Korean J Lab Med.  2009 Oct;29(5):402-405. 10.3343/kjlm.2009.29.5.402.

Concurrent Langerhans Cell Histiocytosis and B-Lineage Lymphoid Proliferation in the Bone Marrow

• Kim M
• Kang HJ
• Shin HY
• Ahn HS
• Lee DS

• KMID: 1077415
• DOI: http://doi.org/10.3343/kjlm.2009.29.5.402

Abstract

We present three cases of concurrent Langerhans cell histiocytosis (LCH) and B-lineage lymphoid cell infiltrations and/or nodules in the bone marrow. The first patient was a 25-month-old boy who presented with LCH on the right shoulder and multiple osteolytic lesions. Bone marrow biopsy showed the presence of LCH and two large lymphoid nodules of B-lineage, which were located in the paratrabecular region. Both LCH and the lymphoid nodules resolved after treatment with prednisone, vinblastine, methotrexate, and cyclophosphamide. The second patient was a 7-month-old girl who presented with LCH in the scalp and bone marrow. In spite of the treatment, a follow-up bone marrow analysis performed after 16 months showed LCH and increased B-lineage lymphoid cells in the interstitial area. The third patient was a 26-month-old girl, and imaging studies revealed reddish skin lesions and multiple osteolytic lesions. Skin biopsy and bone marrow biopsy did not show the presence of LCH; however, we initiated the treatment on the basis of the results of imaging studies. The follow-up study after 6 months showed the presence of LCH and large, patchy infiltration of B-lymphoid cells. We report three rare cases of concurrent bone marrow involvement of LCH and B-lineage lymphoid proliferation, which strongly suggest lymphoid malignancy. Further, clonal changes should be studied to elucidate the common pathogenic mechanism between the two diseases.

Keyword

Langerhans cell histiocytosis; Lymphoid proliferation; Bone marrow

MeSH Terms

Antineoplastic Agents/therapeutic use
B-Lymphocytes/immunology/*pathology
Bone Marrow/immunology/*pathology
Cell Proliferation
Child
Child, Preschool
Cyclophosphamide/therapeutic use
Drug Therapy, Combination
Female
Histiocytosis, Langerhans-Cell/*diagnosis/drug therapy/pathology
Humans
Male
Methotrexate/therapeutic use
Prednisone/therapeutic use
Vinblastine/therapeutic use

Figure

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  Fig. 1. Langerhans cell histiocytosis and multiple large cluster of differentiation 20 (CD20)-positive, B-lineage lymphoid nodules in the bone marrow of patient 1. (A) The left bone marrow biopsy section shows diffuse S100-positive epithelioid infiltration (×100). (B) The right bone marrow biopsy section shows two extremely large lymphoid nodules and three large CD20-positive nodules (×40).

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  Fig. 2. Langerhans cell histiocytosis and interstitially scattered cluster of differentiation 20 (CD20)-positive B-lymphoid cells in the bone marrow of patient 2. (A) The left bone marrow biopsy section showed increased number of S100-positive cells (× 200). (B) The right bone marrow biopsy section showed interstitial infiltration of CD20-positive cells (× 200).

• 

  Fig. 3. Langerhans cell histiocytosis and cluster of differentiation 20 (CD20)-positive B-lymphoid cell infiltration in the bone marrow of patient 3. (A) The left bone marrow biopsy section showed increased number of S100-positive cells (×200). (B) The left bone marrow biopsy section showed large, patchy infiltration of CD20-positive cells (×100).

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