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Expanding the Phenotype of ALK-positive Histiocytosis: A Report of 2 Cases. Pediatr Dev Pathol 2018 Sep-Oct;21(5):449-455

Date

12/12/2017

Pubmed ID

29224419

DOI

10.1177/1093526617740784

Scopus ID

2-s2.0-85053878415   7 Citations

Abstract

ALK-positive histiocytosis is a recently described rare histiocytic proliferative disorder of early infancy. When infants present with anemia, thrombocytopenia, and hepatosplenomegaly, this entity should be included in the differential diagnosis along with hemophagocytic lymphohistiocytosis, metabolic/storage diseases, hematopoietic malignancies, and autoimmune thrombocytopenia. We report 2 new cases of ALK-positive histiocytosis, one with kidney involvement and the other with extensive bone marrow involvement in addition to an overt liver disease. Renal involvement by ALK-positive histiocytosis has not been reported. The infiltrating histiocytes in this case showed Rosai-Dorfman disease-like morphology including emperipolesis. The histiocytes in the second case with extensive bone marrow involvement had foamy cytoplasm initially suggesting storage disease. Our 2 cases highlight previously unrecognized diversity of ALK-positive histiocytosis in clinical manifestation, organ involvement, and cytomorphologic features and further elucidate the diagnostic challenges of this rare entity.

Author List

Huang H, Gheorghe G, North PE, Suchi M

Authors

Paula E. North MD, PhD Professor in the Pathology department at Medical College of Wisconsin
Mariko Suchi MD, PhD Associate Professor in the Pathology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Bone Marrow
Female
Histiocytosis
Humans
Infant, Newborn
Liver
Phenotype
jenkins-FCD Prod-482 91ad8a360b6da540234915ea01ff80e38bfdb40a