D102 CASE REPORTS: UNUSUAL MANIFESTATIONS OF SARCOID AND OTHER SYSTEMIC DISEASES / Poster Discussion

Session / Wednesday, May 19/1:30 PM-4:00 PM / Room 291-292 (Second Level), Morial Convention Center

Systemic Mastocytosis In A Patient With Unusual Neck And Pleural Involvement

J. Kim1, H. kang1, J. Kim1, M. Kim1, S. Kim1, H. Yun1, J. Ahn2, C. Kim1, K. Kim1, J. Song1
1The Catholic University of Korea, College of Medicine, Seoul, Korea, 2The Catholic University of Korea, College of Medicine, seoul, Korea

Introduction
Systemic mastocytosis is a rare disease characterized by an abnormal proliferation of mast cells that infiltrate the skin, bone marrow,
spleen, liver, and lymph nodes. The release of mast cell mediators such as histamine causes systemic attack, with flushing, pruritus,
headache, gastrointestinal symptoms, and even vascular collapse with syncope. To the best of our knowledge severe lymphadenopathy of
neck and pleural involvement of systemic mastocytosis were not reported. We present a case with various manifestations such as
pulmonary, intestinal, bone, and lymph node involvement.
Case Report
A 77-year-old-woman presented with palpable neck mass and headache for 1 month. She did not complained of flushing and pruritus.
Physical examination showed bilateral cervical lymphadenopathy and decreased breathing sound in right lung field. There was no skin
lesion. Laboratory finding showed eosinophilia, elevated liver enzyme, and anemia. Plain chest films and chest computed tomography
(CT) showed large pleural effusion in right hemithorax. Neck CT also revealed multiple bilateral well defined lymphadenopathy along
cervical chain(Fig 1).
Fig 1 - Neck CT

Neck CT shows multiple lymphadenopathies.

Enlarged lymph nodes showed good contrast enhancement without central necrosis. Abdomen CT showed mesenteric and
retroperitoneal lymphadenopathy, ascites, splenomegaly and bowel wall thickening. Irregular and serrated mucosal folds were noted in
ileum on small bowel series. Bone scintigraphy showed increased radiotracer uptakes at the skull, vertebra, pelvis, both femoral heads, and
both femur shafts. Cervical lymph node biopsy and pleural biopsy were performed. Histological examination of both biopsy specimens
showed extensive mast cell infiltrations that stained metachromatically with toluidine blue confirming the diagnosis of systemic
mastocytosis (Fig 2).
Fig 2 - Neck lymph node biopsy specimen
Neck lymph node biopsy, toluidine blue stain : Positive cells are scttered diffusely.
The overall picture corresponded to the aggressive systemic mastocytosis category as defined by WHO criteria. The general status of the
patient improved with the administration of histamine antagonist and corticosteroid
Discussion
Reports of pulmonary manifestations of systemic mastocytosis are extremely rare. To our knowledge massive lymphadenopathy of neck in
systemic mastocytosis is not reported although many reported abdominal lymphadenopathy. Cervical lymphadenopathy with pleural
effusion is not uncommon in the patient of tuberculosis. In Korea, that is important problem where there is a higher incidence of
tuberculosis than Western countries. When there are multiple cervical lymphadenopathy with pleural effusion and eosinophilia, physicians
must be consider the possibility of systemic mastocystosis. It remains to be clarified if this is an isolated case or if this kind of feature is
exclusively found in the type of aggressive systemic mastocytosis.

This abstract is funded by: none

Am J Respir Crit Care Med 181;2010:A6680

Internet address: www.atsjournals.org Online Abstracts Issue