Langerhans cell histiocytosis: skeletal manifestations

Case contributed by Dr Domenico Nicoletti

Presentation

11 years old female with pain and swelling about the left elbow for three months unresponsive to paracetamol.

Patient Data

Age: 11
Gender: Female

There is an osteolytic lesion about 2 cm at the distal humerus metadiaphysis with wide zone of transition, centred in medullary cavity and demonstrates no chondroid or osteoid matrix. There is a lamellar periosteal reaction. Joint effusion also noted. No pathologic fracture is evident.

The left elbow lesion is redemonstrated with low T1 and high T2 signal intensity and avid contrast enhancement. There is associated bone marrow and surrounding soft tissue oedema along with moderate joint effusion.The lesion was biopsied and the diagnosis of Langherans Cell Histiocytosis was confirmed.

PET/CT study did not find any other lesions.

Case Discussion

Langherans cell histiocytosis(LCH) is a rare disease involving clonal proliferation of Langherans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multisystem disease. LCH is part of a group of clinical syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages). These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas.

The disease has gone by several names, including Hand–Schüller–Christian disease, Abt-Letterer-Siwe disease, and histiocytosis X, until it was renamed in 1985 by the Histiocyte Society.

Acknowledgement: Dott.ssa Sandra Pennacchini

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Case information

rID: 36712
Case created: 5th May 2015
Last edited: 18th Dec 2015
Inclusion in quiz mode: Included

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