Eosinophilic Fasciitis with Hypereosinophilia as the Initial Clinical Manifestation of Peripheral T-Cell Lymphoma, Not Otherwise Specified

Abstract

A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.

Keywords: T-cell lymphoma; eosinophilia; eosinophilic fasciitis; hematologic neoplasms; hypereosinophilic syndrome.

Figure 1.

(A) Diffuse inflammatory infiltrates in the fascia, composed mainly of lymphocytes with some eosinophils and histiocytes (Hematoxylin and Eosin staining, ×400 magnification). (B) Thigh muscle magnetic resonance imaging (MRI): Axial fat-suppressed, T2-weighted MRI showing an increased signal intensity within the superficial and deep fascia and enhanced T1-weighted MRI showing fascial enhancement corresponding to locations of T2 signal abnormalities.

Figure 2.

(A, B) The first computed tomography scan with no obvious abnormality. (C, D) The second computed tomography scan, in which hepatosplenomegaly, splenic infarction, renal enlargement, and multiple round low-density lesions in the liver and kidneys (arrowheads) were newly observed.

Figure 3.

(A, B) Large abnormal lymphocytes with a basophilic cytoplasm and azurophilic granules as well as hemophagocytosis in the patient’s bone marrow (May-Giemsa stain, ×1,000 magnification). (C) Bone marrow infiltrates of neoplastic cells with pleomorphic nuclei and prominent nucleoli (Hematoxylin and Eosin staining, ×400 magnification). (D) Neoplastic cells were positive for CD3, (E) CD8, and (F) granzyme B (G) but negative for CD4 (×400 magnification).