Primary splenic histiocytic sarcoma complicated with prolonged idiopathic thrombocytopenia and secondary bone marrow involvement: a unique surgical case presenting with ... View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2012-10-17

AUTHORS

Sohsuke Yamada, Takashi Tasaki1, Naoko Satoh, Atsunori Nabeshima, Shohei Kitada, Hirotsugu Noguchi, Kozue Yamada, Morishige Takeshita, Yasuyuki Sasaguri

ABSTRACT

A 67-year-old Japanese female was followed up due to prolonged idiopathic thrombocytopenia with non-response to steroid therapy for 4 years, but recent progressive pancytopenia, hypo-albuminemia, and hypo-γ-globulinemia were presented. An abdominal CT scan revealed heterogeneously enhanced splenomegaly without any nodular lesions. A splenectomy was performed, and gross examination showed markedly hyperemic red pulp, weighing 760 g, accompanied by multiple foci of peripheral anemic infarction. Surprisingly, microscopic findings exhibited a diffuse proliferation of medium-sized to large tumor cells having pleomorphic nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm, predominantly within the sinuses and cords of the red pulp, which occasionally displayed conspicuous hemophagocytosis and vascular permeation. In immunohistochemistry, these atypical cells were specifically positive for CD68 (KP-1), CD163, and lysozyme, which was consistent with histiocytic sarcoma (HS) of the spleen. Subsequently, section from the aspiration of bone marrow showed infiltration of the neoplastic cells associated with erythrophagocytosis 2 months after the operation, but never before it. Therefore, primary splenic HS presenting with secondary bone marrow involvement was conclusively diagnosed. Since early diagnosis and treatment are necessary for the HS patients with poor outcomes, splenic HS should be considered as a differential diagnosis in cases with chronic thrombocytopenia and splenomegaly. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1009474924812827. More... »

PAGES

143-143

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/1746-1596-7-143

DOI

http://dx.doi.org/10.1186/1746-1596-7-143

DIMENSIONS

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PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/23075171


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90 pancytopenia
91 patients
92 peripheral anemic infarction
93 permeation
94 pleomorphic nuclei
95 poor outcome
96 primary splenic HS
97 progressive pancytopenia
98 proliferation
99 prolonged idiopathic thrombocytopenia
100 prominent nucleoli
101 pulp
102 recent progressive pancytopenia
103 red pulp
104 sarcoma
105 scans
106 secondary bone marrow involvement
107 sections
108 sinus
109 spleen
110 splenectomy
111 splenic histiocytic sarcoma
112 splenomegaly
113 surgical cases
114 therapy
115 thrombocytopenia
116 treatment
117 tumor cells
118 unique surgical case
119 vascular permeation
120 years
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