PMID- 32150530 OWN - NLM STAT- MEDLINE DCOM- 20201130 LR - 20201130 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 21 DP - 2020 Mar 9 TI - Composite Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma and Mantle Cell Lymphoma; Small Cell Variant: A Real Diagnostic Challenge. Case Presentation and Review of Literature. PG - e921131 LID - 10.12659/AJCR.921131 [doi] AB - BACKGROUND Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and mantle cell lymphoma (MCL) both have a common origin arising from mature CD5+ B-lymphocytes. Their distinction is crucial since MCL is a considerably more aggressive disease. Composite lymphoma consisting of CLL/SLL and MCL has been rarely reported. This type of composite lymphoma may be under-diagnosed as the 2 neoplasms have many features in common, both morphologically and immunophenotypically. CASE REPORT We report the case of a 57-year-old male patient who presented with a 4-month history of recurrent abdominal pain and distention with hepatosplenomegaly. Peripheral blood showed a high leukocytes count (46.7x10(3)/uL) with marked lymphocytosis of 35.0x10(3)/uL, mostly small mature-looking, with some showing nuclear irregularities, with approximately 3% prolymphocytes. Immunophenotyping by flow cytometry and immunohistochemistry revealed 2 immunophenotypically distinct abnormal CD5+monotypic B-cell populations. Fluorescence in situ hybridization (FISH) on peripheral blood demonstrated IGH/CCND1 rearrangement consistent with t(11;14) in 65% of cells analyzed. Accordingly, based on compilation of findings from morphology, flow cytometry, immunohistochemistry, and FISH, A diagnosis of composite lymphoma consisting of MCL; small cell variant and CLL/SLL was concluded. CONCLUSIONS We describe a case of composite lymphoma of MCL (small cell variant) and CLL/SLL that emphasizes the crucial role of the multiparametric approach, including vigilant cyto-histopathologic examination, immunophenotyping by flow cytometry and immunohistochemistry, as well as genetic testing, to achieve the correct diagnosis. FAU - Ibrahim, Feryal AU - Ibrahim F AD - Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar. FAU - Al Sabbagh, Ahmad AU - Al Sabbagh A AD - Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar. FAU - Amer, Aliaa AU - Amer A AD - Department of Laboratory Medicine and Pathology, Central Clinical Laboratory, Hamad Medical Corporation, Doha, Qatar. FAU - Soliman, Dina S AU - Soliman DS AD - Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar. AD - Department of Clinical Pathology, National Cancer Institute, Cairo University, Cairo, Egypt. FAU - Al Sabah, Hesham AU - Al Sabah H AD - Department of Hematology and Medical Oncology, National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20200309 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 RN - 0 (Biomarkers, Tumor) RN - 0 (IGH-CCND1 fusion protein, human) RN - 0 (Oncogene Proteins, Fusion) SB - IM MH - Biomarkers, Tumor MH - Composite Lymphoma/*diagnosis/*pathology MH - Diagnosis, Differential MH - Flow Cytometry MH - Gene Rearrangement MH - Humans MH - Immunohistochemistry MH - Immunophenotyping MH - In Situ Hybridization, Fluorescence MH - Leukemia, Lymphocytic, Chronic, B-Cell/*diagnosis/*pathology MH - Lymphoma, Mantle-Cell/*diagnosis/*pathology MH - Male MH - Middle Aged MH - Oncogene Proteins, Fusion/genetics PMC - PMC7083592 COIS- Conflict of interest: None declared Conflict of interest None. EDAT- 2020/03/10 06:00 MHDA- 2020/12/01 06:00 PMCR- 2020/03/09 CRDT- 2020/03/10 06:00 PHST- 2020/03/10 06:00 [entrez] PHST- 2020/03/10 06:00 [pubmed] PHST- 2020/12/01 06:00 [medline] PHST- 2020/03/09 00:00 [pmc-release] AID - 921131 [pii] AID - 10.12659/AJCR.921131 [doi] PST - epublish SO - Am J Case Rep. 2020 Mar 9;21:e921131. doi: 10.12659/AJCR.921131.