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IMAGES IN HEMATOLOGY

Incidental littoral cell angioma in refractory

immune thrombocytopenic purpura

A B C

D E F
c Juli-Anne Gardner and Katherine Devitt, University of Vermont Medical Center

A 21-year-old man presented with immune thrombocytopenic purpura refractory to steroids and intravenous
immunoglobulin. Imaging revealed mild splenomegaly without lesions. Laparoscopic splenectomy was performed
for refractory thrombocytopenia. Gross examination showed a 250-g spleen without abnormalities. Histologically, a
single, well-circumscribed, 2-mm subcapsular lesion with anastomosing vascular channels was identified. The vascular
spaces were lined with tall columnar endothelial cells without atypia (panels A and C, hematoxylin and eosin stain;
panel A, original magnification 34; panel C, original magnification 350). The lesion was negative for CD8 (panel
B; original magnification 34) and CD34 (panel D; original magnification 350) and positive for CD31 (panel E;
original magnification 350) and CD163 (panel F; original magnification 350) by immunohistochemistry.
Littoral cell angiomas (LCAs) are rare primary vascular neoplasms of the spleen originating from littoral cells that
line red pulp sinuses. Unlike normal littoral cells, LCAs are classically negative for CD8. They are often discovered
incidentally or the patient may present with abdominal pain, fever, and symptoms of hypersplenism. In this case,
it is unlikely that such a small solitary lesion contributed to the patient’s thrombocytopenia. Definitive diagnosis
requires histologic and immunohistochemical studies after splenectomy. LCAs were originally thought to be benign,
but recently, they have been described as having some malignant potential as well as an association with visceral
malignancies and immunologic disorders. Given these possibilities, close clinical follow-up is recommended.

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DOI 10.1182/blood-2016-10-748772 © 2017 by The American Society of Hematology

1564 BLOOD, 16 MARCH 2017 x VOLUME 129, NUMBER 11

 From www.bloodjournal.org by guest on April 13, 2017. For personal use only.

2017 129: 1564

doi:10.1182/blood-2016-10-748772

Incidental littoral cell angioma in refractory immune thrombocytopenic

purpura
Juli-Anne Gardner and Katherine Devitt

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