Articles

Bone marrow infiltration in Langerhan’s cell histiocytosis - An unusual but important determinant for staging and treatment

Abstract

Background: Langerhans' cell histiocytosis (LCH) is a reactive proliferative disease of unknown pathogenesis characterized by proliferation of Langerhans cells. Involvement of bone marrow (BM), liver and lung are related to high risk factors and poor survival. The aim of this report is to highlight the clinical and haematological findings of 5 cases of LCH with BM infiltration which may help to predict involvement of BM.
Case series: Five cases of Langerhan’s cell histiocytosis with bone marrow infiltration were retrieved from archives of Department of Hematology, PGIMER and Chandigarh for review and further analysis.Male to female ratio was 3:2 with mean age of 9.4 months. Two out of 5 patients had obvious skull swelling; however, radiography of the skull revealed lytic lesion of skull in 4 cases and 2 had skin rashes. Hepatomegaly was present in 4 cases and 2 of whom also had lymphadenopathy and splenomegaly. All patients had anaemia at the time of presentation. Bone marrow aspiration and trephine biopsy in all 5 cases revealed infiltration by large histiocytes with abundant cytoplasm and coffee bean shaped nucleus. Nodules of these Langerhans cells with admixture of eosinophils were seen on trephine biopsy. Immunohistochemistry showed positivity for CD1a stain.
Conclusion: BM evaluation is important in LCH patients to categorize disease which further determines the type of therapy to be given. Clinical details may help to predict the BM involvement; however, demonstration of CD1a positive cells in marrow is most important tool to diagnose marrow infiltration by LCH.

Willman CL, Busque L, Griffith BB, et al. Langerhans’-cell histiocytosis (histiocytosis X)-a clonal proliferative disease. N Engl J Med. 1994 Jul 21; 331(3):154–60.

Egeler RM, D’Angio GJ. Langerhans cell histiocytosis. J Pediatr. 1995 Jul; 127(1):1–11.

Alston RD, Tatevossian RG, McNally RJQ, et al. Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998. Pediatr Blood Cancer. 2007 May; 48(5): 555–60.

Al-Abbadi M, Masih A, Braylan RC, et al. Soft tissue Langerhans’ cell histiocytosis in an adult. A case presentation with flow cytometric analysis and literature review. Arch Pathol Lab Med. 1997 Feb; 121(2):169–72.

Minkov M, Grois N, McClain K, et al. Histiocyte Society Evaluation and Treatment Guidelines, Langerhans cell histiocytosis. April 2009.

Haupt R, Minkov M, Astigarraga I, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013 Feb; 60(2): 175–84.

Bansal D, Marwaha RK, Trehan A, et al. Langerhans’ cell histiocytosis: experience from a single center. Indian Pediatr. 2008 Aug; 45(8): 685–8.

Glotzbecker MP, Carpentieri DF, Dormans JP. langerhans cell histiocytosis: Clinical presentation, pathogenesis, and treatment from the LCH etiology. Univ Pa Orthop J. 2002; 15:67–73.

Braier J, Chantada G, Rosso D, et al. Langerhans cell histiocytosis: retrospective evaluation of 123 patients at a single institution. Pediatr Hematol Oncol. 1999 Oct; 16(5):377–85.

A multicentre retrospective survey of Langerhans’ cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans’ Cell Histiocytosis Study Group. Arch Dis Child. 1996 Jul; 75(1):17–24.

Rivera-Luna R, Alter-Molchadsky N, Cardenas-Cardos R, et al. Langerhans cell histiocytosis in children under 2 years of age. Med Pediatr Oncol. 1996 May; 26(5):334–43.

Goyal A, Rani S, Singh T, et al. Childhood histiocytoses: a review of twenty two cases. Indian Pediatr. 1998 Feb; 35(2): 151–6.

Minkov M, Prosch H, Steiner M, et al. Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer. 2005 Nov; 45(6): 802–7.

Lahey ME. Prognostic factors in histiocytosis X. Am J PediatrHematolOncol. 1981; 3(1):57-60.

Minkov M, Pötschger U, Grois N, et al. Bone marrow assessment in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007 Oct 15; 49(5):694–8.

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IssueVol 9, No 4 (2015) QRcode
SectionArticles
Keywords
Bone marrow Histiocytes Langerhan’s cells
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How to Cite
1.
Kumar M, Sachdeva MU, Naseem S, Ahluwalia J, Das R, Varma N, Marwaha R. Bone marrow infiltration in Langerhan’s cell histiocytosis - An unusual but important determinant for staging and treatment. Int J Hematol Oncol Stem Cell Res. 2016;9(4):193-197.