Annals of African Medicine
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Table of Contents
CASE REPORT
Year : 2011  |  Volume : 10  |  Issue : 4  |  Page : 310-313  

Disseminated Langerhan's cell histiocytosis: A case report and review of the literature


Department of Pathology, Ahmadu Bello University Teaching Hospital Zaria, Nigeria

Date of Web Publication2-Nov-2011

Correspondence Address:
Abdullahi Mohammed
Department of Pathology, Ahmadu Bello University Teaching Hospital Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1596-3519.87050

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   Abstract 

Langerhan's cell histiocytosis is characterized by the presence of large mononucleated cells associated with inflammatory cells. We describe the clinical presentation, histopathology and some immunohistochemistry findings, in an eight-year-old female patient.

   Abstract in French 

Histiocytose de cellule de Langerhan (LCH) est caractérisée par la présence de grandes mononucléés cellules associées aux cellules inflammatoires. Nous décrivons la présentation clinique, histopathologie et certaines conclusions immunohistochimie, dans une patiente âgée de huit ans.
Mot clés: Disseminated, Histiocytose de cellule du Langerhan

Keywords: Disseminated, langerhan′s cell histiocytosis, rare


How to cite this article:
Mohammed A, Abdullahi K, Mohammed U, Aliyu HO, Liman AA, Abdullahi S, Bello HM, Abubakar N, Abubakar M, Akpobi KC, Mafara UB. Disseminated Langerhan's cell histiocytosis: A case report and review of the literature. Ann Afr Med 2011;10:310-3

How to cite this URL:
Mohammed A, Abdullahi K, Mohammed U, Aliyu HO, Liman AA, Abdullahi S, Bello HM, Abubakar N, Abubakar M, Akpobi KC, Mafara UB. Disseminated Langerhan's cell histiocytosis: A case report and review of the literature. Ann Afr Med [serial online] 2011 [cited 2020 Aug 3];10:310-3. Available from: http://www.annalsafrmed.org/text.asp?2011/10/4/310/87050


   Introduction Top


Langerhan's cell histiocytosis (LCH) is characterized by the presence of large mononucleated cells, but occasional ones contain several nuclei, associated with inflammatory cells. Their nuclei are highly characteristic, irregular, usually elongated with prominent grooves and folds that traverse in all directions. The cytoplasm is abundant and acidophilic, sometimes to the point that an embryonal rhabdomyosarcoma is simulated. [1],[2] LCH embraces several previous eponyms, including histiocytosis X, Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and Hashimoto-Pritzker syndrome. [1],[3] It may involve the skin, bone, or may be disseminated to any organ. Hence extremely variable manifestations and an erratic course have made systemic study difficult. Many basic questions concerning the natural history, treatment, response to therapy, and prognosis remain unanswered. [4],[5],[6],[7]

Although LCH was described a century ago [8] the prevailing opinion is that LCH is a reactive disorder rather than a neoplastic process, [9] although the possibility that LCH has a viral pathogenesis is of considerable interest. McClain et al., using molecular methods to detect the genomes of the Epstein-Barr Virus, human herpes virus Type 6, and seven other viruses, found no trace of viral DNA in 50 cases of LCH. [4],[10] Leahy et al., have implicated human herpes virus Type 6, but their study requires confirmation. [4],[11] Recent ultra-structural studies also found no evidence of the virus. [4],[12] However, the remote possibility remains that LCH could result from a virally induced clonal proliferation of dendritic Langerhan's cells. [4]


   Case Report Top


An eight-year-old girl presented with a four-month history of left maxillary swelling which was increasing gradually in size, there was associated toothache and palatal swelling which increased gradually and later ulcerated. There was associated history of fever, however, no trauma, chronic cough, loss of tooth or weight loss. Past medical and family history were not contributory. When examined patient was ill-looking, mildly pale, had finger clubbing and peripheral lymphadenopathy. On maxillofacial examination there was halitosis, asymmetrical facial swelling with an oral swelling extending from the left side of the face to the right side which was firm, tender, attached to the underlying tissue. There was gross disfigurement of the nose, areas of pus collection and almost complete occlusion of the right nose [Figure 1]. Internal oral examination revealed areas of necrosis on the hard palate with soft palate involvement. There was gingival inflammation. Other systemic examination revealed liver and spleen enlargement (4 cm each).
Figure 1: Left maxillary involvement by histiocytosis

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Laboratory investigations showed:

Pack cell volume [PCV] 21%, White blood cell count [WBC]- 2.9 x 10 9 / L, platelet 145 x 10 9 / L , poikilocytosis +++, anisocytosis ++, neutrophils 63%, eosinophils 0.7%, lymphocytes 30%, and monocyte 2%.

  • Retroviral studies-Negative
  • Stool and urine tests were normal
  • Electrolyte/Urea/Creatinine [E/U/Cr]- Urea 7.5 mol/L(elevated) and Sodium [Na] 130 mol/L (decreased)
  • Liver function tests [LFT] Aspartate transaminase [AST] 29 1 U/l is raised other serum hepatic enzymes remained within normal limits.
  • X-ray showed destruction of the maxilla on the left side with associated loosening of teeth [Figure 2].
  • Figure 2: Destruction of the left maxilla and loosening of teeth

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  • Histological sections of a cervical lymph node show effacement of the nodal architecture by a cellular lesion growing in sheets. It was composed of histiocytoid cells with oval, occasionally grooved vesicular nuclei and moderate to abundant amphophilic foamy cytoplasm. Numerous normal and abnormal mitoses were noted. The stroma was scanty and infiltrated moderately by lympho-plasma cells. Areas of necrosis were seen [Figure 3]. Immunohistochemistry showed negativity for CD45 [Figure 4], Cytokeratin and EMA (epithelial membrane antigen, [Figure 5]) was positive for vimentin [Figure 6]. The overall features were those of a malignant histiocytic tumor.
  • Bone marrow biopsy showed features similar to the lymph node.
  • Figure 3: Histology of lymph node showing the lesion with cells having nuclear grooves [H and E, ×400]

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    Figure 4: Histology of tumor showing negativity for CD45 while other inflammatory cells are positive [×400]

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    Figure 5: Histology showing tumor cells negative for Epithelial membrane antigen [EMA] [×400]

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    Figure 6: Histology showing tumor cells positive for Vimentin [×400]

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  • A diagnosis of Langerhan's cell histiocytosis was made.


Outcome

The patient was placed on medical treatment (ceproxin, prednisolone) for infections before being placed on a single course of vincristine (due to her poor clinical state). Three weeks post diagnosis, she had an accidental fall which led to profuse hemorrhage with thrombocytopenia, and her subsequent death from hypovolemic shock.


   Discussion Top


LCH, an enigmatic disorder, has been variously classified as a neoplastic process, a reactive disorder, or an aberrant immune response. [4],[13] A wide spectrum of diseases and variable clinical behavior are characteristic. Although histopathological examination cannot predict clinical outcome, recent studies have shown that Langerhan's cells within the lesions of LCH are intrinsically proliferative. [4],[14]

There was an initial delay in diagnosis as she was being managed as a case of gangrenous stomatitis (Cancrum Oris). Following poor response to management, biopsy of a cervical lymph node and her bone marrow was done. This is a common occurrence as the varied presentation may mimic  Rosai-Dorfman Disease More Details (RDD), parasitic infections, Kimura's disease and some types of malignant lymphomas (Hodgkin's lymphoma, peripheral T-cell lymphoma). Light microscopy of both tissues showed features of a malignant histiocytic tumor on Hematoxylin and Eosin-stained paraffin section. Although the most useful formalin-resistant epitopes for LCH are S-100 protein and CD1a, [2] these were not available in this centre, hence we performed immunohistochemical studies of the case using four clonal antibodies (cytokeratin, EMA, CD45 and vimentin) to differentiate the cell type. The tumor cells were found to be negative for CD45, EMA and cytokeratin. They, however, showed positivity for vimentin. [2]

Considering her age and the generalized nature of her condition, our result confirmed disseminated LCH.

Multisystem disease should be treated with systemic multi-agent chemotherapy. Several different regimens have been proposed, but a central element is the inclusion of either vinblastine or etoposide. [15] In this case, due to the frail condition of the patient and the unavailability of the recommended key drugs, vincristine was substituted. The response rate to therapy may be high, especially if the diagnosis is accurately and expeditiously ascertained. [15] Our patient, however, had many poor prognostic factors, ranging from delay in diagnosis to poor supportive therapy.

Young patients with multisystem disease and poor response to initial therapy may benefit from immunosuppressive therapy, certain new agents (2-chlorodeoxyadenosine) and bone marrow transplantation. [15]


   Acknowledgment Top


We acknowledge the assistance of the following people for their contribution in arranging the final manuscript: Dr. Tunde Talib Sholadoye, Registrar, Department of Surgery, ABUTH, Zaria, Nigeria and Mr. Lucky I. Aghemunu, Department of Histopathology, ABUTH, Zaria, Nigeria.

 
   References Top

1.Emile JF, Fraitag S, Leborgne M, de Prost Y, Brousse N. Langerhans' Cell Histiocytosis Cells are activated Langerhans Cell. Jour pathol 1994;174:71-6.  Back to cited text no. 1
    
2.Rosai J. Lymph nodes. In: Rosai, Ackerman's, editors. Surgical Pathology. 29 th ed. Netherlands: Elsevier; 2004. p. 1877-2017.  Back to cited text no. 2
    
3.Writing group of the Histiocytic society. Histiocytosis syndrome in children. Lancet 1987;1:208-9.  Back to cited text no. 3
    
4.Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, et al. Langerhans'-cell histiocytosis (histiocytosis X)--a clonal proliferative disease. N Engl J Med. 1994;331:154-60.  Back to cited text no. 4
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5.Lieberman PH. Jone CR, Dargon HW, Begg CF. A reappraisal of eosinophilic granuloma of bone, Hand-schuller-Christian syndrome and letterer-siwe syndrome. Medicine 1969;48:375-400.  Back to cited text no. 5
    
6.Greenber JS, Croker AC, Vawter G, Jaffe N, Cassady JR. Results of treatment of 127 patients with systemic histiocytosis. Medicine 1981;60:311-37.  Back to cited text no. 6
    
7.Womer BR, Raney RB, DAngio JG. Healing Rates of treatment and untreated Bone lesions in Histiocytosis X. J Am Ac Pediatr 1985;76:286-8.  Back to cited text no. 7
    
8.Hand A Jr. Polyuria and tuberculosis. Arch Pediatr 1893;10:673-5.  Back to cited text no. 8
    
9.Komp DM. Langerhan's Cell Histiocytosis. N Eng med 1987;316:747-8.  Back to cited text no. 9
    
10.McClain K, Jin H, Gresik V, Favara B. Langerhan's cell histiocytosis-lack of viral etiology. A M J Hematol 1994;47:16-20. (In press).  Back to cited text no. 10
    
11.Leahy MA, Krejci SM, Friednash M, Stockert SS, Wilson H, Huff JC, et al. Human herpes virus 6 is present in lesions of langerhans cell histiocytosis. J invest Dermatol 1993;101:642-5.  Back to cited text no. 11
[PUBMED]    
12.Mierau GW, Wills EJ, Steele PO. Ultrastructural studies in langerhans cell histiocytosis: A search for evidence of viral etiology. Pediatr Patho 1994;14:895-904. (In press).  Back to cited text no. 12
    
13.Favara BE. Langerhan's Cell Histiocytosis: Patho boil. Semin oncol 1991;18:3-7.  Back to cited text no. 13
[PUBMED]    
14.Hage C, Willman CL, Favara BE, Isaacson PG. Langerhan's cell histiocytosis (histiocytosis X). Immunophenotype and growth fraction. Hum Pathol 1993;24:840-5.  Back to cited text no. 14
[PUBMED]    
15.Behrman RE, Kliegman RM, Jenson HB. Class 1 Histiocytoses. Nelson Textbook of Pediatrics, 16 th ed. Philadelphia: W.B. Saunders; 2000. p. 1570-2.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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   Introduction
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   Discussion
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