Original Articles
Near Tetraploidy in Three Cases of Acute Myeloid Leukemia Associated with Mediastinal Granulocytic Sarcoma

https://doi.org/10.1016/S0165-4608(97)00294-XGet rights and content

Abstract

Granulocytic sarcoma (GS) is a rare manifestation of acute myeloid leukemia (AML), blastic transformation of chronic myeloid leukemia, and the myelodysplastic syndromes. The mediastinum is an unusual site of presentation. We report a series of three female patients with mediastinal GS. They were characterized by the presence of large and bizarre blast cells and near tetraploidy on cytogenetic analysis. All three patients responded poorly to chemotherapy. Near tetraploid AML is a rare entity, usually present in male patients, and has not been associated with GS. The clinical and pathological similarities in these three cases suggest a distinct category of poor-risk AML for which more intensive treatment is needed.

Introduction

Granulocytic sarcoma (GS) is a rare presenting feature or complication of acute myeloid leukemia (AML) [1]. Cytogenetic data of GS are limited. Chromosomal abnormalities, t(8;21)(q22;q22) and inv(16)(p13q22), were reported to be associated with a higher incidence of GS. GS affecting the mediastinum is rare, 13 such cases having been reviewed earlier [2]. Three cases of AML associated with mediastinal GS are presented. Cytogenetic analysis in all three cases showed near tetraploidy, a rare cytogenetic abnormality in AML.

Section snippets

Case 1

A 29-year-old woman presented with menstrual irregularity and bilateral ovarian masses. A full blood count showed hemoglobin (Hb), 8.7 g/dL; a white cell count (WBC), 11.2 × 109/L; and a platelet count (Plt), 279 × 109/L. Laparotomy and bilateral salpingo-oophorectomy were performed in another hospital, and the diagnosis was reported as a high-grade lymphoma. Bone-marrow biopsy was normal. Six monthly courses of CHOP (cyclophosphamide, 750 mg/m2; adriamycin, 50 mg/m2; vincristine,1.4 mg/m2;

Histopathology

In patient 1, the salpingo-oophorectomy specimen showed diffuse infiltration of the fallopian tubes by myeloblasts with occasional myelocytes. In patient 2, the right cervical lymph node showed diffuse infiltration by myeloblasts with only occasional remnant follicles. In both cases, the blasts had fine nuclear chromatin and scanty cytoplasm, and many of them were positive for chloroacetate esterase, confirming their myeloid origin.

Morphologic, Immunophenotypic, and Cytogenetic Studies

The morphologic and immunophenotypic features of the cases are

Discussion

Tetraploidy is an uncommon cytogenetic finding in acute leukemia, being most common in childhood acute lymphoblastic leukemia [5]. Only about 15 cases of AML with tetraploidy have been described [6]. The reported features of AML with tetraploidy included the presence of large bizarre blasts, heterogeneity in FAB classification (all FAB classes except M3 and M7 had been described), and an overall poor prognosis 6, 7, 8, 9, 10, 11.

Pathologically, tetraploid AML usually showed large blasts, with

Acknowledgements

The authors thank T.S.K. Wan for technical assistance in cytogenetic analysis.

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