Clinical Information
Seven males and 6 females (median age, 38 years; range, 12-64 years) had FHL in lymph nodes of the neck (6 patients), axilla (3 patients), groin (2 patients), and mediastinum (1 patient) and in the nasopharynx (1 patient). Detailed clinical information was available for the 4 patients with FHL treated at the University of Michigan, with follow-up ranging from 8 months to 10 years.
Case 1. In a 43-year-old woman with a left-sided neck mass, a computed tomography scan revealed 2 lymph nodes measuring 2.2 and 1.2 cm (stage IB). She was given multi-agent chemotherapy (doxorubicin, bleomycin, vinblastine, dacarbazine [ABVD] for 6 cycles), leading to a complete response; follow-up 1 year later showed no evidence of disease.
Case 2. A 28-year-old woman with a 2-cm supraclavicular mass and small ipsilateral infraclavicular lymph nodes sought care at 28 weeks of pregnancy. Staging revealed a 4 4 3-cm mass in the left supraclavicular region and anterior mediastinum, with 2 small suspicious lesions in the spleen (at least stage IIA, possibly IIIs). The disease responded to chemotherapy (ABVD for 6 cycles), and, at last follow-up, the patient had been in remission for 8 months.
Case 3. A 38-year-old man had stage IIB nodular sclerosis Hodgkin disease at initial examination, diagnosed following an inguinal lymph node biopsy. The disease recurred 1 year after treatment (chemotherapy, ABVD for 6 cycles; and radiation) as FHL in axillary lymph nodes, which responded to high-dose cyclophosphamide and a peripheral stem cell transplant.
Case 4. A 31-year-old woman with axillary and epitrochlear lymphadenopathy originally was given a diagnosis of lymphocyte predominance Hodgkin disease (subsequently reclassified as FHL). Staging at this time did not reveal evidence of disease in the spleen, liver, bone marrow, or iliac lymph nodes (stage II, probably A). Six years after radiation therapy, the disease recurred as FHL in iliac and periaortic lymph nodes, with involvement of bone marrow. She then received chemotherapy (ABVD for 6 cycles) and, at last follow-up, had been in remission for 9 years.
Histopathologic and Immunohistochemical Findings
All FHL cases showed a prominent follicular architecture, usually composed of small to intermediate-sized follicles (Figure 1a). The characteristic feature, seen in all cases, was the presence of follicles with expanded mantle zones, occasionally with small, well-defined, often eccentric, germinal centers (Figure 1b). Scattered classic Reed-Sternberg cells and variants (usually lacunar, occasionally mononuclear or mummified) were found within these mantle zones. Hyperplastic reactive follicles were noted in 5 of 13 cases (Figure 2). and apparent primary follicles in 13 cases. Classic Reed-Sternberg cells or variants were identified in the mantle zones of all these follicles. Mild capsular thickening was observed in the absence of intranodal fibrosis. In 11 of 13 cases, a vessel was noted to penetrate occasional follicles with expanded mantle zones, with the histologic appearance reminiscent of follicles in angiofollicular hyperplasia.
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Immunoperoxidase stains for B- and T-cell markers showed a characteristic immunoarchitecture even at low magnification. The B-cell markers highlighted the follicles, with presence of nonstaining holes within expanded mantle zones (Figure 3a), while a corresponding CD3epsilon stain outlined the nodular architecture and rosettes of CD3epsilon+ cells within the mantle zones (Figure 3c). Higher magnification showed these holes to contain a classic Reed-Sternberg cell or variant, with the expected immunophenotype (CD30+, CD15 ±) (Figure 4), surrounded by a rosette of small T lymphocytes (Figure 3b) and (Figure 3d). These T-cell rosettes were CD3epsilon+ CD57 in 11 of 13 cases and CD3epsilon+ CD57+ in 2 of 13 cases. In 6 of 13 cases, the Reed-Sternberg cells showed heterogeneous positivity for a B-cell antigen by immunoperoxidase staining. Stains for CD23 highlighted well-defined germinal centers and the dendritic reticulum meshwork within the follicles.[10] A summary of the clinicopathologic features of all 13 cases of FHL is presented in Table 2.
In general, cases of NLPHL showed effacement of nodal architecture by large, often back-to-back nodules, occasionally with a rim of benign, uninvolved nodal tissue, consistent with our current understanding of the morphologic features of NLPHL. The neoplastic nodules were composed predominantly of small B lymphocytes with scattered Reed-Sternberg variants cytologically consistent with lymphocytic and histiocytic (L&H) cells. Well-defined germinal centers were not identified. Immunoperoxidase stains for B-cell markers decorated the nodules, with a disrupted dendritic reticulum meshwork highlighted by CD23. Immunophenotypically, the L&H cells showed reactivity for B-cell markers in the absence of CD15 or CD30. Occasionally, rosettes of CD3epsilon+ T cells surrounding the L&H cells also were CD57+.
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