Langerhans Cell Histiocytosis in Bone Marrow

Langerhans Cell Histiocytosis in Bone Marrow

Wright-stained bone marrow aspirate smear from an infant with Langerhans cell histiocytosis demonstrates 2 Langerhans cells with elongated dendritic projections.

Hematoxylin and eosin-stained section of a solitary lytic lesion of the skull demonstrates a proliferation of Langerhans cells image with grooved nuclei intermixed with many eosinophils.



  • Langerhans cell histiocytosis (LCH)


  • Histiocytosis X, eosinophilic granuloma (solitary lesion)

  • Hand-Schüller-Christian disease (multiple lesions)

  • Letterer-Siwe disease (disseminated disease or visceral involvement)


  • Nonneoplastic Langerhans cells (LC)

    • First described by Paul Langerhans in 1868

    • Bone marrow-derived antigen-presenting cells

    • Normally reside in mucosal sites and epidermis

    • Migrate to lymph node following antigenic stimulation

  • Langerhans cell histiocytosis

    • Clonal proliferation of LCs in all forms except smoking-related pulmonary LCH

    • Morphologic, immunophenotypic, and ultra-structural features are similar to nonneoplastic LCs


Etiology Remains Poorly Understood

  • Documented affected relative in 1% of patients

  • Higher rate of concordance for LCH in monozygotic (92%) vs. dizygotic (10%) twins

  • Reported chromosomal instability and loss of heterozygosity on chromosomes 1, 4, 6, 7, 9, 16, 17, and 22

  • No apparent genomic aberrations in some studies



  • Incidence

    • 8-9 cases per 1,000,000 in children

    • 1-2 cases per 1,000,000 in adults

  • Gender

    • More common in males (M:F ratio 3.7:1)


  • 2 major groups (per Histiocyte Society)

  • Single-system Langerhans cell histiocytosis

    • Single site disease (unifocal lymph node, skin, lung, pituitary, or bone)

    • Multifocal disease (multifocal bone or multiple lymph nodes)

  • Multi-system Langerhans cell histiocytosis

    • 2 or more organs involved at diagnosis without organ dysfunction

    • 2 or more organs involved at diagnosis with organ dysfunction

      • High risk: Involvement of 1 or more risk organs (liver, lung, spleen, or hematopoietic system)

      • Low risk: Involvement of other organs


Jun 13, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Langerhans Cell Histiocytosis in Bone Marrow
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