storing histiocytosis of the skin associated with multiple myeloma. Int J Dermatol. 2006;45:1408–11.PubMedCrossRefGoogle Scholar
  40. 40.
    Prasad MJ, Charney DA, Sarlin J, Keller SM. Pulmonary immunocytoma with massive crystal storing histiocytosis. Am J Surg Pathol. 1998;22:1148–53.PubMedCrossRefGoogle Scholar
  41. 41.
    Rao NA, Font RL. Plasmacytic conjunctivitis with crystalline inclusions. Immunohistochemical and ultrastructural studies. Arch Ophthalmol. 1980;98(5):836–41.PubMedCrossRefGoogle Scholar
  42. 42.
    Robak T, Urbanska-Rys H, Jerzmanowski P, Bartkowiak J, Liberski P, Kordek R. Lymphoplasmacytic lymphoma with monoclonal gammopathy-related pseudo- Gaucher cell infiltration in bone marrow and spleen—diagnostic and therapeutic dilemmas. Leuk Lymphoma. 2002;43:2343–50.PubMedGoogle Scholar
  43. 43.
    Rossi G, Morandi U, Nannini N, Fontana G, Pifferi M, Casali C. Crystal-storing histiocytosis presenting with pleural disease. Histopathology. 2010;56(3):403–5.PubMedCrossRefGoogle Scholar
  44. 44.
    Sailey CJ, Alexiev BA, Gammie JS, Pinell-Salles P, Stafford JL, Burke A. Crystal-storing histiocytosis as a cause of symptomatic cardiac mass. Arch Pathol Lab Med. 2009;133(11):1861–4.PubMedGoogle Scholar
  45. 45.
    Schaefer HE. Gammopathy-related crystal-storing histiocytosis, pseudo- and pseudo-pseudo-Gaucher cells. Critical commentary and mini-review. Pathol Res Pract. 1996;192(11):1152–62. Review.PubMedCrossRefGoogle Scholar
  46. 46.
    Sethi S, Cuiffo BP, Pinkus GS, Rennke HG. Crystal-storing histiocytosis involving the kidney in a low-grade B-cell lymphoproliferative disorder. Am J Kidney Dis. 2002;39:183–8.PubMedCrossRefGoogle Scholar
  47. 47.
    Stokes MB, Aronoff B, Siegel D, D’Agati VD. Dysproteinemia related nephropathy associated with crystal-storing histiocytosis. Kidney Int. 2006;70:597–602.PubMedCrossRefGoogle Scholar
  48. 48.
    Sun Y, Tawfiqul B, Valderrama E, Kline G, Kahn LB. Pulmonary crystal-storing histiocytosis and extranodal marginal zone B-cell lymphoma associated with a fibroleiomyomatous hamartoma. Ann Diagn Pathol. 2003;7:47–53.PubMedCrossRefGoogle Scholar
  49. 49.
    Takahashi K, Naito M, Takatsuki K, et al. Multiple myeloma, IgA kappa type, accompanying crystal-storing histiocytosis and amyloidosis. Acta Pathol Jpn. 1987;37:141–54.PubMedGoogle Scholar
  50. 50.
    Terashima K, Takahashi K, Kojima M, Imai Y, Tsuchida S, Migita S, Ebina S, Itoh C. Kappa-type light chain crystal storage histiocytosis. Acta Pathol Jpn. 1978;28:111–38.PubMedGoogle Scholar
  51. 51.
    Tholouli E, Krebs M, Reeve R, Houghton JB. Crystal-storing histiocytosis in a patient with IgGK multiple myeloma. Br J Haematol. 2005;128:412.PubMedCrossRefGoogle Scholar
  52. 52.
    Thorson P, Hess JL. Transformation of monocytoid B-cell lymphoma to large cell lymphoma associated with crystal-storing histiocytes. Arch Pathol Lab Med. 2000;124:460–2.PubMedGoogle Scholar
  53. 53.
    Todd WU, Drabick JJ, Benninghoff MG, Frauenhoffer EE, Zander DS. Pulmonary crystal-storing histiocytosis diagnosed by computed tomography-guided fine-needle aspiration. Diagn Cytopathol. 2010;38(4):274–8.PubMedGoogle Scholar
  54. 54.
    Tomioka M, Ueki K, Nakahashi H, Isoda A, Kuroiwa T, Kaneko Y, Hiromura K, Nojima Y. Widespread crystalline inclusions affecting podocytes, tubular cells and interstitial histiocytes in the myeloma kidney. Clin Nephrol. 2004;62:229–33.PubMedGoogle Scholar
  55. 55.
    Weichman K, Dember LM, Prokaeva T, Wright DG, Quillen K, Rosenzweig M, Skinner M, Seldin DC, Sanchorawala V. Clinical and molecular characteristics of patients with non-amyloid light chain deposition disorders, and outcome following treatment with high-dose melphalan and autologous stem cell transplantation. Bone Marrow Transplant. 2006;38(5):339–43. Epub 2006 Jul 24.PubMedCrossRefGoogle Scholar
  56. 56.
    Yamamoto T, Hishida A, Honda N, Ito I, Shirasawa H, Nagase M. Crystal-storing histiocytosis and crystalline tissue deposition in multiple myeloma. Arch Pathol Lab Med. 1991;115:351–4.PubMedGoogle Scholar
  57. 57.
    Zioni F, Giovanardi P, Bozzoli M, Artusi T, Bonacorsi G, Sighinolfi P. Massive bone marrow crystal-storing histiocytosis in a patient with IgA-lambda multiple myeloma and extensive extramedullary disease. A case report. Tumori. 2004;90:348–51.PubMedGoogle Scholar
  58. 58.
    Stewart CJR, Spagnolo DV. Crystalline plasma cell inclusions in helicobacter-associated gastritis. J Clin Pathol. 2006;59:851–4.PubMedCrossRefGoogle Scholar
  59. 59.
    Alayed KM, Alabdulaali MK, Alkhairy KS, Elnour S, Alhajjaj A. Aggressive systemic mastocytosis with Charcot-Leyden crystals-associated crystal storing histiocytosis in bone marrow. Pathology. 2010;42(1):85–7.PubMedCrossRefGoogle Scholar
  60. 60.
    Dvorak AM, Weller PF, Monahan-Earley RA, Letourneau L, Ackerman SJ. Ultrastructural localization of Charcot-Leyden crystal protein (lysophospholipase) and peroxidase in macrophages, eosinophils, and extracellular matrix of the skin in the hypereosinophilic syndrome. Lab Invest. 1990;62(5):590–607.PubMedGoogle Scholar
  61. 61.
    Weiss SW, Enzinger FM, Johnson FB. Silca reaction simulating fibrous histioctoma. Cancer. 1978;42:2738–43.PubMedCrossRefGoogle Scholar
  62. 62.
    Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003;27(5):579–93.PubMedCrossRefGoogle Scholar
  63. 63.
    Rodriguez J, Ackerman AB. Xanthogranuloma in adults. Arch Dermatol. 1976;112(1):43–4.PubMedCrossRefGoogle Scholar
  64. 64.
    Chen M, Wang J. Gaucher disease: review of the literature. Arch Pathol Lab Med. 2008;132(5):851–3. Review.PubMedGoogle Scholar
  65. 65.
    Yousef GM, Naghibi B, Hamodat MM. Malakoplakia outside the urinary tract. Arch Pathol Lab Med. 2007 Feb;131(2):297-300. Review. Erratum in: Arch Pathol Lab Med. 2009 Jun;133(6):850. Hamodat, Mowafak M [added].Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Snjezana Dogan
    • 1
  • Leon Barnes
    • 2
  • Wilhelmina P. Cruz-Vetrano
    • 3
  1. 1.Department of PathologyMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  2. 2.Emeritus Professor of PathologyUniversity of Pittsburgh Medical Center, Presbyterian HospitalPittsburghUSA
  3. 3.Laboratory ServicesAltoona Regional Health SystemAltoonaUSA

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