American Society of Hematology

Fig. 2.

$Fig. 2. Histopathology and immunopathology of lymphomas in ALPS patients. / (A) T-cell and histiocyte-rich B-cell lymphoma diagnosed in patient 3-II-3. Large polylobated tumor cells, which stained for CD20, are seen in a background of T lymphocytes and histiocytes. (B-D) Nodular lymphocyte-predominant Hodgkin disease diagnosed in patient 3-II-1. (B) A rim of epithelioid granulomas surrounds a large nodule containing neoplastic cells. (C) A single polylobated L&H cell is identified in a background of small lymphocytes. (D) A CD20+ L&H cell is rosetted by a rim of CD20− T cells. Numerous CD20+ small B cells are present in the background. (E) Burkitt lymphoma presenting as an omental mass in patient 26-II-4. Tumor cells are moderate in size with clumped chromatin and basophilic nucleoli. A prominent starry sky is present. (F) Tumor cells uniformly express CD20. The growth fraction was 100% with MIB-1 (Ki-67) staining. Identical histologic and immunophenotypic features were seen in lymphoma involving small bowel in patient 30-III-2. (G) An unusual form of follicular lymphoma was diagnosed in patient 55-II-1. Neoplastic cells surround hyalinized and possibly regressed follicular remnants. (H) CD20 immunohistochemistry emphasized follicular pattern. (I) The neoplastic cells are moderate in size with clumped chromatin. (J) A follicle-center origin for tumor cells is supported by Bcl-6 immunohistochemistry, which selectively stains the neoplastic cells within the follicles. Original magnification (A), ×400; (B), ×60; (C), ×400; (D), ×400; (E), ×400; (F), ×400; (G), ×200; (H), ×200; (I), ×400; and (J), ×250.$

Histopathology and immunopathology of lymphomas in ALPS patients.

(A) T-cell and histiocyte-rich B-cell lymphoma diagnosed in patient 3-II-3. Large polylobated tumor cells, which stained for CD20, are seen in a background of T lymphocytes and histiocytes. (B-D) Nodular lymphocyte-predominant Hodgkin disease diagnosed in patient 3-II-1. (B) A rim of epithelioid granulomas surrounds a large nodule containing neoplastic cells. (C) A single polylobated L&H cell is identified in a background of small lymphocytes. (D) A CD20⁺ L&H cell is rosetted by a rim of CD20⁻ T cells. Numerous CD20⁺ small B cells are present in the background. (E) Burkitt lymphoma presenting as an omental mass in patient 26-II-4. Tumor cells are moderate in size with clumped chromatin and basophilic nucleoli. A prominent starry sky is present. (F) Tumor cells uniformly express CD20. The growth fraction was 100% with MIB-1 (Ki-67) staining. Identical histologic and immunophenotypic features were seen in lymphoma involving small bowel in patient 30-III-2. (G) An unusual form of follicular lymphoma was diagnosed in patient 55-II-1. Neoplastic cells surround hyalinized and possibly regressed follicular remnants. (H) CD20 immunohistochemistry emphasized follicular pattern. (I) The neoplastic cells are moderate in size with clumped chromatin. (J) A follicle-center origin for tumor cells is supported by Bcl-6 immunohistochemistry, which selectively stains the neoplastic cells within the follicles. Original magnification (A), ×400; (B), ×60; (C), ×400; (D), ×400; (E), ×400; (F), ×400; (G), ×200; (H), ×200; (I), ×400; and (J), ×250.

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