Pseudo-Gaucher Cells in the Bone Marrow of a Patient with Hodgkin’s Disease

Abstract

The authors studied an 18-year-old woman with stage IIIB nodular sclerosis Hodgkin's disease whose bone marrow contained abnormal storage cells that resembled Gaucher cells by light microscopic examination ("pseudo-Gaucher" cells). Electron microscopic examination revealed that these cells differed from true Gaucher cells and resembled storage cells previously described in chronic myelogenous leukemia. The patient's peripheral blood leukocyte beta-glucosidase and serum acid phosphatase levels were elevated, ruling out the diagnosis of inherited Gaucher's disease. After treatment with six monthly cycles of systemic chemotherapy (nitrogen mustard, vincristine, procarbazine, bleomycin, doxorubicin, and prednisone), all signs of Hodgkin's disease and pseudo-Gaucher cells disappeared. Repeat leukocyte beta-glucosidase and serum acid phosphatase levels were unchanged. The present case is unique with its documentation of classical enzyme patterns for beta-glucosidase and acid phosphatase and electron microscopic features. The authors postulate that pseudo-Gaucher cells result from excessive cell breakdown with an overload of available beta-glucosidase.

Pseudo-Gaucher cells in myelodysplasia.

“…1 Since that original report, Gaucher-like or pseudo-Gaucher cells have been noted in a variety of conditions including acute lymphoblastic leukaemia, 2 Hodgkin's disease, 3 thalassaemia, 4 and multiple myeloma. 5 These cells display the "wrinkled tissue paper" appearance of the cytoplasm and the eccentric, lobulated nucleus typical of true Gaucher cells, 6 but they do not contain the tubular inclusions which are found in authentic Gaucher cells.…”

Gaucher or pseudo-Gaucher? The challenge of several diseases colliding in a pediatric patient

“…Cells morphologically very similar to Gaucher cells can be found in patients with hematologic malignancies in the absence of GD [8][9][10]. These so-called pseudo-Gaucher cells represent macrophages induced by increased turnover in rapidly dividing tumor cell populations, and they are often seen in association with hematologic malignancies involving the bone marrow, which may also feature activation of the phagocytic monocyte/macrophage system.…”

Pseudo-Gaucher cells in peritoneal fluid: An uncommon manifestation of extramedullary hematopoiesis

“…Gaucher-like cells have been described most frequently in the bone marrow of patients with chronic myelogenous leukemia 1-3 but have also been observed in patients with numerous hematologic disorders, including acute lymphoblastic leukemia 4 and Hodgkin's disease, 5 as well as nonneoplastic conditions, such as thalassemia major and immune thrombocytopenic purpura. Pseudo-Gaucher cells have been identified at extramedullary sites, including soft tissue and lung, 6 in connection with neoplastic, infectious, and inflammatory disorders (such as rheumatoid arthritis).…”

“…Also seen were numerous polygonal and elongated cells, with one or two oval to crescent-shaped nuclei and abundant cytoplasm containing prominent birefringent linear fibrils, consistent with pseudo-Gaucher cells (Figs. 4,5). The findings were indicative of extramedullary hematopoiesis (EMH).…”