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Research Article| Volume 19, ISSUE 1, P135-156, March 1999

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The Histiocytoses

  • Ronald Jaffe
    Correspondence
    Address reprint requests to: Ronald Jaffe, MB, BCh, Pathology Department, Children’s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213.
    Affiliations
    From the Department of Pathology, University of Pittsburgh School of Medicine; and Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania
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      This paper is only available as a PDF. To read, Please Download here.
      The systemic histiocytoses are uncommon disorders, generally confined to childhood. An explosion in interest in dendritic cells in general is leading to greater insight into the lineage affiliations of the constituent cells. Only multi-institutional prospective studies will elucidate the natural history and biological potential of the more unusual examples described in this overview.
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      References

        • Adle-Biassette H.
        • Chetritt J.
        • Bergemer-Fouquet A.M.
        • et al.
        Pathology of the central nervous system in Chester-Erdheim disease: Report of three cases.
        J Neuropathol Exp Neurol. 1997; 56: 1207
        • Arico M.
        • Janka G.
        • Fischer A.
        • et al.
        Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society.
        Leukemia. 1996; 10: 197
        • Babu R.P.
        • Lansen T.A.
        • Chadburn A.
        • et al.
        Erdheim-Chester disease of the central nervous system. Report of two cases.
        J Neurosurg. 1997; 86: 888
        • Baumgartner I.
        • von Hochstetter A.
        • Baumert B.
        • et al.
        Langerhans’-cell histiocytosis in adults.
        Med Pediatr Oncol. 1997; 28: 9
        • Bhatia S.
        • Nesbit Jr, M.E.
        • Egeler R.M.
        • et al.
        Epidemiologic study of Langerhans cell histiocytosis in children.
        J Pediatr. 1997; 130: 774
        • Caputo R.
        • Veraldi S.
        • Grimalt R.
        • et al.
        The various clinical patterns of xanthoma disseminatum. Consideration of seven cases and review of the literature.
        Dermatology. 1995; 190: 19
        • Cella M.
        • Sallusto F.
        • Lanzavecchia A.
        Origin, maturation and antigen presenting function of dendritic cells.
        Curr Opin Immunol. 1997; 9: 10
        • Cerio R.
        • Griffiths C.E.
        • Cooper K.D.
        • et al.
        Characterization of Factor XHIa positive dermal dendritic cells in normal and inflamed skin.
        Br J Dermatol. 1989; 121: 421
        • Cerio R.
        • Spaull J.
        • Jones E.W.
        Histiocytoma cutis: A tumour of dermal dendrocytes (dermal dendrocytoma).
        Br J Dermatol. 1989; 120: 197
        • de Graaf J.
        • Timens W.
        • Tamminga R.Y.
        • et al.
        Deep juvenile xanthogranuloma: A lesion related to dermal indeterminate cells.
        Hum Pathol. 1992; 23: 905
        • Dolezal M.V.
        • Kamel O.W.
        • van de Rijn M.
        • et al.
        Virus-associated hemophagocytic syndrome characterized by clonal Epstein-Barr virus genome.
        Am J Clin Pathol. 1995; 103: 189
        • Egeler R.M.
        • Neglia J.
        • Puccetti D.M.
        • et al.
        The association of Langerhans cell histiocytosis with malignant neoplasms.
        Cancer. 1993; 71: 865
        • Eisen R.N.
        • Buckley P.J.
        • Rosai J.
        Immunophenotypic characterisation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).
        Semin Diagn Pathol. 1990; 7: 74
        • Favara B.E.
        Histopathology of the liver in Histiocytosis syndromes.
        Pediatr Pathol Lab Med. 1996; 16: 413
        • Favara B.E.
        • Feller A.C.
        • Pauli M.
        • et al.
        Contemporary classification of histiocytic disorders. The WHO Committee on Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society.
        Med Pediatr Oncol. 1997; 29: 157
        • Favara B.E.
        • Jaffe R.
        The histopathology of Langerhans’ cell histiocytosis.
        Br J Cancer. 1994; 70: S17
        • Favara B.E.
        • Steele A.
        Langerhans cell histiocytosis of lymph nodes: A morphological assessment of 43 biopsies.
        Pediatr Pathol Lab Med. 1997; 17: 769
        • Foucar E.
        • Rosai J.
        • Dorfman R.F.
        Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity.
        Semin Diagn Pathol. 1990; 7: 19
        • Freyer D.R.
        • Kennedy R.
        • Bostrom B.C.
        • et al.
        Juvenile xanthogranuloma: Forms of systemic disease and their clinical implications.
        J Pediatr. 1996; 129: 227
        • Galy A.
        • Travis M.
        • Cen D.
        • et al.
        Human T, B, natural killer and dendritic cells arise from a common bone marrow progenitor cell subset.
        Immunity. 1995; 3: 459
        • Geissmann F.
        • Emile J.F.
        • Andry P.
        • et al.
        Lack of expression of E-cadherin is associated with dissemination of Langerhans’ cell histiocytosis and poor outcome.
        J Pathol. 1997; 181: 301
        • Gilcrease M.Z.
        • Rajan B.
        • Ostrowski M.L.
        • et al.
        Localized thymic Langerhans’ cell histiocytosis and its relationship with myasthenia gravis. Immunohistochemical, ultrastructural, and cytometric studies.
        Arch Pathol Lab Med. 1997; 121: 134
        • Glusac E.J.
        • Barr R.J.
        • Everett M.A.
        • et al.
        Epithelioid cell histiocytoma. A report of 10 cases including a new cellular variant.
        Am J Surg Pathol. 1994; 18: 583
        • Grateau G.
        • Bachmeyer C.
        • Blanche P.
        • et al.
        Hemophagocytic syndrome in patients infected with the human immunodeficiency virus: Nine cases and a review.
        J Infect. 1997; 34: 219
        • Grois N.
        • Broadbent V.
        • Favara B.E.
        • et al.
        Report of the Histiocyte Society Workshop on “Central Nervous System (CNS) Disease in Langerhans Cell Histiocytosis (LCH).”.
        Med Pediatr Oncol. 1997; 29: 73
        • Hart D.N.
        Dendritic cells: Unique leukocyte populations which control the primary immune response.
        Blood. 1997; 90: 3245
        • Henter J.I.
        • Eiinder G.
        • Ost A.
        Diagnostic guidelines for hemophagocytic lymphohistiocytosis. FHL Study Group of the Histiocyte Society.
        Semin Oncol. 1991; 18: 29
        • Henter J.I.
        • Eiinder G.
        • Soder O.
        • et al.
        Hypercytokinemia in familial hemophagocytic lymphohistiocytosis.
        Blood. 1991; 78: 2918
        • Henter J.I.
        • Nennesmo I.
        Neuropathologie findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis.
        J Pediatr. 1997; 130: 358
        • Hernandez-Martin A.
        • Baselga E.
        • Drolet B.A.
        • et al.
        Juvenile Xanthogranuloma.
        J Am Acad Dermatol. 1997; 36: 355
        • Hirst W.J.R.
        • Layton D.M.
        • Singh S.
        • et al.
        Hemophagocytic lymphohistiocytosis: Experience at two U.K. centres.
        Br J Haematol. 1994; 88: 731
        • Imashuku S.
        • Hibi S.
        • Morinaga S.
        • et al.
        Hemophagocytic lymphohistiocytosis in association with granular lymphocyte proliferative disorders in early childhood: Characteristic bone marrow morphology.
        Br J Haematol. 1997; 96: 708
        • Imashuku S.
        • Hibi S.
        • Todo S.
        Hemophagocytic lymphohistiocytosis in infancy and childhood.
        J Pediatr. 1997; 130: 352
        • Jaffe R.
        • De Vaughn D.
        • Langhoff E.
        Fascin and the differential diagnosis of childhood histiocytic lesions.
        Pediatr & Dev Pathol. 1998; 1: 216
        • Kambouchner M.
        • Colby T.V.
        • Domenge C.
        • et al.
        Erdheim-Chester disease with prominent pulmonary involvement associated with eosinophilic granuloma of mandibular bone.
        Histopathology. 1997; 30: 353
        • Ladisch S.
        • Gadner H.
        Treatment of Langerhans’ cell histiocytosis—evolution and current approaches.
        Br J Cancer. 1994; 70: S41
        • Lay J.D.
        • Tsao C.J.
        • Chen J.Y.
        • et al.
        Upregulation of tumor necrosis factor alpha gene by Epstein-Barr virus and activation of macrophages in Epstein-Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome.
        J Clin Invest. 1997; 100: 1969
        • Le Blanc A.
        • Hadchouel M.
        • Jehan P.
        • et al.
        Obstructive jaundice in children with histiocytosis X.
        Gastroenterology. 1981; 80: 134
        • Lieberman P.H.
        • Jones C.R.
        • Steinman R.M.
        • et al.
        Langerhans cell (eosinophilic) granulomatosis: Clinicopathologic study encompassing 50 years.
        Am J Surg Pathol. 1996; 20: 519
        • Listinsky C.M.
        Common reactive erythrophagocytosis in axillary lymph nodes.
        Am J Clin Pathol. 1988; 90: 189
        • Liu Y.J.
        • Grouard G.
        • de Bouteiller O.
        • et al.
        Follicular dendritic cells and germinal centers.
        Int Rev Cytol. 1996; 166: 139
        • Manente L.
        • Cotellessa C.
        • Schmitt I.
        • et al.
        Indeterminate cell histiocytosis; a rare histiocytic disorder.
        Am J Dermatopathol. 1997; 19: 276
        • Marder R.J.
        • Variakojis D.
        • Silver J.
        • et al.
        Immunohistochemical analysis of human lymphomas with monoclonal antibodies to B cell and la antigens reactive in paraffin section.
        Lab Invest. 1985; 52: 497
        • Muoy R.
        • Stephan J.L.
        • Piliet P.
        • et al.
        Efficacy of cyclosporin A in the treatment of macrophage activation syndrome in juvenile arthritis: A report of five cases.
        J Pediatr. 1996; 129: 750
        • Murphy G.F.
        • Bhan A.K.
        • Sato S.
        • et al.
        Characterization of Langerhans cells by the use of monoclonal antibodies.
        Lab Invest. 1981; 45: 465
        • Nascimento A.G.
        A clinicopathologic and immunohistochemical comparative study of cutaneous and intramuscular forms of juvenile xanthogranuloma.
        Am J Surg Pathol. 1997; 21: 645
        • Newman C.C.
        • Raimer S.S.
        • Sanchez R.L.
        Nonlipidized juvenile xanthogranuloma: A histologic and immunohistochemical study.
        Pediatr Dermatol. 1997; 14: 98
        • Nezelof C.
        • Basset F.
        • Rousseau M.F.
        Histiocytosis X. Histogenetic arguments for a Langerhans’ cell origin.
        Biomedicine. 1973; 18: 365
        • Nickoloff B.J.
        • Wood G.S.
        • Chu M.
        • et al.
        Disseminated dermal dendrocytoma: A new cutaneous fibrohistiocytic proliferation?.
        Am J Surg Pathol. 1990; 14: 867
        • Okano M.
        • Gross T.G.
        Epstein-Barr virus-associated hemophagocytic syndrome and fatal infectious mononucleosis.
        Am J Hematol. 1996; 53: 111
        • Osugi Y.
        • Hara J.
        • Tagawa S.
        • et al.
        Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis.
        Blood. 1997; 89: 4100
        • Paulli M.
        • Bergamaschi G.
        • Tonon L.
        • et al.
        Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).
        Br J Haematol. 1995; 91: 415
        • Paulli M.
        • Feller A.C.
        • Boveri E.
        • et al.
        Cathepsin D and E co-expression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans’ cell histiocytosis: Further evidences of a phenotypic overlap between these histiocytic disorders.
        Virchows Arch. 1994; 424: 601
        • Pinkus G.S.
        • Pinkus J.L.
        • Langhoff E.
        • et al.
        Fascin, a sensitive new marker for Reed-Sternberg cells of Hodgkin’s disease. Evidence for a dendritic or B cell derivation?.
        Am J Pathol. 1997; 150: 543
        • Poulter L.W.
        • Campbell D.A.
        • Munro C.
        • et al.
        Discrimination of human macrophages and dendritic cells by means of monoclonal antibodies.
        Scand J Immunol. 1986; 24: 351
        • Rubin C.M.
        • Burke B.A.
        • McKenna R.W.
        • et al.
        The accelerated phase of Chediak-Higashi syndrome. An expression of the virus-associated hemophagocytic syndrome?.
        Cancer. 1985; 56: 524
        • Salisbury J.R.
        • Hall P.A.
        • Williams H.C.
        • et al.
        Multicentric reticulohistiocytosis. Detailed immunophenotyping confirms macrophage origin.
        Am J Surg Pathol. 1990; 14: 687
        • Sangueza O.P.
        • Salmon J.K.
        • White Jr C.R.
        • et al.
        Juvenile xanthogranuloma: A clinical, histopathologic and immunohistochemical study.
        J Cutan Pathol. 1995; 22: 327
        • Shin S.S.
        • Sheibani K.
        • Kezirian J.
        • et al.
        Immunoarchitecture of normal human bone marrow. A study of frozen and fixed tissue sections.
        Hum Pathol. 1992; 23: 686
        • Sidoroff A.
        • Zeiger B.
        • Steiner H.
        • et al.
        Indeterminate cell histiocytosis—a clinicopathological entity with features of both X-and non-X histiocytosis.
        Br J Dermatol. 1996; 134: 525
        • Sorg U.R.
        • Morse T.M.
        • Patton W.N.
        • et al.
        Hodgkins cells express CD83, a dendritic cell lineage associated antigen.
        Pathology. 1997; 29: 294
        • Su I.J.
        • Hsu Y.H.
        • Lin M.T.
        • et al.
        Epstein-Barr virus containing T-cell lymphoma presents with hemophagocytic syndrome mimicking malignant histiocytosis.
        Cancer. 1993; 72: 2019
        • Suster S.
        • Hilsenbeck S.
        • Rywlin A.M.
        Reactive histiocytic hyperplasia with hemophago-cytosis in hematopoietic organs. A reevaluation of benign hemophagocytic proliferations.
        Hum Pathol. 1988; 19: 705
        • Suwabe H.
        • Tsutsumi Y.
        Reticulohistiocytoma involving the skin, subcutaneous tissue and a regional lymphnode.
        Pathol Int. 1996; 46: 531
        • Uccini S.
        • Vitolo D.
        • Stoppacciaro A.
        • et al.
        Immunoreactivity for S100 protein in dendritic and in lymphocyte-like cells in human lymphoid tissues.
        Virchows Arch B Cell Pathol. 1986; 52: 129
        • Veyssier-Belot C.
        • Cacoub P.
        • Caparros-Lefebvre D.
        • et al.
        Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.
        Medicine. 1996; 75: 157
        • Wacker H.H.
        • Frahm S.O.
        • Heidebrecht H.J.
        • et al.
        Sinus-lining cells of the lymphnodes recognised as a dendritic cell by the new monoclonal antibody Ki-M9.
        Am J Pathol. 1997; 151: 423
        • Weiss N.
        • Keller C.
        Xanthoma disseminatum: A rare normolipemic xanthomatosis.
        Clin Invest. 1993; 71: 233
        • Williams J.W.
        • Dorfman R.F.
        Lymphadenopathy as the initial manifestation of histiocytosis X.
        Am J Surg Pathol. 1979; 3: 405
        • Willman C.L.
        • Busque L.
        • Griffith B.B.
        • et al.
        Langerhans cell histiocytosis (Histiocytosis X): A clonal proliferative disease.
        N Engl J Med. 1994; 331: 154
        • Wood G.S.
        • Hu C.H.
        • Beckstead J.H.
        • et al.
        The indeterminate cell proliferative disorder: Report of a case manifesting as an unusual cutaneous histiocytosis.
        J Dermatol Surg Oncol. 1985; 11: 1111
        • Writing Group of the Histiocyte Society
        Histiocytosis syndromes in children.
        Lancet. 1987; 1: 208
        • Yu R.C.
        • Chu C.
        • Buluwela L.
        • et al.
        Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis.
        Lancet. 1994; 343: 767
        • Zeiger B.
        • Cerio R.
        • Soyer H.P.
        • et al.
        Reticulohistiocytoma and multicentric reticulohistio-cytosis. Histopathologic and immunophenotypic distinct entities.
        Am J Dermatopathol. 1994; 16: 577
        • Zeiger B.G.
        • Zeiger B.
        • Steiner H.
        • et al.
        Solitary giant xanthogranuloma and benign cephalic histiocytosis - variants of juvenile xanthogranuloma.
        Br J Dermatol. 1995; 133: 598
        • Zeiger B.W.
        • Sidoroff A.
        • Orchard G.
        • et al.
        Non-Langerhans cell histiocytoses. A new unifying concept.
        Am J Dermatopathol. 1996; 18: 490
        • Zenz W.M.
        • Urban C.E.
        • Popper H.H.
        • et al.
        Fatal pulmonary involvement in a patient with familial hemophagocytic lymphohistiocytosis.
        Pediatr Pulmonol. 1994; 17: 197
        • Zhou L.J.
        • Schwarting R.
        • Smith H.M.
        • et al.
        A novel cell-surface molecule expressed by human interdigitating reticulum cells, Langerhans cells, and activated lymphocytes is a new member of the Ig superfamily.
        J Immunol. 1992; 149: 735
        • Zvulunov A.
        • Barak Y.
        • Metzker A.
        Juvenile xanthogranuloma, neurofibromatosis and juvenile chronic myelogenous leukemia: World statistical analysis.
        Arch Dermatol. 1995; 131: 904