Journal of Clinical Oncology, Vol 20, Issue 7
(April), 2002: 1942-1943
© 2002 American Society for Clinical Oncology
Unusual Presentations of Lymphoma
Case 1. Sea-Blue Histiocytes in Non-Hodgkin's Lymphoma
Thein H. Oo,
Leo S. Aish,
Hani Hassoun
St Elizabeth’s Medical Center of Boston, Tufts University School of Medicine, Boston, MA
A previously healthy 52-year-old Laotian woman presented with a recent history of mild postprandial epigastric discomfort and fatigue. Her physical examination revealed a spleen 3 cm below the left costal margin and no lymphadenopathy. The complete blood cell (CBC) count showed a hemoglobin level of 11.7 g/dL, a hematocrit level of 34.4%, a platelet count of 68,000/mm3, and a WBC count of 3,400/mm3 with a normal differential count. The peripheral-blood smear was unremarkable. Computed tomography showed a spleen span of 15 cm with minimal lymphadenopathy in the inguinal, anterior mediastinal, and aortopulmonary areas. Bone marrow examination disclosed multiple lymphoid aggregates with some larger lymphocytes in a germinal center–like configuration, suggesting bone marrow involvement by follicular lymphoma. Also noted was a prominent population of phagocytic histiocytes, the so-called sea-blue histiocytes (Figs 1 and 2). They were characterized by a single eccentric nucleus and cytoplasm containing large blue lysosomal granules on Giemsa stain. Flow cytometry revealed lymphocytosis with predominance of small, mature-appearing B lymphocytes (Fig 3) expressing CD19 and CD20 antigens and an immunoglobulin light-chain kappa/lambda ratio of 59:1. These cells were negative for CD5 and CD10. A diagnosis of follicular low-grade lymphocytic lymphoma was established.
The primary syndrome of sea-blue histiocytosis1 is characterized by splenomegaly, thrombocytopenia, and a relatively benign clinical course. Clusters of sea-blue histiocytes are observed in the spleen, liver, and bone marrow. Although the pathogenesis of the stored material is not well established, phospholipids, glycosphingolipids, and ceroids have been identified. Sea-blue histiocytes containing other types of lipids or glycolipids have been described in the setting of inherited enzyme deficiencies, such as Nieman-Pick disease, and in association with hyperlipidemia, total parenteral nutrition, chronic granulomatous disease, Takayasu arteritis, and hepatic porphyria. Several hematologic conditions, including idiopathic thrombocytopenic purpura,2 chronic granulocytic leukemia,3 lymphoma,4 mycosis fungoides,5 primary myelofibrosis,6 and myelodysplastic syndromes,7 have also been associated with sea-blue histiocytosis in the bone marrow. Although this is the third case of non-Hodgkin’s lymphoma presenting with sea-blue histiocytes, this association remains speculative.
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