PMID- 23464972 OWN - NLM STAT- MEDLINE DCOM- 20130819 LR - 20171116 IS - 1440-1827 (Electronic) IS - 1320-5463 (Linking) VI - 63 IP - 2 DP - 2013 Feb TI - CD4 and CD8 double-negative adult T-cell leukemia/lymphoma with monomorphic cells expressing CD99: a diagnostic challenge in a country non-endemic for human T-cell leukemia virus. PG - 132-7 LID - 10.1111/pin.12040 [doi] AB - Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell neoplasm caused by human T-cell lymphotropic virus type I (HTLV-I). The neoplastic cells are highly pleomorphic and are usually CD4+ and CD8- phenotypically. We reported the case of a 46-year-old woman presenting with fever, abdominal distention, lymphadenopathy, leukocytosis and hypercalcemia. Nodal biopsy showed diffuse infiltration by monomorphic small to medium-sized atypical lymphocytes expressing CD3, CD25, CD30 and CD99, but not CD1a, CD4, CD8, CD34, terminal deoxynucleotidyl transferase or ALK. An initial diagnosis of T-lymphoblastic leukemia/lymphoma was made based on cytomorphology, CD4 and CD8 double negativity, and the expression of CD99. The diagnosis was later revised to ATLL based on the positive serology study for anti-HTLV I/II antibody and confirmation by the clonal integration of HTLV-I proviral DNA into the tumor tissues by Southern blotting analysis. The patient had a stage IVB disease and died of septic shock after 2 courses of chemotherapy 3 months after diagnosis. Immunohistochemical staining for CD99 in archival ATLL tissues showed a positive rate of 67% (4 of 6 tumors). Our case showed that ATLL with atypical morphology and immunophenotype in HTLV non-endemic areas might pose a diagnostic challenge and CD99 expression is frequent in ATLL. CI - (c) 2013 The Authors. Pathology International (c) 2013 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd. FAU - Lin, Tsung-Hsien AU - Lin TH AD - Department of Pathology, Chi-Mei Medical Center, Tainan, Taiwan. FAU - Wu, Hung-Chang AU - Wu HC FAU - Hsieh, Yen-Chuan AU - Hsieh YC FAU - Tseng, Chih-En AU - Tseng CE FAU - Ichinohasama, Ryo AU - Ichinohasama R FAU - Chuang, Shih-Sung AU - Chuang SS LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - Pathol Int JT - Pathology international JID - 9431380 RN - 0 (12E7 Antigen) RN - 0 (Antigens, CD) RN - 0 (Biomarkers, Tumor) RN - 0 (CD4 Antigens) RN - 0 (CD8 Antigens) RN - 0 (CD99 protein, human) RN - 0 (Cell Adhesion Molecules) SB - IM MH - 12E7 Antigen MH - Antigens, CD/analysis/*biosynthesis MH - Biomarkers, Tumor/*analysis MH - Biopsy MH - CD4 Antigens MH - CD8 Antigens MH - Cell Adhesion Molecules/analysis/*biosynthesis MH - *Diagnostic Errors MH - Female MH - Humans MH - Immunohistochemistry MH - Immunophenotyping MH - Leukemia-Lymphoma, Adult T-Cell/*diagnosis/*immunology MH - Middle Aged MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/immunology EDAT- 2013/03/08 06:00 MHDA- 2013/08/21 06:00 CRDT- 2013/03/08 06:00 PHST- 2012/12/02 00:00 [received] PHST- 2013/01/29 00:00 [accepted] PHST- 2013/03/08 06:00 [entrez] PHST- 2013/03/08 06:00 [pubmed] PHST- 2013/08/21 06:00 [medline] AID - 10.1111/pin.12040 [doi] PST - ppublish SO - Pathol Int. 2013 Feb;63(2):132-7. doi: 10.1111/pin.12040.