Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis.
OBJECTIVE
Crystal-storing histiocytosis (CSH) is a very rare immunoglobulin (Ig) deposition disorder that can be associated with B-cell neoplasms as well as some reactive Ig-secreting lymphoproliferative disorders. This article reports the clinical, histopathologic, and molecular biologic findings of CSH in association with an extranodal marginal zone lymphoma (EMZL) of the lacrimal gland.


METHODS
Interventional case report with clinicopathologic correlation.


METHODS
Treatment consisted of conjunctival and orbital biopsies, as well as low-dose radiation of the left orbit.


METHODS
Histopathologic findings of the conjunctival and orbital biopsies were compared. Further, extensive immunohistochemistry, polymerase chain reaction (PCR) for the detection of Ig heavy chain (IgH) gene rearrangement, Gene Scan analysis, and DNA sequencing were performed on all tissues.


RESULTS
The tumor manifestations in the specimens demonstrated a similar morphologic and immunophenotypic characteristics consistent with the diagnosis of EMZL. Immunoglobulin H PCR and Gene Scan analysis showed B cells derived from the same clone. In association with the orbital EMZL were large accumulations histiocytes filled with refractile crystals, consistent with the diagnosis of CSH.


CONCLUSIONS
The current case describes an EMZL of the lacrimal gland appearing 5 years after excision and low-dose radiation of a conjunctival lymphoma. The orbital recurrence was associated with surrounding CSH, a rare Ig storage disorder more often reported in patients with multiple myeloma or lymphoplasmacytic lymphoma or immunocytoma. Ophthalmic pathologists should be aware of the diagnosis when reviewing orbital biopsies, because difficulties may arise when the crystal-storing macrophages occupy more tissue space than the Ig-secreting cells or when they resemble cell types such as rhabdomyoblasts.
Coupland
Sarah E
SE
Department of Pathology, University Hospital Benjamin Franklin, Freie Universität, Hindenburgdamm 30, D-12200 Berlin, Germany. secoupland@yahoo.de
Foss
Hans-Dieter
HD
Hummel
Michael
M
Stein
Harald
H
D015703:Antigens, CD / Q000032:analysis; D003460:Crystallization; D004247:DNA / Q000032:analysis; D005134:Eye Neoplasms / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D005260:Female; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain / Q000235:genetics; D005803:Genes, Immunoglobulin / Q000235:genetics; D015614:Histiocytosis / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D007154:Immune System Diseases / Q000150:complications* / Q000175:diagnosis; D007124:Immunoenzyme Techniques; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D016130:Immunophenotyping; D002479:Inclusion Bodies / Q000473:pathology; D007766:Lacrimal Apparatus Diseases / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D008875:Middle Aged; D016133:Polymerase Chain Reaction
D015703:Antigens, CD; D007143:Immunoglobulin Heavy Chains; D004247:DNA