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

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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 Blymphoid cells. We report three rare cases of concurrent bone marrow involvement of LCH and Blineage lymphoid proliferation, which strongly suggest lymphoid malignancy. Further, clonal changes should be studied to elucidate the common pathogenic mechanism between the two diseases.

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 Blymphoid cells. We report three rare cases of concurrent bone marrow involvement of LCH and Blineage lymphoid proliferation, which strongly suggest lymphoid malignancy. Further, clonal changes should be studied to elucidate the common pathogenic mechanism between the two diseases.

참고문헌 (5)

  1. Adu-Poku K, "Langerhans cell histiocytosis in sequential discordant lymphoma", J Clin Pathol, 58, pp.104~106 (2005)
  2. Swerdlow SH, "WHO classification of tumors of hematopoietic and lymphoid tissues. 2nd ed", pp.358~360 (2008), IARC Press
  3. Feldman AL, "Clonal relationship between precursor T-lymphoblastic leukemia", lymphoma and Langerhans-cell histiocytosis, 2005, pp.6, 435~437
  4. Licci S, "Concurrence of marginal zone B-cell lymphoma MALT-type and Langerhans cell histiocytosis in a thyroid gland with Hashimoto disease", Ann Hematol, 87, pp.855~857 (2008)
  5. Thiele J, "Focal lymphoid aggregates (nodules) in bone marrow biopsies: differentiation between benign hyperplasia and malignant lymphoma--a practical guideline", J Clin Pathol, 52, pp.294~300 (1999)

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